Zespół rakowiczy
Leczenie

Zespół rakowiczy (carcinoid syndrome) jest wynikiem nadmiernego wydzielania serotoniny i innych hormonów przez neuroendokrynne guzy (NET), najczęściej zlokalizowane w środkowej części przewodu pokarmowego z przerzutami do wątroby. Objawy kliniczne obejmują zaczerwienienie skóry, biegunkę, skurcze oskrzeli oraz rozwój choroby sercowo-naczyniowej. Podstawą leczenia jest chirurgiczne usunięcie guza pierwotnego lub przerzutów, co może prowadzić do całkowitego wyleczenia lub złagodzenia objawów. W przypadku nieoperacyjnych zmian stosuje się debulking, embolizację wątroby, ablację oraz farmakoterapię analogami somatostatyny (oktreotyd, lanreotyd), które kontrolują objawy u 50-70% pacjentów i spowalniają progresję guza. Dawkowanie analogów somatostatyny obejmuje formy długodziałające podawane co miesiąc oraz szybkie formy podskórne stosowane w sytuacjach nagłych, np. przełomu rakowiczego. Telotristat (250 mg trzy razy dziennie) jest stosowany w biegunce opornej na analogi somatostatyny, redukując częstość wypróżnień u 40% pacjentów. Ewerolimus, inhibitor mTOR, w połączeniu z oktreotydem LAR wydłuża czas wolny od progresji u zaawansowanych NET.

Wprowadzenie do leczenia zespołu rakowiczego

Zespół rakowiczy (ang. carcinoid syndrome) jest rzadkim zespołem objawów klinicznych spowodowanym przez guzy neuroendokrynne (NET), najczęściej pochodzące ze środkowej części przewodu pokarmowego, które przerzutowały do wątroby. Zespół charakteryzuje się nadmiernym wydzielaniem hormonów, głównie serotoniny, przez te guzy, co prowadzi do charakterystycznych objawów, takich jak zaczerwienienie skóry, biegunka, skurcze oskrzeli oraz potencjalnie do rozwoju choroby sercowo-naczyniowej.12

Leczenie zespołu rakowiczego obejmuje zarówno terapię skierowaną na guza pierwotnego, jak i leczenie objawowe. Wybór właściwego podejścia terapeutycznego zależy od wielu czynników, takich jak lokalizacja guza, stadium zaawansowania, stopień złośliwości, rodzaj wydzielanych hormonów, obecność przerzutów oraz ogólny stan zdrowia pacjenta.34

Leczenie chirurgiczne

Leczenie chirurgiczne stanowi podstawę terapii zespołu rakowiczego, szczególnie gdy guz zostanie wykryty we wczesnym stadium. Całkowite usunięcie guza poprzez zabieg chirurgiczny może prowadzić do trwałego wyleczenia i ustąpienia objawów zespołu rakowiczego.56

Rola resekcji chirurgicznej

Chirurgiczne usunięcie guza jest najlepszym sposobem leczenia zespołu rakowiczego, gdy jest to wykonalne, ponieważ może prowadzić do całkowitego i trwałego wyleczenia. W przypadku wczesnego wykrycia guzów neuroendokrynnych oskrzeli, żołądka czy odbytnicy (mniejszych niż 1 cm), ich usunięcie może prowadzić do całkowitego ustąpienia objawów zespołu rakowiczego.78

Nawet w przypadku zaawansowanej choroby, gdy całkowite usunięcie guza nie jest możliwe, zabieg chirurgicznego zmniejszenia masy guza (tzw. „debulking”) może znacząco złagodzić objawy poprzez zmniejszenie ilości hormonów wydzielanych przez guz i poprawić jakość życia pacjenta.910

Leczenie przerzutów do wątroby

Guzy neuroendokrynne często dają przerzuty do wątroby. Jeśli przerzuty do wątroby mogą być chirurgicznie usunięte, często prowadzi to do poprawy objawów zespołu rakowiczego. W przypadku pacjentów z dużym obciążeniem guzem i przerzutami, zmniejszenie masy guza (debulking) może poprawić objawy, a także przedłużyć i poprawić ogólny stan zdrowia pacjenta.11

Dla pacjentów z nieoperacyjnymi przerzutami do wątroby, terapie ukierunkowane na wątrobę mogą zmniejszyć zachorowalność i poprawić jakość życia. Opcje obejmują przezskórną przeztętniczą embolizację wątroby, radioembolizację z mikrosferami itru-90 oraz ablację.12

Analogi somatostatyny

Analogi somatostatyny (SSA) są podstawą leczenia farmakologicznego zespołu rakowiczego. Dwa najczęściej stosowane leki z tej grupy to oktreotyd (Sandostatin, Bynfezia Pen) i lanreotyd (Somatuline Depot).1314

Działanie i skuteczność analogów somatostatyny

Analogi somatostatyny naśladują działanie naturalnie występującego hormonu – somatostatyny, który hamuje wydzielanie hormonów żołądkowo-jelitowych i endokrynnych. Oktreotyd i lanreotyd wiążą się z receptorami somatostatyny na powierzchni komórek nowotworowych i hamują wydzielanie amin biogennych, co prowadzi do kontroli objawów takich jak zaczerwienienie skóry i biegunka.15

Oba leki wykazują podobną skuteczność i zapewniają złagodzenie objawów u 50% do 70% osób z zespołem rakowiczym. Poza łagodzeniem objawów, analogi somatostatyny mogą również spowalniać wzrost guza.1617

Oktreotyd LAR znacząco wydłuża czas do progresji guza w porównaniu z placebo u pacjentów z guzami neuroendokrynnymi środkowej części przewodu pokarmowego.18

Wskazania i sposób podawania

Analogi somatostatyny są podawane poprzez iniekcje podskórne lub domięśniowe. Oktreotyd LAR lub lanreotyd są standardowym leczeniem długoterminowej kontroli objawów i wzrostu guza. Lek jest podawany raz w miesiącu i zwykle przyjmowany tak długo, jak jest skuteczny, a nawet przez całe życie pacjenta.19

Szybko działająca forma oktreotydu (oktreotyd IR) jest podawana kilka razy dziennie. Często jest stosowana przez krótki czas, gdy lek jest po raz pierwszy włączany, aby sprawdzić, czy pacjent może go tolerować. Jest również stosowany przed operacją lub innymi zabiegami, aby zapobiec i opanować przełom rakowiczy.20

Działania niepożądane

Działania niepożądane związane ze stosowaniem analogów somatostatyny mogą obejmować ból brzucha, wzdęcia i biegunkę. Inne potencjalne skutki uboczne to ból w miejscu wstrzyknięcia, zmęczenie, przejściowa gorączka, podwyższony poziom glukozy w surowicy i bezobjawowa kamica dróg żółciowych.2122

Inne leki stosowane w leczeniu zespołu rakowiczego

Telotristat

Telotristat (Xermelo) jest inhibitorem enzymu hydroksylazy tryptofanu, który katalizuje etap ograniczający szybkość konwersji tryptofanu do serotoniny. Został zatwierdzony do leczenia biegunki w zespole rakowiczym u dorosłych, którzy nie są wystarczająco kontrolowani samą terapią analogami somatostatyny.2324

Telotristat zmniejsza częstotliwość wypróżnień u 40% pacjentów z biegunką oporną na leczenie analogami somatostatyny. Zalecana dawka to 250 mg trzy razy dziennie z posiłkiem.2526

Ewerolimus

Randomizowane, kontrolowane placebo badanie trzeciej fazy RADIANT-2 wykazało, że ewerolimus plus oktreotyd LAR poprawiają przeżycie wolne od progresji u pacjentów z zaawansowanymi guzami neuroendokrynnymi związanymi z zespołem rakowiczym.27

Ewerolimus jest inhibitorem mTOR, który blokuje szlak ssaczego celu rapamycyny (mTOR), odgrywający kluczową rolę w regulacji wzrostu komórek, proliferacji, metabolizmu i przeżycia.28

Interferon alfa

Interferon alfa był stosowany w kontroli objawów guzów neuroendokrynnych, ale ma mniej korzystny profil toksyczności i zwykle jest dodawany jako terapia drugiej linii w przypadku objawów opornych na leczenie. Kontroluje objawy zespołu rakowiczego u 45-63% przypadków.2930

Interferony są skuteczne w kontrolowaniu zarówno biegunki, jak i zaczerwienienia, chociaż są gorsze od analogów somatostatyny. Interferon alfa ma działanie przeciwnowotworowe poprzez zatrzymanie cyklu komórkowego komórek rakowych i stymulację odpowiedzi immunologicznych.3132

Terapie ukierunkowane na wątrobę

Pacjenci z guzami neuroendokrynnymi przewodu pokarmowego, szczególnie ci z przerzutami do wątroby, mogą być leczeni technikami radiologii interwencyjnej, takimi jak ablacja prądem o częstotliwości radiowej lub przeztętnicza chemoembolizacja (TACE). Te podejścia pomagają zmniejszyć rozmiar guza i kontrolować objawy.33

Embolizacja wątrobowa

Embolizacja tętnicy wątrobowej, wykonywana chemicznie lub poprzez zamknięcie mechaniczne, może kontrolować objawy hormonalne i przedłużać przeżycie u pacjentów reagujących biochemicznie. Jest cenną opcją paliatywną dla pacjentów z zespołem rakowiczym środkowej części przewodu pokarmowego z powodu przerzutów do wątroby i może być powtarzana u pacjentów z korzystną odpowiedzią na pierwszą procedurę.34

Połączenie różnych modalności ukierunkowanych na wątrobę skutkowało odpowiedzią objawową u 393 z 479 (82%) pacjentów, podczas gdy poziomy 5-HIAA w moczu zostały znacznie zmniejszone u 116 z 191 (61%) pacjentów.35

Ablacja o częstotliwości radiowej

Ablacja o częstotliwości radiowej dostarcza ciepło przez igłę do komórek przerzutowych w wątrobie, powodując śmierć komórek.36

Ablacja prądem o częstotliwości radiowej powoduje śmierć komórek guza rakowiczego w wątrobie.37

Krioablacja

Krioablacja wykorzystuje cykle zamrażania i rozmrażania do zabijania komórek nowotworowych.38

W wybranych przypadkach krioterapia może być skuteczna. Krioterapia bronchoskopowa została z powodzeniem zastosowana w leczeniu izolowanego guza rakowiczego śródskrzelowego u dorosłego pacjenta.39

Terapia radionuklidowa receptorów peptydowych (PRRT)

Terapia radionuklidowa receptorów peptydowych (PRRT) jest formą terapii radiologandowej. Polega na połączeniu analogu somatostatyny z substancją radioaktywną (np. lutetem-177), co pozwala na dostarczenie ukierunkowanego promieniowania bezpośrednio do komórek guza, które wykazują ekspresję receptorów somatostatyny.4041

Działanie i skuteczność PRRT

PRRT jest skuteczną formą terapii radiologandowej, która łączy analog somatostatyny z radionuklidem, takim jak lutet-177 (Lu), dostarczając ukierunkowane promieniowanie do guzów wykazujących ekspresję receptorów somatostatyny. Pacjenci z zespołem rakowiczym wykazali wysokie wskaźniki odpowiedzi objawowej na tę terapię.42

Terapia ta może być wykonywana u pacjentów, którzy nie reagują na terapię oktreotrydem. Jest to wykonywane poprzez znakowanie radioaktywne analogu somatostatyny lutetem-177 (Lu), który dostarcza ukierunkowane promieniowanie do guzów wykazujących ekspresję receptorów somatostatyny.43

Wskazania i przeciwwskazania

PRRT z 177Lu-DOTATATE stanowi skuteczną i bezpieczną opcję systemową wśród szeregu możliwości leczenia pacjentów z guzami neuroendokrynnymi z dodatnim obrazowaniem receptorów somatostatyny oraz poprawy objawów w postępującej chorobie.44

Nie wszystkie guzy rakowiczy są odpowiednie do tego leczenia. Radioaktywny oktreotyd lub oktreotate to wstrzyknięcie, które pozwala na dostarczenie radioaktywności bezpośrednio do guzów rakowiczych, które pobierają oktreotyd.45

Chemioterapia

Chemioterapia wykorzystuje silne leki do zabijania komórek nowotworowych lub spowolnienia ich wzrostu. Może być podawana dożylnie lub doustnie.46

Wskazania do chemioterapii

Chemioterapia jest zazwyczaj zarezerwowana dla pacjentów z wysokiej złośliwości guzami neuroendokrynnymi lub tych, którzy nie reagują na inne leczenie. Schematy mogą obejmować kapecytabinę, streptozocynę i temozolomid.47

Niestety, chemioterapia nie zawsze działa dobrze w przypadku guzów rakowiczych. Jest zazwyczaj stosowana tylko w przypadku niektórych typów guzów rakowiczych, takich jak te w trzustce, lub gdy guzy powodują poważne objawy, rozprzestrzeniły się lub inne leczenie nie zadziałało.4849

Schematy chemioterapii

Obecnie stosowane leki chemioterapeutyczne w badaniach klinicznych w celu złagodzenia przerzutowej choroby rakowiczej obejmują: środki alkilujące, doksorubicynę, 5-fluorouracyl, dakarbazyna, aktynomycynę D, cisplatynę, etopozyd, streptozocynę. Zwykle stosuje się kombinację wymienionych środków.50

Chemioterapia rzadko jest stosowana w praktyce klinicznej ze względu na ograniczoną rolę w kontroli objawów zespołu rakowiczego. Większość dostępnych danych jest stara i skąpa, nie opisuje wyników specyficznych dla zespołu rakowiczego, ale niestety ogranicza się do odpowiedzi biochemicznej.51

Terapia celowana

Terapie celowane koncentrują się na określonych nieprawidłowościach obecnych w komórkach nowotworowych. Blokując te nieprawidłowości, celowane leczenie może prowadzić do śmierci komórek nowotworowych. Terapia celowana jest zwykle łączona z chemioterapią w przypadku zaawansowanych guzów rakowiczych.52

Rodzaje terapii celowanej

Terapie celowane stosowane w leczeniu guzów neuroendokrynnych obejmują inhibitor kinazy mTOR (mammalian target of rapamycin) ewerolimus oraz inhibitory kinaz tyrozynowych, takie jak sunitynib. Te leki blokują lub hamują działanie cząsteczek, które sygnalizują komórkom, aby rosły lub dzieliły się.53

W badaniu randomizowanym, kontrolowanym placebo, wykazano, że ewerolimus w połączeniu z oktreotydem LAR poprawia przeżycie wolne od progresji u pacjentów z zaawansowanymi guzami neuroendokrynnymi związanymi z zespołem rakowiczym.54

Postępowanie w sytuacjach nagłych – przełom rakowiczy

Przełom rakowiczy to potencjalnie zagrażające życiu powikłanie charakteryzujące się ciężką niestabilnością hemodynamiczną, zaczerwienieniem, skurczem oskrzeli i niedociśnieniem, często wywołanym przez operację, znieczulenie lub martwicę guza. Zazwyczaj występuje u pacjentów z wysokim poziomem krążących peptydów wazoaktywnych, np. serotoniny.55

Zapobieganie i leczenie przełomu rakowiczego

Aby zapobiec przełomowi rakowiczemu podczas operacji, pacjenta należy leczyć analogiem somatostatyny, takim jak oktreotyd, albo przed, albo w trakcie zabiegu. Profilaktyczne podanie oktreotydu jest zalecane u pacjentów niestosujących SSA jako część leczenia.5657

Niektórzy pracownicy służby zdrowia mogą nie być świadomi zagrożenia przełomem rakowiczym i jak przed nim chronić. Dlatego ważne jest, aby rozmawiać ze wszystkimi pracownikami służby zdrowia o diagnozie zespołu rakowiczego, aby poinformować ich o ryzyku przełomu rakowiczego.58

U osób, które już przyjmują długo działające leczenie oktreotydem, takie jak Sandostatin LAR lub lanreotyd, mogą być potrzebne wyższe dawki oktreotydu podczas operacji. Może to wynikać z faktu, że ich organizmy przyzwyczajają się do leku w czasie (zwane tachyfilaksją), więc wyższe ilości są potrzebne, aby uzyskać ten sam efekt.59

Leczenie ostrego przełomu rakowiczego

W przypadku przełomu rakowiczego pacjent może być leczony: analogami somatostatyny, steroidami, płynami podawanymi dożylnie oraz niektórymi rodzajami leków, które mogą kontrolować ciśnienie krwi.60

Przed, w trakcie i po operacji może być konieczne podanie analogu somatostatyny o nazwie oktreotyd w postaci ciągłego wlewu do żyły, aby zapobiec przełomowi rakowiczemu.61

Leczenie powikłań zespołu rakowiczego

Choroba sercowa w zespole rakowiczym

Choroba sercowa w zespole rakowiczym jest zbiorem wszystkich problemów z sercem związanych z osobami, które mają zespół rakowiczy.62

Leczenie choroby sercowej w przebiegu zespołu rakowiczego obejmuje: leki stosowane w leczeniu chorób serca, zmiany w diecie – na przykład ostrożne podejście do przyjmowania płynów i soli, operację w celu zastąpienia dotkniętych zastawek serca sztuczną zastawką (protezą).63

Ponieważ telotristat etyl zmniejsza poziom 5-HIAA w dobowych testach moczu, może być obiecującym leczeniem, aby zapobiec lub opóźnić wystąpienie choroby sercowej w przebiegu zespołu rakowiczego.64

Pelagra i problemy żołądkowo-jelitowe

Pelagra, która jest uważana za wynik pośredni nieprawidłowo wysokich poziomów serotoniny produkowanych przez organizm, może być leczona suplementacją niacyny.6566

Tkanka bliznowata może tworzyć się w krezce u pacjentów z zespołem rakowiczym, a ta tkanka bliznowata może powodować problemy w jelicie.67

Pacjenci z zespołem rakowiczym powinni przyjmować suplementy witaminowe, szczególnie kwas nikotynowy, ponieważ guzy rakowiczy mogą powodować niedobór kwasu nikotynowego.68

Zalecenia dietetyczne i styl życia

Pewne nawyki dietetyczne i związane ze stylem życia mogą pogarszać lub przyspieszać objawy zespołu rakowiczego. Dlatego podejścia żywieniowe i związane ze stylem życia mogą pomóc w zarządzaniu zaburzeniami hormonalnymi i wspierać układ odpornościowy.69

Dieta w zespole rakowiczym

Dieta dla zespołu rakowiczego składa się zwykle z kilku mniejszych posiłków, które są bogate w białka, takie jak orzechy, fasola, soczewica i rośliny strączkowe, i nie zawierają zbyt dużo tłuszczu na raz, ponieważ duże tłuste posiłki mogą pogorszyć objawy.70

Osoby z zespołem rakowiczym powinny unikać alkoholu, który może wywoływać zaczerwienienie i inne objawy, ponieważ również zawiera aminy.71

Pacjenci powinni również unikać pewnych potraw, które mogą pogarszać objawy, takich jak alkohol, orzechy, niektóre sery i potrawy z kapsaicyną (takie jak papryczki chili).72

Inne zalecenia dotyczące stylu życia

Unikanie stresujących sytuacji i angażowanie się w pewne rodzaje aktywności fizycznej może również pomóc w zespole rakowiczym.73

Regularne stosowanie praktyk zarządzania stresem, takich jak ćwiczenia oddechowe, może pomóc uspokoić układ nerwowy i aktywować odpowiedź przywspółczulną.74

Zintegrowane podejście do leczenia

Ze względu na złożoność zespołu rakowiczego, optymalne leczenie wymaga podejścia multidyscyplinarnego, łączącego różne metody terapeutyczne. Podejście to powinno być dostosowane do indywidualnych potrzeb pacjenta, biorąc pod uwagę cechy guza i ogólny stan zdrowia.75

Sekwencja leczenia

Po leczeniu pierwszej linii z SSA, indywidualne cechy pacjenta są podstawą do podejmowania decyzji o wyborze kilku opcji drugiej lub trzeciej linii leczenia o skuteczności udowodnionej w rozsądnym stopniu.76

W leczeniu klinicznym zespół rakowiczy jest leczony w połączeniu ze statusem wydajnościowym pacjenta, pochodzeniem guza, stopniem, stadium i szybkością wzrostu, zarówno w odniesieniu do efektu anty-hormonalnego, jak i anty-proliferacyjnego.77

Badania kliniczne i nowe terapie

Istnieje kilka nowych terapii, które mogą być również pomocne w leczeniu guzów rakowiczych. Badacze analizują również nowy rodzaj radioterapii, zwany radiofarmaceutykami.78

Crinetics opracowuje paltusotynę do leczenia guzów neuroendokrynnych i zespołu rakowiczego. Paltusotyna ustanawia nową klasę doustnych, selektywnych, niepeptydowych agonistów receptora somatostatyny typu 2 (SST2). To podawane raz dziennie doustnie badane leczenie jest pierwszym środkiem w swojej klasie z raportowanymi wynikami badań klinicznych w leczeniu akromegalii.79

Istnieje niezaspokojona potrzeba dalszych dobrze zaprojektowanych randomizowanych badań kontrolowanych placebo i bezpośrednich porównań, które systematycznie oceniają kontrolę objawów zespołu rakowiczego i odpowiedź biochemiczną po określonej interwencji.80

Podsumowanie

Leczenie zespołu rakowiczego jest kompleksowe i wymaga indywidualnego podejścia. Chirurgia pozostaje najskuteczniejszą metodą, gdy jest możliwa całkowita resekcja guza. W przypadkach zaawansowanych, gdy chirurgia nie jest możliwa, dostępne są liczne opcje farmakologiczne, z analogami somatostatyny jako podstawą leczenia.8182

Dla pacjentów z objawami opornymi na leczenie analogami somatostatyny, dostępne są terapie drugiej linii, takie jak telotristat, ewerolimus, interferon alfa, terapie ukierunkowane na wątrobę oraz PRRT. Kluczowe jest również zapobieganie i leczenie powikłań, takich jak przełom rakowiczy i choroba sercowa.8384

Zintegrowane podejście, łączące leczenie medyczne, chirurgiczne, zabiegi radiologiczne, a także modyfikacje diety i stylu życia, oferuje najlepsze perspektywy dla pacjentów z zespołem rakowiczym, poprawiając jakość życia i potencjalnie przedłużając przeżycie.85

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  1. 10.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Carcinoid Syndrome – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK448096/
    Carcinoid syndrome is a rare clinical condition caused by metastatic, well-differentiated neuroendocrine tumors, primarily originating in the midgut and associated with liver metastases. […] Management includes medications to control hormone secretion and surgical interventions to reduce tumor burden. […] Diagnosis is primarily achieved through measuring urinary 5-hydroxyindoleacetic acid (5-HIAA), supported by imaging for tumor localization and staging. Management depends on the primary tumor’s type, location, and extent, including somatostatin analogs (eg, octreotide, lanreotide) to control hormone secretion, peptide receptor radioligand therapy (PRRT), and surgical or liver-directed interventions to reduce tumor burden. […] Somatostatin analogs (SSA) are the cornerstone of medical management. Two commonly used analogs, octreotide and lanreotide, mimic the action of somatostatin, an inhibitory peptide hormone that regulates the release of gastrointestinal and endocrine hormones. These drugs inhibit the secretion of biogenic amines, thereby controlling symptoms such as flushing and diarrhea.
  • #2 Carcinoid syndrome: update on the pathophysiology and treatment | Clinics
    https://www.elsevier.es/en-revista-clinics-22-articulo-carcinoid-syndrome-update-on-pathophysiology-S1807593222011310
    Approximately 30-40% of patients with well-differentiated neuroendocrine tumors present with carcinoid syndrome, which is a paraneoplastic syndrome associated with the secretion of several humoral factors. […] For several decades, patients with neuroendocrine tumors and carcinoid syndrome have been treated with somatostatin analogues as the first-line treatment. While these agents provide significant relief from carcinoid syndrome symptoms, there is inevitable clinical progression, and new therapeutic interventions are needed. […] Given the complexity involving the origin, diagnosis and management of patients with carcinoid syndrome, we have undertaken a comprehensive review to update information about the pathophysiology, diagnostic tools and treatment sequence of this syndrome, which currently comprises a multidisciplinary approach.
  • #3 Carcinoid tumors – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/carcinoid-tumors/diagnosis-treatment/drc-20351044
    Treatment for a carcinoid tumor depends on the tumor’s location, whether cancer has spread to other areas of the body, the types of hormones the tumor secretes, your overall health and your own preferences. […] Carcinoid tumor treatment options may include: […] Surgery. When detected early, a carcinoid tumor may be removed completely using surgery. If carcinoid tumors are advanced when discovered, complete removal may not be possible. In some situations, surgeons may try to remove as much of the tumor as possible, to help control signs and symptoms. […] Medications to control excess hormones. Using medications to block hormones secreted by the tumor may reduce the signs and symptoms of carcinoid syndrome and slow tumor growth. Octreotide (Sandostatin, Bynfezia Pen) and lanreotide (Somatuline Depot) are given as injections under the skin. Side effects from either medication may include abdominal pain, bloating and diarrhea. Telotristat (Xermelo) is a pill that is sometimes used in combination with octreotide or lanreotide to further try to improve the symptoms of carcinoid syndrome.
  • #4 Treating Gastrointestinal Carcinoid Tumors | American Cancer Society
    https://www.cancer.org/cancer/types/gastrointestinal-carcinoid-tumor/treating.html
    If you’ve been diagnosed with a gastrointestinal (GI) carcinoid tumor, your cancer care team will discuss your treatment options with you. It’s important to weigh the benefits of each treatment option against the possible risks and side effects. […] The main types of treatment for GI carcinoid tumors are: Surgery for Gastrointestinal Carcinoid Tumors, Chemotherapy and Other Drugs for Gastrointestinal Carcinoid Tumors, Radiation Therapy for Gastrointestinal Carcinoid Tumors. […] In some cases, doctors may recommend combining two or more types of treatment. Some of the factors that might influence your treatment options are: What organ the tumor started in, The tumor size and location, Whether it has spread to lymph nodes, liver, bones, or other organs, Whether you have any other serious medical conditions, Whether the tumor is causing bothersome symptoms (including symptoms of carcinoid syndrome).
  • #5 Carcinoid tumors – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/carcinoid-tumors/diagnosis-treatment/drc-20351044
    Treatment for a carcinoid tumor depends on the tumor’s location, whether cancer has spread to other areas of the body, the types of hormones the tumor secretes, your overall health and your own preferences. […] Carcinoid tumor treatment options may include: […] Surgery. When detected early, a carcinoid tumor may be removed completely using surgery. If carcinoid tumors are advanced when discovered, complete removal may not be possible. In some situations, surgeons may try to remove as much of the tumor as possible, to help control signs and symptoms. […] Medications to control excess hormones. Using medications to block hormones secreted by the tumor may reduce the signs and symptoms of carcinoid syndrome and slow tumor growth. Octreotide (Sandostatin, Bynfezia Pen) and lanreotide (Somatuline Depot) are given as injections under the skin. Side effects from either medication may include abdominal pain, bloating and diarrhea. Telotristat (Xermelo) is a pill that is sometimes used in combination with octreotide or lanreotide to further try to improve the symptoms of carcinoid syndrome.
  • #6 Malignant Carcinoid Syndrome Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/282515-treatment
    Complete surgical removal of all tumor tissues, when feasible, is the best treatment for carcinoid syndrome because it may result in a complete and permanent cure. Systemic medical therapy should be used to control humorally mediated symptoms when the cancer spreads elsewhere. […] Systemic therapy should be used to control humorally mediated symptoms when the cancer spreads elsewhere. Initially, somatostatin analogs (SSAs) are useful in approximately 40% of patients. […] The SSAs octreotide and lanreotide are used to control carcinoid symptoms and tumor progression in advanced inoperable disease. […] Octreotide LAR significantly extends the time to tumor progression compared with placebo for patients with midgut NETs. […] A randomized, placebo-controlled phase 3 RADIANT-2 trial found that everolimus plus octreotide LAR improved progression-free survival (PFS) in patients with advanced NETs associated with carcinoid syndrome.
  • #7 Carcinoid Syndrome Treatment – NETRF
    https://netrf.org/old-for-patients/living-with-nets/carcinoid-syndrome/carcinoid-syndrome-treatment/
    How is carcinoid syndrome treated? There are a variety of carcinoid syndrome treatments, including somatostatin analogs and other medications, liver-directed therapies, surgery, and chemotherapy. […] Somatostatin analogs include Octreotide and Lanreotide. […] Both Octreotide and Lanreotide have similar effectiveness and provide symptom relief in 50% to 70% of people with carcinoid syndrome. […] Surgical treatments aim to remove or reduce the volume of NETs, causing carcinoid syndrome. Surgery is generally reserved for patients in which 90% of the tumor can be removed and who do not have compromised liver function or extensive liver metastases. […] If liver metastases can be surgically removed, it often leads to the improvement of carcinoid syndrome symptoms. […] For patients with heavy tumor burden and metastases, tumor debulking (removing large portions of the tumor) may improve symptoms as well as extend and improve the overall health of the patient.
  • #8 Carcinoid Syndrome Treatment – NETRF
    https://netrf.org/old-for-patients/living-with-nets/carcinoid-syndrome/carcinoid-syndrome-treatment/
    If diagnosed early, bronchial NETs may be removed through surgery. The outcome of this therapy may offer total relief of symptoms caused by carcinoid syndrome. […] Surgical removal of stomach and rectal tumors (smaller than 1 cm) that are detected early may lead to total relief of the symptoms caused by carcinoid syndrome. […] Liver embolization therapy treats liver tumors by blocking the blood (or nutrient) supply to the tumor. […] Chemotherapy drugs aim to kill cancer cells and, in some cases, may shrink the tumor. […] PRRT is a nuclear medicine treatment that involves using a radioactive nuclide attached to a somatostatin analog. This systemic treatment delivers radiation directly to the tumor to kill cancer cells.
  • #9 Carcinoid tumors – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/carcinoid-tumors/diagnosis-treatment/drc-20351044
    Treatment for cancer that spreads to the liver. Carcinoid tumors commonly spread to the liver. Treatments may include surgery to remove part of the liver, blocking blood flow to the liver (hepatic artery embolization), and using heat and cold to kill cancer cells. Radiofrequency ablation delivers heat treatments that cause carcinoid tumor cells in the liver to die. Cryoablation uses cycles of freezing and thawing to kill cancer cells.
  • #10 Malignant Carcinoid Syndrome Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/282515-treatment
    Surgery should also be considered for resection of hepatic recurrence, even after previous resection, but only if the lesions are in a site where resection can be performed with minimal morbidity. […] For any patient with controlled carcinoid symptoms and heart involvement, cardiac surgery must be considered for symptomatic carcinoid heart disease and must be performed by an experienced team (including medical, surgical, and anesthesiology experts) in order to provide the best management of this condition. […] Surgical resection can provide effective palliation in carefully selected patients.
  • #11 Carcinoid Syndrome Treatment – NETRF
    https://netrf.org/old-for-patients/living-with-nets/carcinoid-syndrome/carcinoid-syndrome-treatment/
    How is carcinoid syndrome treated? There are a variety of carcinoid syndrome treatments, including somatostatin analogs and other medications, liver-directed therapies, surgery, and chemotherapy. […] Somatostatin analogs include Octreotide and Lanreotide. […] Both Octreotide and Lanreotide have similar effectiveness and provide symptom relief in 50% to 70% of people with carcinoid syndrome. […] Surgical treatments aim to remove or reduce the volume of NETs, causing carcinoid syndrome. Surgery is generally reserved for patients in which 90% of the tumor can be removed and who do not have compromised liver function or extensive liver metastases. […] If liver metastases can be surgically removed, it often leads to the improvement of carcinoid syndrome symptoms. […] For patients with heavy tumor burden and metastases, tumor debulking (removing large portions of the tumor) may improve symptoms as well as extend and improve the overall health of the patient.
  • #12 Carcinoid Syndrome – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK448096/
    Telotristat is an FDA-approved agent to treat refractory diarrhea not responding to SSA. It inhibits the enzyme tryptophan hydroxylase, which catalyzes a rate-limiting step in the conversion of the tryptophan to serotonin. […] PRRT is a targeted systemic treatment for advanced neuroendocrine tumors that express somatostatin receptors, utilizing radiolabeled somatostatin analogs (eg, lutetium-177) to deliver localized ionizing radiation to tumor cells. […] Surgery is critical in managing patients with disease that is amenable to cytoreduction. In metastatic midgut tumors, removing over 70% of the tumor burden improves symptoms and overall survival, while resection can be curative in other tumor sites (eg, the bronchus). […] For patients with inoperable hepatic metastases, liver-directed therapies can reduce morbidity and improve quality of life. Options include percutaneous hepatic transarterial embolization, radioembolization with yttrium-90 microspheres, and ablation.
  • #13 Carcinoid tumors – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/carcinoid-tumors/diagnosis-treatment/drc-20351044
    Treatment for a carcinoid tumor depends on the tumor’s location, whether cancer has spread to other areas of the body, the types of hormones the tumor secretes, your overall health and your own preferences. […] Carcinoid tumor treatment options may include: […] Surgery. When detected early, a carcinoid tumor may be removed completely using surgery. If carcinoid tumors are advanced when discovered, complete removal may not be possible. In some situations, surgeons may try to remove as much of the tumor as possible, to help control signs and symptoms. […] Medications to control excess hormones. Using medications to block hormones secreted by the tumor may reduce the signs and symptoms of carcinoid syndrome and slow tumor growth. Octreotide (Sandostatin, Bynfezia Pen) and lanreotide (Somatuline Depot) are given as injections under the skin. Side effects from either medication may include abdominal pain, bloating and diarrhea. Telotristat (Xermelo) is a pill that is sometimes used in combination with octreotide or lanreotide to further try to improve the symptoms of carcinoid syndrome.
  • #14 Carcinoid syndrome: update on the pathophysiology and treatment | Clinics
    https://www.elsevier.es/en-revista-clinics-22-articulo-carcinoid-syndrome-update-on-pathophysiology-S1807593222011310
    The main pillar of treatment for CS is the use of somatostatin analogues, such as octreotide and lanreotide. Approximately 80% of well-differentiated tumors express somatostatin receptors in the NET cell surface. Octreotide and lanreotide bind to somatostatin receptors and inhibit the secretion of several hormones and vasoactive substances, thus improving flushing and diarrhea symptoms in over 80% patients with CS. […] Unfortunately, all patients experience symptomatic progression of CS after a median of several months to years. In this scenario, several therapeutic options have been successfully tested and are discussed below. […] Telotristat ethyl was recently approved by the US Food and Drug Administration. Together with octreotide, telotristat reduces the frequency of diarrhea. […] An alternative to somatostatin analogues for the treatment of refractory diarrhea and flushing is interferon alpha, which, in retrospective studies, has been shown to provide symptom relief in up to 40-50% patients.
  • #15 Carcinoid Syndrome – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK448096/
    Carcinoid syndrome is a rare clinical condition caused by metastatic, well-differentiated neuroendocrine tumors, primarily originating in the midgut and associated with liver metastases. […] Management includes medications to control hormone secretion and surgical interventions to reduce tumor burden. […] Diagnosis is primarily achieved through measuring urinary 5-hydroxyindoleacetic acid (5-HIAA), supported by imaging for tumor localization and staging. Management depends on the primary tumor’s type, location, and extent, including somatostatin analogs (eg, octreotide, lanreotide) to control hormone secretion, peptide receptor radioligand therapy (PRRT), and surgical or liver-directed interventions to reduce tumor burden. […] Somatostatin analogs (SSA) are the cornerstone of medical management. Two commonly used analogs, octreotide and lanreotide, mimic the action of somatostatin, an inhibitory peptide hormone that regulates the release of gastrointestinal and endocrine hormones. These drugs inhibit the secretion of biogenic amines, thereby controlling symptoms such as flushing and diarrhea.
  • #16 Carcinoid Syndrome Treatment – NETRF
    https://netrf.org/old-for-patients/living-with-nets/carcinoid-syndrome/carcinoid-syndrome-treatment/
    How is carcinoid syndrome treated? There are a variety of carcinoid syndrome treatments, including somatostatin analogs and other medications, liver-directed therapies, surgery, and chemotherapy. […] Somatostatin analogs include Octreotide and Lanreotide. […] Both Octreotide and Lanreotide have similar effectiveness and provide symptom relief in 50% to 70% of people with carcinoid syndrome. […] Surgical treatments aim to remove or reduce the volume of NETs, causing carcinoid syndrome. Surgery is generally reserved for patients in which 90% of the tumor can be removed and who do not have compromised liver function or extensive liver metastases. […] If liver metastases can be surgically removed, it often leads to the improvement of carcinoid syndrome symptoms. […] For patients with heavy tumor burden and metastases, tumor debulking (removing large portions of the tumor) may improve symptoms as well as extend and improve the overall health of the patient.
  • #17 Malignant Carcinoid Syndrome Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/282515-treatment
    Complete surgical removal of all tumor tissues, when feasible, is the best treatment for carcinoid syndrome because it may result in a complete and permanent cure. Systemic medical therapy should be used to control humorally mediated symptoms when the cancer spreads elsewhere. […] Systemic therapy should be used to control humorally mediated symptoms when the cancer spreads elsewhere. Initially, somatostatin analogs (SSAs) are useful in approximately 40% of patients. […] The SSAs octreotide and lanreotide are used to control carcinoid symptoms and tumor progression in advanced inoperable disease. […] Octreotide LAR significantly extends the time to tumor progression compared with placebo for patients with midgut NETs. […] A randomized, placebo-controlled phase 3 RADIANT-2 trial found that everolimus plus octreotide LAR improved progression-free survival (PFS) in patients with advanced NETs associated with carcinoid syndrome.
  • #18 Malignant Carcinoid Syndrome Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/282515-treatment
    Complete surgical removal of all tumor tissues, when feasible, is the best treatment for carcinoid syndrome because it may result in a complete and permanent cure. Systemic medical therapy should be used to control humorally mediated symptoms when the cancer spreads elsewhere. […] Systemic therapy should be used to control humorally mediated symptoms when the cancer spreads elsewhere. Initially, somatostatin analogs (SSAs) are useful in approximately 40% of patients. […] The SSAs octreotide and lanreotide are used to control carcinoid symptoms and tumor progression in advanced inoperable disease. […] Octreotide LAR significantly extends the time to tumor progression compared with placebo for patients with midgut NETs. […] A randomized, placebo-controlled phase 3 RADIANT-2 trial found that everolimus plus octreotide LAR improved progression-free survival (PFS) in patients with advanced NETs associated with carcinoid syndrome.
  • #19 Drug therapy for neuroendocrine tumours (NETs) | Canadian Cancer Society
    https://cancer.ca/en/cancer-information/cancer-types/neuroendocrine-tumours/treatment/drug-therapy
    Drug therapy is commonly used to treat and manage neuroendocrine tumours (NETs). Drugs are given for different reasons. You may have drug therapy to: […] Somatostatin analogues are drugs that lower the amount of hormones made and released by NETs. They are mainly used to control symptoms of carcinoid syndrome. […] Octreotide LAR or lanreotide is standard treatment for the long-term control of symptoms and tumour growth. The drug is given once a month. It is usually taken for as long as it works. It may be taken for the rest of your life. […] The fast-acting form of octreotide (octreotide IR) is given several times a day. It is often used for a short time when the drug is first started to check that you can cope with it. It is also used before surgery or other treatments to prevent and manage carcinoid crisis. […] Supportive drugs may be used to manage or prevent problems caused by NETs. […] Telotristat ethyl (Xermelo) to treat carcinoid syndrome diarrhea, along with a somatostatin analogue, in people whose diarrhea is not controlled by somatostatin analogues alone.
  • #20 Drug therapy for neuroendocrine tumours (NETs) | Canadian Cancer Society
    https://cancer.ca/en/cancer-information/cancer-types/neuroendocrine-tumours/treatment/drug-therapy
    Drug therapy is commonly used to treat and manage neuroendocrine tumours (NETs). Drugs are given for different reasons. You may have drug therapy to: […] Somatostatin analogues are drugs that lower the amount of hormones made and released by NETs. They are mainly used to control symptoms of carcinoid syndrome. […] Octreotide LAR or lanreotide is standard treatment for the long-term control of symptoms and tumour growth. The drug is given once a month. It is usually taken for as long as it works. It may be taken for the rest of your life. […] The fast-acting form of octreotide (octreotide IR) is given several times a day. It is often used for a short time when the drug is first started to check that you can cope with it. It is also used before surgery or other treatments to prevent and manage carcinoid crisis. […] Supportive drugs may be used to manage or prevent problems caused by NETs. […] Telotristat ethyl (Xermelo) to treat carcinoid syndrome diarrhea, along with a somatostatin analogue, in people whose diarrhea is not controlled by somatostatin analogues alone.
  • #21 Carcinoid tumors – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/carcinoid-tumors/diagnosis-treatment/drc-20351044
    Treatment for a carcinoid tumor depends on the tumor’s location, whether cancer has spread to other areas of the body, the types of hormones the tumor secretes, your overall health and your own preferences. […] Carcinoid tumor treatment options may include: […] Surgery. When detected early, a carcinoid tumor may be removed completely using surgery. If carcinoid tumors are advanced when discovered, complete removal may not be possible. In some situations, surgeons may try to remove as much of the tumor as possible, to help control signs and symptoms. […] Medications to control excess hormones. Using medications to block hormones secreted by the tumor may reduce the signs and symptoms of carcinoid syndrome and slow tumor growth. Octreotide (Sandostatin, Bynfezia Pen) and lanreotide (Somatuline Depot) are given as injections under the skin. Side effects from either medication may include abdominal pain, bloating and diarrhea. Telotristat (Xermelo) is a pill that is sometimes used in combination with octreotide or lanreotide to further try to improve the symptoms of carcinoid syndrome.
  • #22 FF #137Carcinoid Syndrome: Symptom Management | Palliative Care Network of Wisconsin
    https://www.mypcnow.org/fast-fact/carcinoid-syndrome-symptom-management/
    Somatostatin Analogs These drugs are the treatment of choice for CS. Three formulations are available: short-acting octreotide (continuous infusion or 50-500 mcg TID, IV or subcutaneously); depot octreotide (standard dose is 30 mg intramuscularly every 4 weeks; however, doses up to 40 to 60 mg every 4 weeks may offer added benefit); and lanreotide (standard dosing is 30 mg every other week intramuscularly). 50-70% of patients experience a significant reduction of diarrhea and flushing episodes within seven days. Efficacy and side effect profiles for the three preparations are similar. Side effects include pain at the injection site, abdominal bloating, fatigue, transient fever, elevated serum glucose, and asymptomatic biliary lithiasis. Annual cost varies between $25,000-$60,000, but most insurances cover it.
  • #23 Carcinoid tumors – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/carcinoid-tumors/diagnosis-treatment/drc-20351044
    Treatment for a carcinoid tumor depends on the tumor’s location, whether cancer has spread to other areas of the body, the types of hormones the tumor secretes, your overall health and your own preferences. […] Carcinoid tumor treatment options may include: […] Surgery. When detected early, a carcinoid tumor may be removed completely using surgery. If carcinoid tumors are advanced when discovered, complete removal may not be possible. In some situations, surgeons may try to remove as much of the tumor as possible, to help control signs and symptoms. […] Medications to control excess hormones. Using medications to block hormones secreted by the tumor may reduce the signs and symptoms of carcinoid syndrome and slow tumor growth. Octreotide (Sandostatin, Bynfezia Pen) and lanreotide (Somatuline Depot) are given as injections under the skin. Side effects from either medication may include abdominal pain, bloating and diarrhea. Telotristat (Xermelo) is a pill that is sometimes used in combination with octreotide or lanreotide to further try to improve the symptoms of carcinoid syndrome.
  • #24 Malignant Carcinoid Syndrome Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/282515-treatment
    Telotristat ethyl (Xermelo) was approved by the FDA in 2017 for carcinoid syndrome diarrhea in combination with SSA therapy in adults inadequately controlled by an SSA. […] Patients benefit from specific drugs that either suppress production of vasoactive amines or block the peripheral effects. […] Interferon alfa has been used for NET symptom control but has a less favorable toxicity profile and is usually added as second-line therapy for refractory symptoms. […] Radiotherapy or chemotherapy with streptozotocin, cisplatin, etoposide, and doxorubicin, either alone or in combination, has been used, and reports show some success; a good response occurs in only 20-30% of cases. […] Complete surgical removal of all tumor tissues, when feasible, is the best treatment because it may result in a complete and permanent cure.
  • #25 Management of carcinoid syndrome: a systematic review and meta-analysis in: Endocrine-Related Cancer Volume 26 Issue 3 (2019)
    https://erc.bioscientifica.com/view/journals/erc/26/3/ERC-18-0495.xml
    Carcinoid syndrome (CS) is a debilitating disease caused by functional neuroendocrine tumors. Several treatment options are available to alleviate the hormonal symptoms, but their relative efficacy is unknown. […] The somatostatin analogs octreotide and lanreotide induced symptomatic improvement in 65-72% and biochemical response in 45-46% of patients. An increase in dose or frequency or interclass switch led to a reduction of flushes and/or diarrhea in 72-84% of cases. […] Retrospective, institutional series showed that liver-directed therapy can improve symptoms in 82% of CS patients with a liver-dominant disease. The serotonin synthesis inhibitor telotristat ethyl reduced bowel movements in 40% of patients with diarrhea refractory to somatostatin analogs. Interferon-alpha controlled CS symptoms in 45-63% of cases.
  • #26 FF #137Carcinoid Syndrome: Symptom Management | Palliative Care Network of Wisconsin
    https://www.mypcnow.org/fast-fact/carcinoid-syndrome-symptom-management/
    Interferon Interferon alpha is effective in controlling both diarrhea and flushing, although it is inferior to the somatostatin analogs. The dose is 3-9 mU subcutaneously three to seven times per week. Interferon alpha therapy is often limited by its side effects: fever, anemia, thrombocytopenia, neutropenia, fatigue, depression, and flu-like symptoms. […] Telotristat: this medication is an oral tryptophan hydroxylas inhibitor. It has been approved in the United States for the treatment of refractory diarrhea in adults with CS that is inadequately controlled by somatostatin therapy alone. The recommended dose is 250 mg three times a day with food. Nausea and depression are common side effects at higher doses. Annual cost averages around $90,000 but most insurances will cover it. […] Other symptom-specific treatments Diarrhea: Besides somatostatin analogs, interferon, and telostristat, cyproheptadine is an alternative treatment for carcinoid-associated diarrhea. 60% of patients report improvement within one week. The dosage is 4 mg TID given orally as a tablet; it can be titrated up to 0.5 mg/kg per day. Side effects include sedation, dry mouth, dizziness, mild blurring of vision, nausea, and vomiting. Loperamide and opioids are non-specific anti-diarrheal agents that can be used for mild symptoms. […] Other treatments for refractory symptoms include systemic chemotherapy, hepatic artery embolization, hepatic chemoembolization, or debulking surgery of hepatic metastases. Radiolabeled somatostatin analog 177-Lutetium Dotatate can be used for patients with somatostatin receptor expressing tumors.
  • #27 Malignant Carcinoid Syndrome Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/282515-treatment
    Complete surgical removal of all tumor tissues, when feasible, is the best treatment for carcinoid syndrome because it may result in a complete and permanent cure. Systemic medical therapy should be used to control humorally mediated symptoms when the cancer spreads elsewhere. […] Systemic therapy should be used to control humorally mediated symptoms when the cancer spreads elsewhere. Initially, somatostatin analogs (SSAs) are useful in approximately 40% of patients. […] The SSAs octreotide and lanreotide are used to control carcinoid symptoms and tumor progression in advanced inoperable disease. […] Octreotide LAR significantly extends the time to tumor progression compared with placebo for patients with midgut NETs. […] A randomized, placebo-controlled phase 3 RADIANT-2 trial found that everolimus plus octreotide LAR improved progression-free survival (PFS) in patients with advanced NETs associated with carcinoid syndrome.
  • #28 Exploring Carcinoid Syndrome in Neuroendocrine Tumors: Insights from a Multidisciplinary Narrative Review
    https://www.mdpi.com/2072-6694/16/22/3831
    Everolimus is an mTOR inhibitor, which blocks the mammalian target of the rapamycin (mTOR) pathway, which plays a key role in regulating cell growth, proliferation, metabolism, and survival. […] Peptide receptor radionuclide therapy (PRRT) with 177Lu-DOTATATE represents an efficient and safe systemic option among the wide possibilities to treat NET patients with positive somatostatin receptor imaging, and to improve symptoms in progressive disease. […] Chemotherapy is rarely applied in clinical practice because of its limited role in CS symptom control. Most data available are old and scarce, and are not descriptive of CS-specific outcomes, but, unfortunately, they are restricted to the biochemical response.
  • #29 Malignant Carcinoid Syndrome Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/282515-treatment
    Telotristat ethyl (Xermelo) was approved by the FDA in 2017 for carcinoid syndrome diarrhea in combination with SSA therapy in adults inadequately controlled by an SSA. […] Patients benefit from specific drugs that either suppress production of vasoactive amines or block the peripheral effects. […] Interferon alfa has been used for NET symptom control but has a less favorable toxicity profile and is usually added as second-line therapy for refractory symptoms. […] Radiotherapy or chemotherapy with streptozotocin, cisplatin, etoposide, and doxorubicin, either alone or in combination, has been used, and reports show some success; a good response occurs in only 20-30% of cases. […] Complete surgical removal of all tumor tissues, when feasible, is the best treatment because it may result in a complete and permanent cure.
  • #30 Management of carcinoid syndrome: a systematic review and meta-analysis in: Endocrine-Related Cancer Volume 26 Issue 3 (2019)
    https://erc.bioscientifica.com/view/journals/erc/26/3/ERC-18-0495.xml
    Carcinoid syndrome (CS) is a debilitating disease caused by functional neuroendocrine tumors. Several treatment options are available to alleviate the hormonal symptoms, but their relative efficacy is unknown. […] The somatostatin analogs octreotide and lanreotide induced symptomatic improvement in 65-72% and biochemical response in 45-46% of patients. An increase in dose or frequency or interclass switch led to a reduction of flushes and/or diarrhea in 72-84% of cases. […] Retrospective, institutional series showed that liver-directed therapy can improve symptoms in 82% of CS patients with a liver-dominant disease. The serotonin synthesis inhibitor telotristat ethyl reduced bowel movements in 40% of patients with diarrhea refractory to somatostatin analogs. Interferon-alpha controlled CS symptoms in 45-63% of cases.
  • #31 SciELO Brazil – Carcinoid syndrome: diagnosis and medical management Carcinoid syndrome: diagnosis and medical management
    https://www.scielo.br/j/abem/a/XsNGMwcFV8D6R6Lk5ZLR78j/?format=html
    Somatostatin analogs decrease the release of bioactive secreted products with effective resolution of flushing and diarrhea in between 70% and 80% of patients. […] Recombinant leukocyte interferon-a may be of some use in the treatment of disseminated carcinoid tumors and carcinoid syndrome. […] Supportive care of carcinoid tumors or carcinoid syndrome includes avoiding stress and conditions or substances that precipitate symptoms; dietary supplementation with nicotinamide is also recommended. […] In general, carcinoids are resistant to radiotherapy, although external beam therapy has been used for palliation of bone metastases and the management of spinal cord compression and brain metastases. […] The use of 131I-MIBG therapy can be considered early in an adjuvant setting, after surgery to eradicate occult disease, or later for treatment of disseminated disease.
  • #32 FF #137Carcinoid Syndrome: Symptom Management | Palliative Care Network of Wisconsin
    https://www.mypcnow.org/fast-fact/carcinoid-syndrome-symptom-management/
    Interferon Interferon alpha is effective in controlling both diarrhea and flushing, although it is inferior to the somatostatin analogs. The dose is 3-9 mU subcutaneously three to seven times per week. Interferon alpha therapy is often limited by its side effects: fever, anemia, thrombocytopenia, neutropenia, fatigue, depression, and flu-like symptoms. […] Telotristat: this medication is an oral tryptophan hydroxylas inhibitor. It has been approved in the United States for the treatment of refractory diarrhea in adults with CS that is inadequately controlled by somatostatin therapy alone. The recommended dose is 250 mg three times a day with food. Nausea and depression are common side effects at higher doses. Annual cost averages around $90,000 but most insurances will cover it. […] Other symptom-specific treatments Diarrhea: Besides somatostatin analogs, interferon, and telostristat, cyproheptadine is an alternative treatment for carcinoid-associated diarrhea. 60% of patients report improvement within one week. The dosage is 4 mg TID given orally as a tablet; it can be titrated up to 0.5 mg/kg per day. Side effects include sedation, dry mouth, dizziness, mild blurring of vision, nausea, and vomiting. Loperamide and opioids are non-specific anti-diarrheal agents that can be used for mild symptoms. […] Other treatments for refractory symptoms include systemic chemotherapy, hepatic artery embolization, hepatic chemoembolization, or debulking surgery of hepatic metastases. Radiolabeled somatostatin analog 177-Lutetium Dotatate can be used for patients with somatostatin receptor expressing tumors.
  • #33 Neuroendocrine Tumors – Diagnosis & Disease Information for HCPs
    https://www.cancertherapyadvisor.com/ddi/neuroendocrine-tumors/
    Patients with GI neuroendocrine tumors, particularly those with liver metastases, may be treated with interventional radiology techniques such as radiofrequency ablation or transarterial chemoembolization (TACE). These approaches help reduce tumor size and control symptoms. For certain patients, liver transplantation may be considered. When making treatment decisions, clinicians should conduct a comprehensive assessment of the tumors biology, the patients overall health, and the goal of care whether it is curative, palliative, or focused on symptom management. Regular monitoring with imaging and biomarker assessments is crucial to evaluate treatment efficacy and adjust the management plan as needed.
  • #34 Treatment Options – Carcinoid Cancer Foundation
    https://www.carcinoid.org/for-patients/treatment/treatment-options/
    Octreotide has largely replaced the use of other drugs both for symptomatic control and acute treatment of the symptoms associated with carcinoid syndrome. […] We suggest that hypertensive as well as hypotensive carcinoid crises respond to octreotide and that this agent should be considered for prophylactic and emergency use in all carcinoid syndrome patients prior to and during anesthesia and surgery. […] Hepatic Artery Embolization is safe, provides good control of hormonal symptoms, and prolongs survival in biochemically responsive patients. It is a valuable palliative option for patients with midgut carcinoid syndrome due to liver metastases and can be repeated in patients with a favourable response to the first procedure. […] High-dose 111In-Pentetreotide (~500 mCi/patient) is now offered in the U.S. for therapy in somatostatin receptor expressing neuroendocrine tumors. This therapy can be applied to the category of neuroendocrine tumors which include Carcinoid, Islet Cell Carcinoma of the Pancreas, Oat Cell Carcinoma of the Lung, and Medullary Thyroid Carcinoma.
  • #35 Management of carcinoid syndrome: a systematic review and meta-analysis in: Endocrine-Related Cancer Volume 26 Issue 3 (2019)
    https://erc.bioscientifica.com/view/journals/erc/26/3/ERC-18-0495.xml
    The combination of the different liver-directed modalities resulted in a symptomatic response in 393 out of 479 (82%) patients, whereas urinary 5-HIAA levels were significantly reduced in 116 out of 191 (61%) patients. […] Following first-line therapy with SSAs, individual patient characteristics are the basis for decision making on how to go for several second- or third-line options with efficacy proven to a reasonable degree.
  • #36 Carcinoid Syndrome | General Surgery
    https://generalsurgery.ucsf.edu/condition/carcinoid-syndrome
    Surgery may be performed to remove the primary tumor and debulk liver metastases in carefully selected patients after multidisciplinary evaluation. […] Somatostatin analog (Sandostatin, Lanreotide) injections may reduce the signs and symptoms of carcinoid syndrome, including skin flushing and diarrhea. […] Embolization is a therapy to treat liver tumors by blocking their blood supply. […] Radiofrequency ablation delivers heat through a needle to the metastatic cells in the liver, causing cell death. […] Chemotherapy drugs may shrink neuroendocrine tumors, in particular, those of pancreatic origin. […] Anti-diarrheal and anti-cholinergic medications are also used. Patients are advised to avoid alcohol and foods with a high concentration of tyramine, which may worsen symptoms.
  • #37 Carcinoid tumors – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/carcinoid-tumors/diagnosis-treatment/drc-20351044
    Treatment for cancer that spreads to the liver. Carcinoid tumors commonly spread to the liver. Treatments may include surgery to remove part of the liver, blocking blood flow to the liver (hepatic artery embolization), and using heat and cold to kill cancer cells. Radiofrequency ablation delivers heat treatments that cause carcinoid tumor cells in the liver to die. Cryoablation uses cycles of freezing and thawing to kill cancer cells.
  • #38 Carcinoid tumors – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/carcinoid-tumors/diagnosis-treatment/drc-20351044
    Treatment for cancer that spreads to the liver. Carcinoid tumors commonly spread to the liver. Treatments may include surgery to remove part of the liver, blocking blood flow to the liver (hepatic artery embolization), and using heat and cold to kill cancer cells. Radiofrequency ablation delivers heat treatments that cause carcinoid tumor cells in the liver to die. Cryoablation uses cycles of freezing and thawing to kill cancer cells.
  • #39 Carcinoid Tumor Treatment & Management: Medical Care, Surgical Care
    https://emedicine.medscape.com/article/986050-treatment
    When total resection is not possible, debulking may provide symptomatic relief. […] In selected cases, cryotherapy can be effective. Bronchoscopic cryotherapy has been successfully applied in treatment of isolated endoluminal carcinoid tumor in an adult patient. […] In patients with tumors less than 1 cm located in the appendix, appendectomy is the treatment of choice. More extensive surgery is indicated for tumors larger than 2 cm, lymphatic invasion, lymph node involvement, mesoappendix infiltration, positive resection margins, and cellular pleomorphism with a high mitotic index. For tumors larger than 2 cm, accepted treatment has been hemicolectomy; however, a survival advantage over simple appendectomy has not been demonstrated.
  • #40 The role of serotonin inhibition within the treatment of carcinoid syndrome in: Endocrine Oncology Volume 3 Issue 1 (2023)
    https://eo.bioscientifica.com/view/journals/eo/3/1/EO-22-0077.xml
    Peptide receptor radionuclide therapy (PRRT) is an effective form of radioligand therapy. […] Everolimus is a derivative of sirolimus (rapamycin) bearing a 2-hydroxyethyl chain at position 40. […] Interferon (IFN) was discovered in 1957 and was later identified as a group of related protein cytokines secreted by cells in response to stimulation by a virus or other foreign substance. […] Chemotherapy should be primarily reserved for patients with high proliferation tumours, except possibly for pancreatic primary sites Ki-67 of 10%, and widespread disease might support systemic chemotherapy as a first-line treatment.
  • #41 Exploring Carcinoid Syndrome in Neuroendocrine Tumors: Insights from a Multidisciplinary Narrative Review
    https://www.mdpi.com/2072-6694/16/22/3831
    Everolimus is an mTOR inhibitor, which blocks the mammalian target of the rapamycin (mTOR) pathway, which plays a key role in regulating cell growth, proliferation, metabolism, and survival. […] Peptide receptor radionuclide therapy (PRRT) with 177Lu-DOTATATE represents an efficient and safe systemic option among the wide possibilities to treat NET patients with positive somatostatin receptor imaging, and to improve symptoms in progressive disease. […] Chemotherapy is rarely applied in clinical practice because of its limited role in CS symptom control. Most data available are old and scarce, and are not descriptive of CS-specific outcomes, but, unfortunately, they are restricted to the biochemical response.
  • #42 Carcinoid Syndrome, Symptoms, Diagnosis, Treatment
    https://crinetics.com/carcinoid-syndrome-symptoms-diagnosis-treatment/
    Two of these somatostatin analogs, octreotide and lanreotide, bind to receptor types 2, 3, and 5. They are effective at inhibiting the release of serotonin and other humoral factors; therefore, they are mainstays of treatment. Flushing and diarrhea improve significantly in over 80% of patients undergoing this therapy. […] In cases where patients don’t respond to octreotide therapy, peptide receptor radioligand therapy (PRRT) may be performed. This is done by radio-labeling a somatostatin analog with 177-Lutetium (Lu), which delivers targeted radiation to somatostatin receptor-expressing tumors. Patients with carcinoid syndrome have demonstrated high symptomatic response rates with this therapy. […] Hepatic artery chemo-embolization or radio-embolization may be performed in some patients to, basically, starve the cancer cells of their blood supply.
  • #43 Carcinoid Syndrome, Symptoms, Diagnosis, Treatment
    https://crinetics.com/carcinoid-syndrome-symptoms-diagnosis-treatment/
    Two of these somatostatin analogs, octreotide and lanreotide, bind to receptor types 2, 3, and 5. They are effective at inhibiting the release of serotonin and other humoral factors; therefore, they are mainstays of treatment. Flushing and diarrhea improve significantly in over 80% of patients undergoing this therapy. […] In cases where patients don’t respond to octreotide therapy, peptide receptor radioligand therapy (PRRT) may be performed. This is done by radio-labeling a somatostatin analog with 177-Lutetium (Lu), which delivers targeted radiation to somatostatin receptor-expressing tumors. Patients with carcinoid syndrome have demonstrated high symptomatic response rates with this therapy. […] Hepatic artery chemo-embolization or radio-embolization may be performed in some patients to, basically, starve the cancer cells of their blood supply.
  • #44 Exploring Carcinoid Syndrome in Neuroendocrine Tumors: Insights from a Multidisciplinary Narrative Review
    https://www.mdpi.com/2072-6694/16/22/3831
    Everolimus is an mTOR inhibitor, which blocks the mammalian target of the rapamycin (mTOR) pathway, which plays a key role in regulating cell growth, proliferation, metabolism, and survival. […] Peptide receptor radionuclide therapy (PRRT) with 177Lu-DOTATATE represents an efficient and safe systemic option among the wide possibilities to treat NET patients with positive somatostatin receptor imaging, and to improve symptoms in progressive disease. […] Chemotherapy is rarely applied in clinical practice because of its limited role in CS symptom control. Most data available are old and scarce, and are not descriptive of CS-specific outcomes, but, unfortunately, they are restricted to the biochemical response.
  • #45
    https://www.healthshare.com.au/questions/43131-how-is-carcinoid-syndrome-treated/
    This is sometimes combined with chemotherapy or radioactive octreotide. […] Radioactive octreotide or octreotate is an injection that allows radioactivity to be delivered directly to carcinoids that take up the octreotide chemical. […] Not all carcinoids are suitable for this treatment. […] Octreotide can also be used without radioactivity to suppress the hormonal activity of carcinoids. […] A long-acting preparation called lanreotide can be used, or several injections of normal octreotide can be injected per day. […] This can be done at home, similar to insulin injections.
  • #46 Carcinoid tumors – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/carcinoid-tumors/diagnosis-treatment/drc-20351044
    Chemotherapy. Chemotherapy uses strong drugs to kill tumor cells. It can be given through a vein in your arm or taken as a pill. Chemotherapy is sometimes recommended for treating advanced carcinoid tumors that can’t be removed with surgery. […] Targeted drug therapy. Targeted drug treatments focus on specific abnormalities present within tumor cells. By blocking these abnormalities, targeted drug treatments can cause tumor cells to die. Targeted drug therapy is usually combined with chemotherapy for advanced carcinoid tumors. […] Drugs that deliver radiation directly to the cancer cells. Peptide receptor radionuclide therapy (PRRT) combines a drug that seeks out cancer cells with a radioactive substance that kills them. In PRRT for carcinoid tumors, the drug is injected into your body, where it travels to the cancer cells, binds to the cells and delivers the radiation directly to them. This therapy may be an option for people with advanced carcinoid tumors.
  • #47 Neuroendocrine Tumors – Diagnosis & Disease Information for HCPs
    https://www.cancertherapyadvisor.com/home/decision-support-in-medicine/hospital-medicine/carcinoid-syndrome
    Surgery is the mainstay of treatment for localized neuroendocrine tumors and may be curative if the tumor is completely resected. Even when complete resection is not possible, debulking surgery may help reduce tumor burden and alleviate symptoms, particularly in patients with functional tumors. […] Somatostatin analogs such as octreotide and lanreotide are the cornerstone of treatment for functional neuroendocrine tumors. These agents control symptoms by inhibiting hormone secretion and may also slow tumor growth. […] Chemotherapy is typically reserved for patients with high-grade neuroendocrine tumors or those unresponsive to other treatments. Regimens may include capecitabine, streptozocin, and temozolomide. Peptide receptor radionuclide therapy with lutetium Lu 177 dotatate is an option for patients with advanced, somatostatin receptor-positive neuroendocrine tumors, offering delivery of high doses of radiation directly to the tumor cells while sparing surrounding tissues.
  • #48 Treating Carcinoid Tumors: When Surgery Isn’t an Option
    https://www.webmd.com/cancer/features/treating-carcinoid-tumors
    Medications can help slow the growth of tumors and ease the symptoms of carcinoid syndrome. […] The drug most commonly used to treat carcinoid tumors is octreotide (Sandostatin). This drug is similar to a hormone that occurs naturally in the body. It can help relieve the flushing and diarrhea caused by carcinoid syndrome, and slow tumor growth. […] Interferon drugs can help reduce the symptoms of carcinoid syndrome and may help slow the growth of carcinoid tumors. […] Researchers are also looking at other drugs to help slow or stop the growth of carcinoid tumors. […] Ablation is a way to destroy or shrink the tumor by injecting it directly with a substance that will kill it. […] Chemotherapy uses drugs to help kill cancer cells. […] Unfortunately, chemotherapy doesn’t always work well against carcinoid tumors.
  • #49 Treating Carcinoid Tumors: When Surgery Isn’t an Option
    https://www.webmd.com/cancer/features/treating-carcinoid-tumors
    „Chemotherapy is usually only used for certain types of carcinoid tumors, such as those in the pancreas,” says Yao. […] Sometimes, however, doctors recommend chemotherapy when the tumors are causing severe symptoms, have spread, or other treatments have not worked. […] Radiation therapy is a treatment using high-energy rays to kill cancer cells. […] There are a number of new treatments that may also be helpful in treating carcinoid tumors. […] Researchers are also looking at a new type of radiation, called radiopharmaceuticals. […] In rare cases, an organ transplant may be an option when the carcinoid tumor has spread to the liver. […] „While liver transplant is certainly not standard treatment, it can be a benefit for certain patients,” says Yao. […] „We’ve developed many new treatments in the last 10 years,” says Warner. „So we’re now able to offer a wider variety of treatments for tumors that cannot be surgically removed.”
  • #50 Carcinoid Tumor Treatment & Management: Medical Care, Surgical Care
    https://emedicine.medscape.com/article/986050-treatment
    If metastasis of carcinoid tumor has occurred and in cases where surgical excision is not suitable, consider treatment with currently recommended chemotherapy. […] Chemotherapeutic agents currently used in clinical trials to palliate metastatic carcinoid disease include the following: Alkylating agents, Doxorubicin, 5-Fluorouracil, Dacarbazine, Actinomycin D, Cisplatin, Etoposide, Streptozotocin, Interferon alfa, Somatostatin analogs with a radioactive load, Experimental agents such as 177Lu-Dotatate. […] A combination of the agents listed above is typically used. […] Chemoembolization of hepatic artery for treatment of metastatic carcinoid tumor has been widely used in adults. […] In one study, 8 adults with carcinoid tumor metastatic to liver were treated with intra-arterial 5-fluorouracil and embolization of hepatic tumors with bovine collagen fiber admixed with iohexol, cisplatin, mitomycin C, and doxorubicin. This treatment resulted in symptomatic relief and tumor regression in 4 patients and stabilized the disease in the rest of the patients.
  • #51 Exploring Carcinoid Syndrome in Neuroendocrine Tumors: Insights from a Multidisciplinary Narrative Review
    https://www.mdpi.com/2072-6694/16/22/3831
    Everolimus is an mTOR inhibitor, which blocks the mammalian target of the rapamycin (mTOR) pathway, which plays a key role in regulating cell growth, proliferation, metabolism, and survival. […] Peptide receptor radionuclide therapy (PRRT) with 177Lu-DOTATATE represents an efficient and safe systemic option among the wide possibilities to treat NET patients with positive somatostatin receptor imaging, and to improve symptoms in progressive disease. […] Chemotherapy is rarely applied in clinical practice because of its limited role in CS symptom control. Most data available are old and scarce, and are not descriptive of CS-specific outcomes, but, unfortunately, they are restricted to the biochemical response.
  • #52 Carcinoid tumors – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/carcinoid-tumors/diagnosis-treatment/drc-20351044
    Chemotherapy. Chemotherapy uses strong drugs to kill tumor cells. It can be given through a vein in your arm or taken as a pill. Chemotherapy is sometimes recommended for treating advanced carcinoid tumors that can’t be removed with surgery. […] Targeted drug therapy. Targeted drug treatments focus on specific abnormalities present within tumor cells. By blocking these abnormalities, targeted drug treatments can cause tumor cells to die. Targeted drug therapy is usually combined with chemotherapy for advanced carcinoid tumors. […] Drugs that deliver radiation directly to the cancer cells. Peptide receptor radionuclide therapy (PRRT) combines a drug that seeks out cancer cells with a radioactive substance that kills them. In PRRT for carcinoid tumors, the drug is injected into your body, where it travels to the cancer cells, binds to the cells and delivers the radiation directly to them. This therapy may be an option for people with advanced carcinoid tumors.
  • #53 Neuroendocrine Tumors – Diagnosis & Disease Information for HCPs
    https://www.cancertherapyadvisor.com/home/decision-support-in-medicine/hospital-medicine/carcinoid-syndrome
    Targeted therapies used to treat neuroendocrine tumors include the mammalian target of rapamycin (mTOR) kinase inhibitor everolimus and the tyrosine kinase inhibitors sunitinib. These medications block or inhibit the action of molecules that signal cells to grow or divide. […] Patients with GI neuroendocrine tumors, particularly those with liver metastases, may be treated with interventional radiology techniques such as radiofrequency ablation or transarterial chemoembolization (TACE). These approaches help reduce tumor size and control symptoms. For certain patients, liver transplantation may be considered. […] When making treatment decisions, clinicians should conduct a comprehensive assessment of the tumors biology, the patients overall health, and the goal of care whether it is curative, palliative, or focused on symptom management. Regular monitoring with imaging and biomarker assessments is crucial to evaluate treatment efficacy and adjust the management plan as needed.
  • #54 Malignant Carcinoid Syndrome Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/282515-treatment
    Complete surgical removal of all tumor tissues, when feasible, is the best treatment for carcinoid syndrome because it may result in a complete and permanent cure. Systemic medical therapy should be used to control humorally mediated symptoms when the cancer spreads elsewhere. […] Systemic therapy should be used to control humorally mediated symptoms when the cancer spreads elsewhere. Initially, somatostatin analogs (SSAs) are useful in approximately 40% of patients. […] The SSAs octreotide and lanreotide are used to control carcinoid symptoms and tumor progression in advanced inoperable disease. […] Octreotide LAR significantly extends the time to tumor progression compared with placebo for patients with midgut NETs. […] A randomized, placebo-controlled phase 3 RADIANT-2 trial found that everolimus plus octreotide LAR improved progression-free survival (PFS) in patients with advanced NETs associated with carcinoid syndrome.
  • #55 Carcinoid Syndrome – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK448096/
    Carcinoid crisis is a potentially life-threatening complication characterized by severe hemodynamic instability, flushing, bronchoconstriction, and hypotension, often triggered by surgery, anesthesia, or tumor necrosis. It typically occurs in patients with high levels of circulating vasoactive peptides, eg, serotonin. Prophylactic administration of octreotide is recommended in patients not on SSA as part of treatment.
  • #56 Carcinoid Crisis – NETRF
    https://netrf.org/old-for-patients/living-with-nets/carcinoid-syndrome/carcinoid-crisis/
    To protect against a carcinoid crisis during surgery, a patient should be treated with a somatostatin analog, such as Octreotide, either before and/or during the procedure. […] Some health care providers may not be aware of the threat of a carcinoid crisis and how to protect against it. Therefore, it is important to talk to all health care providers about a carcinoid syndrome diagnosis to educate them about the risk of carcinoid crisis. […] Pellagra is thought to occur as an indirect result of abnormally high levels of serotonin being produced by the body. […] Scar tissue may form in the mesentery of patients with carcinoid syndrome, and this scar tissue may cause problems in the intestine. […] Carcinoid heart disease is the collective term used to describe all heart problems associated with people who have carcinoid syndrome.
  • #57 Carcinoid Syndrome – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK448096/
    Carcinoid crisis is a potentially life-threatening complication characterized by severe hemodynamic instability, flushing, bronchoconstriction, and hypotension, often triggered by surgery, anesthesia, or tumor necrosis. It typically occurs in patients with high levels of circulating vasoactive peptides, eg, serotonin. Prophylactic administration of octreotide is recommended in patients not on SSA as part of treatment.
  • #58 Carcinoid Crisis – NETRF
    https://netrf.org/old-for-patients/living-with-nets/carcinoid-syndrome/carcinoid-crisis/
    To protect against a carcinoid crisis during surgery, a patient should be treated with a somatostatin analog, such as Octreotide, either before and/or during the procedure. […] Some health care providers may not be aware of the threat of a carcinoid crisis and how to protect against it. Therefore, it is important to talk to all health care providers about a carcinoid syndrome diagnosis to educate them about the risk of carcinoid crisis. […] Pellagra is thought to occur as an indirect result of abnormally high levels of serotonin being produced by the body. […] Scar tissue may form in the mesentery of patients with carcinoid syndrome, and this scar tissue may cause problems in the intestine. […] Carcinoid heart disease is the collective term used to describe all heart problems associated with people who have carcinoid syndrome.
  • #59 Carcinoid Crisis and Anaesthesia: Signs, Risks, Triggers & More
    https://neuroendocrine.org.au/treatments/carcinoid-crisis-and-anaesthesia/
    People who are already taking long-acting octreotide treatments like Sandostatin LAR or Lanreotide might need higher doses of octreotide during surgery. This might be because their bodies are getting used to the drug over time (called tachyphylaxis), so higher amounts are needed to get the same effect. […] Octreotide continue infusion at 100-1000gm/hr with boluses when indicated […] Bolus doses of octreotide 100-1000gm […] Continue octreotide infusion and wean over days.
  • #60 Carcinoid syndrome in neuroendocrine tumours (NETs)| Cancer Research UK
    https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/carcinoid-syndrome/complications
    To treat carcinoid heart disease your doctor might suggest: medicines for treating heart conditions; changes to your diet – for example being careful about fluid and salt intake; surgery to replace affected heart valves with an artificial valve (prosthesis). […] To prevent carcinoid crisis, you might have a somatostatin analogue called octreotide. You can have it as an injection, or as a continuous drip into your vein. […] Doctors monitor you closely for carcinoid crisis. If you develop it, you may have treatment with: somatostatin analogues; steroids; fluids into your vein (intravenous); some types of drug which can control your blood pressure. […] Before, during and after surgery, you might need to have a somatostatin analogue called octreotide as a continuous drip into a vein to prevent carcinoid crisis.
  • #61 Carcinoid syndrome in neuroendocrine tumours (NETs)| Cancer Research UK
    https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/carcinoid-syndrome/complications
    To treat carcinoid heart disease your doctor might suggest: medicines for treating heart conditions; changes to your diet – for example being careful about fluid and salt intake; surgery to replace affected heart valves with an artificial valve (prosthesis). […] To prevent carcinoid crisis, you might have a somatostatin analogue called octreotide. You can have it as an injection, or as a continuous drip into your vein. […] Doctors monitor you closely for carcinoid crisis. If you develop it, you may have treatment with: somatostatin analogues; steroids; fluids into your vein (intravenous); some types of drug which can control your blood pressure. […] Before, during and after surgery, you might need to have a somatostatin analogue called octreotide as a continuous drip into a vein to prevent carcinoid crisis.
  • #62 Carcinoid Crisis – NETRF
    https://netrf.org/old-for-patients/living-with-nets/carcinoid-syndrome/carcinoid-crisis/
    To protect against a carcinoid crisis during surgery, a patient should be treated with a somatostatin analog, such as Octreotide, either before and/or during the procedure. […] Some health care providers may not be aware of the threat of a carcinoid crisis and how to protect against it. Therefore, it is important to talk to all health care providers about a carcinoid syndrome diagnosis to educate them about the risk of carcinoid crisis. […] Pellagra is thought to occur as an indirect result of abnormally high levels of serotonin being produced by the body. […] Scar tissue may form in the mesentery of patients with carcinoid syndrome, and this scar tissue may cause problems in the intestine. […] Carcinoid heart disease is the collective term used to describe all heart problems associated with people who have carcinoid syndrome.
  • #63 Carcinoid syndrome in neuroendocrine tumours (NETs)| Cancer Research UK
    https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/carcinoid-syndrome/complications
    To treat carcinoid heart disease your doctor might suggest: medicines for treating heart conditions; changes to your diet – for example being careful about fluid and salt intake; surgery to replace affected heart valves with an artificial valve (prosthesis). […] To prevent carcinoid crisis, you might have a somatostatin analogue called octreotide. You can have it as an injection, or as a continuous drip into your vein. […] Doctors monitor you closely for carcinoid crisis. If you develop it, you may have treatment with: somatostatin analogues; steroids; fluids into your vein (intravenous); some types of drug which can control your blood pressure. […] Before, during and after surgery, you might need to have a somatostatin analogue called octreotide as a continuous drip into a vein to prevent carcinoid crisis.
  • #64 Carcinoid syndrome: update on the pathophysiology and treatment | Clinics
    https://www.elsevier.es/en-revista-clinics-22-articulo-carcinoid-syndrome-update-on-pathophysiology-S1807593222011310
    Because telotristat ethyl reduces the levels of 5-HIAA in 24-h urine tests, it is may be a promising treatment to prevent or delay the onset of CHD. […] However, once present, there is no specific treatment to alleviate the symptoms associated with this CS complication. However, its development can be prevented by resection of the primary midgut 5-HT-producing NET, and this is recommended by guidelines.
  • #65 Carcinoid Crisis – NETRF
    https://netrf.org/old-for-patients/living-with-nets/carcinoid-syndrome/carcinoid-crisis/
    To protect against a carcinoid crisis during surgery, a patient should be treated with a somatostatin analog, such as Octreotide, either before and/or during the procedure. […] Some health care providers may not be aware of the threat of a carcinoid crisis and how to protect against it. Therefore, it is important to talk to all health care providers about a carcinoid syndrome diagnosis to educate them about the risk of carcinoid crisis. […] Pellagra is thought to occur as an indirect result of abnormally high levels of serotonin being produced by the body. […] Scar tissue may form in the mesentery of patients with carcinoid syndrome, and this scar tissue may cause problems in the intestine. […] Carcinoid heart disease is the collective term used to describe all heart problems associated with people who have carcinoid syndrome.
  • #66 Treatment Options – Carcinoid Cancer Foundation
    https://www.carcinoid.org/for-patients/treatment/treatment-options/
    Hope for the patients with carcinoid syndrome: A diagnostic tool to help neuroendocrine cancer patients […] It has been shown that giving niacin supplementation to neuroendocrine cancer patients not only resolves several common symptoms of carcinoid/neuroendocrine tumors(NETs) and pellagra, such as skin lesions and diarrhea/ steatorrhea, but also generally improves the health of the NET patients. Therefore, our results warrant that niacin status should be determined for all NET patients, so that active niacin replacement could be provided to biochemically niacin-deficient patients. In areas of the world where preformed niacin is not added to the food supply and screening of niacin status is not possible, all carcinoid patients should be supplemented with niacin as a preventative therapy.
  • #67 Carcinoid Crisis – NETRF
    https://netrf.org/old-for-patients/living-with-nets/carcinoid-syndrome/carcinoid-crisis/
    To protect against a carcinoid crisis during surgery, a patient should be treated with a somatostatin analog, such as Octreotide, either before and/or during the procedure. […] Some health care providers may not be aware of the threat of a carcinoid crisis and how to protect against it. Therefore, it is important to talk to all health care providers about a carcinoid syndrome diagnosis to educate them about the risk of carcinoid crisis. […] Pellagra is thought to occur as an indirect result of abnormally high levels of serotonin being produced by the body. […] Scar tissue may form in the mesentery of patients with carcinoid syndrome, and this scar tissue may cause problems in the intestine. […] Carcinoid heart disease is the collective term used to describe all heart problems associated with people who have carcinoid syndrome.
  • #68 Carcinoid Tumor: Treatment, Symptoms, Meaning
    https://www.medicinenet.com/carcinoid_syndrome/article.htm
    Patients with carcinoid syndrome should take vitamin supplements, especially nicotinic acid since carcinoid tumors can cause a deficiency of nicotinic acid. In some patients, diarrhea caused by the carcinoid syndrome may respond to Imodium, Lomotil, ondansetron (Zofran), or cyproheptadine (Periactin). Patients also should avoid alcohol, spicy foods, physical stress, and ephedrine-containing medications such as nasal decongestants to avoid the precipitation of carcinoid syndrome by the release of hormones and chemical substances from the tumor.
  • #69 What is Carcinoid Syndrome, and How Can Functional Medicine Help?
    https://www.rupahealth.com/post/what-is-carcinoid-syndrome-and-how-can-functional-medicine-help
    Certain dietary and lifestyle habits may worsen or precipitate the symptoms of carcinoid syndrome. Therefore, nutrition and lifestyle approaches can help manage hormonal imbalances and support the immune system. […] A diet for carcinoid syndrome is usually made up of several smaller meals that are rich in proteins like nuts, beans, lentils, and legumes and do not have too much fat at one time since large fatty meals can worsen symptoms. […] People with carcinoid syndrome should avoid alcohol, which can trigger flushing and other symptoms since it also contains amines. […] Integrating treatments for carcinoid syndrome by bringing together both conventional and functional medicine strategies allows for the most effective and comprehensive care.
  • #70 What is Carcinoid Syndrome, and How Can Functional Medicine Help?
    https://www.rupahealth.com/post/what-is-carcinoid-syndrome-and-how-can-functional-medicine-help
    Certain dietary and lifestyle habits may worsen or precipitate the symptoms of carcinoid syndrome. Therefore, nutrition and lifestyle approaches can help manage hormonal imbalances and support the immune system. […] A diet for carcinoid syndrome is usually made up of several smaller meals that are rich in proteins like nuts, beans, lentils, and legumes and do not have too much fat at one time since large fatty meals can worsen symptoms. […] People with carcinoid syndrome should avoid alcohol, which can trigger flushing and other symptoms since it also contains amines. […] Integrating treatments for carcinoid syndrome by bringing together both conventional and functional medicine strategies allows for the most effective and comprehensive care.
  • #71 What is Carcinoid Syndrome, and How Can Functional Medicine Help?
    https://www.rupahealth.com/post/what-is-carcinoid-syndrome-and-how-can-functional-medicine-help
    Certain dietary and lifestyle habits may worsen or precipitate the symptoms of carcinoid syndrome. Therefore, nutrition and lifestyle approaches can help manage hormonal imbalances and support the immune system. […] A diet for carcinoid syndrome is usually made up of several smaller meals that are rich in proteins like nuts, beans, lentils, and legumes and do not have too much fat at one time since large fatty meals can worsen symptoms. […] People with carcinoid syndrome should avoid alcohol, which can trigger flushing and other symptoms since it also contains amines. […] Integrating treatments for carcinoid syndrome by bringing together both conventional and functional medicine strategies allows for the most effective and comprehensive care.
  • #72 Carcinoid Cancer: Definition, Symptoms, Prognosis, and More
    https://www.verywellhealth.com/carcinoid-tumor-5205919
    Surgery is typically the first-choice treatment for carcinoid cancer, with the goal of removing the entire tumor, if possible, and debulking (removing as much as possible) metastases (spread of cancer cells). […] Treatments for carcinoid syndrome may include: hormone therapy with a somatostatin analog, octreotide or lanreotide, interferon therapy, and medications for specific symptoms. […] People with carcinoid syndrome may find it helpful to avoid things that may cause flushing or increasing breathing difficulty, such as alcohol, nuts, certain cheeses, and foods with capsaicin (such as chili peppers). […] Avoiding stressful situations and engaging in certain types of physical activity may also help with carcinoid syndrome. […] Metastatic carcinoid cancer treatments may include: somatostatin analogs, which may help stop the body from producing too many hormones and may slow the growth of the tumor when cancer cells have metastasized, and targeted therapy, which uses drugs that target certain genes or proteins to kill cancer cells. […] Chemotherapy medicines may be injected into the liver. […] Debulking of liver metastases may be done through procedures such as surgical excision, cryoablation, radiofrequency ablation, and hepatic artery catheterization.
  • #73 Carcinoid Cancer: Definition, Symptoms, Prognosis, and More
    https://www.verywellhealth.com/carcinoid-tumor-5205919
    Surgery is typically the first-choice treatment for carcinoid cancer, with the goal of removing the entire tumor, if possible, and debulking (removing as much as possible) metastases (spread of cancer cells). […] Treatments for carcinoid syndrome may include: hormone therapy with a somatostatin analog, octreotide or lanreotide, interferon therapy, and medications for specific symptoms. […] People with carcinoid syndrome may find it helpful to avoid things that may cause flushing or increasing breathing difficulty, such as alcohol, nuts, certain cheeses, and foods with capsaicin (such as chili peppers). […] Avoiding stressful situations and engaging in certain types of physical activity may also help with carcinoid syndrome. […] Metastatic carcinoid cancer treatments may include: somatostatin analogs, which may help stop the body from producing too many hormones and may slow the growth of the tumor when cancer cells have metastasized, and targeted therapy, which uses drugs that target certain genes or proteins to kill cancer cells. […] Chemotherapy medicines may be injected into the liver. […] Debulking of liver metastases may be done through procedures such as surgical excision, cryoablation, radiofrequency ablation, and hepatic artery catheterization.
  • #74 What is Carcinoid Syndrome, and How Can Functional Medicine Help?
    https://www.rupahealth.com/post/what-is-carcinoid-syndrome-and-how-can-functional-medicine-help
    In addition to targeting and trying to reduce the neuroendocrine tumor(s) resulting in carcinoid syndrome, conventional treatments for carcinoid syndrome also aim to reduce and manage symptoms. For example, the oral tryptophan hydroxylase inhibitor telotristat can help reduce diarrhea when used in combination with somatostatin analogs. […] Functional medicine for carcinoid syndrome looks at the factors causing symptoms and addresses all parts of a persons health. This patient-centered and holistic approach to neuroendocrine tumors allows for a personalized look at factors that impact an individuals mind, body, and spiritual health. […] Regularly utilizing stress management practices like breath work can help calm the nervous system and activate your parasympathetic response. […] If you have pancreatic exocrine insufficiency, pancreatic enzyme replacement therapy taken before and during eating can help break down fats.
  • #75 What is Carcinoid Syndrome, and How Can Functional Medicine Help?
    https://www.rupahealth.com/post/what-is-carcinoid-syndrome-and-how-can-functional-medicine-help
    Certain dietary and lifestyle habits may worsen or precipitate the symptoms of carcinoid syndrome. Therefore, nutrition and lifestyle approaches can help manage hormonal imbalances and support the immune system. […] A diet for carcinoid syndrome is usually made up of several smaller meals that are rich in proteins like nuts, beans, lentils, and legumes and do not have too much fat at one time since large fatty meals can worsen symptoms. […] People with carcinoid syndrome should avoid alcohol, which can trigger flushing and other symptoms since it also contains amines. […] Integrating treatments for carcinoid syndrome by bringing together both conventional and functional medicine strategies allows for the most effective and comprehensive care.
  • #76 Management of carcinoid syndrome: a systematic review and meta-analysis in: Endocrine-Related Cancer Volume 26 Issue 3 (2019)
    https://erc.bioscientifica.com/view/journals/erc/26/3/ERC-18-0495.xml
    The combination of the different liver-directed modalities resulted in a symptomatic response in 393 out of 479 (82%) patients, whereas urinary 5-HIAA levels were significantly reduced in 116 out of 191 (61%) patients. […] Following first-line therapy with SSAs, individual patient characteristics are the basis for decision making on how to go for several second- or third-line options with efficacy proven to a reasonable degree.
  • #77
    https://link.springer.com/article/10.1007/s11864-019-0671-0
    In the clinical setting, the treatment of CS is guided in conjunction with patients performance status, tumor origin, grade, stage, and growth rate, with regard to both anti-hormonal, as well as anti-proliferative effect. […] There is an unmet need for further well-designed randomized placebo-controlled and head-to-head studies that systematically assess CS symptom control and biochemical response following a specific intervention. […] Management of carcinoid syndrome: a systematic review and meta-analysis. Endocr Relat Cancer. 2019;1 A literature review and meta-analysis regarding pharmacological therapies in CS, focusing on treatment efficacy in terms of symptomatic and biochemical response. […] Clinical benefits of above-standard dose of octreotide LAR in patients with neuroendocrine tumors for control of carcinoid syndrome symptoms: a multicenter retrospective chart review study.
  • #78 Treating Carcinoid Tumors: When Surgery Isn’t an Option
    https://www.webmd.com/cancer/features/treating-carcinoid-tumors
    „Chemotherapy is usually only used for certain types of carcinoid tumors, such as those in the pancreas,” says Yao. […] Sometimes, however, doctors recommend chemotherapy when the tumors are causing severe symptoms, have spread, or other treatments have not worked. […] Radiation therapy is a treatment using high-energy rays to kill cancer cells. […] There are a number of new treatments that may also be helpful in treating carcinoid tumors. […] Researchers are also looking at a new type of radiation, called radiopharmaceuticals. […] In rare cases, an organ transplant may be an option when the carcinoid tumor has spread to the liver. […] „While liver transplant is certainly not standard treatment, it can be a benefit for certain patients,” says Yao. […] „We’ve developed many new treatments in the last 10 years,” says Warner. „So we’re now able to offer a wider variety of treatments for tumors that cannot be surgically removed.”
  • #79 Carcinoid Syndrome, Symptoms, Diagnosis, Treatment
    https://crinetics.com/carcinoid-syndrome-symptoms-diagnosis-treatment/
    Another medication, telotristat, stops the metabolism of tryptophan into serotonin and can help reduce diarrhea. It is prescribed in addition to somatostatin analog therapy. […] Depending on the patient’s response, a combination of therapies may be used. A number of different side effects are possible. Please refer to product labeling for a complete list of side effects associated with any of these therapies. […] Crinetics is developing paltusotine for the treatment of NETs and carcinoid syndrome. Paltusotine establishes a new class of oral, selective, nonpeptide, somatostatin receptor type 2 (SST2) agonists. This once-daily oral investigational treatment is the first agent in its class with reported clinical trial results for the treatment of acromegaly.
  • #80
    https://link.springer.com/article/10.1007/s11864-019-0671-0
    In the clinical setting, the treatment of CS is guided in conjunction with patients performance status, tumor origin, grade, stage, and growth rate, with regard to both anti-hormonal, as well as anti-proliferative effect. […] There is an unmet need for further well-designed randomized placebo-controlled and head-to-head studies that systematically assess CS symptom control and biochemical response following a specific intervention. […] Management of carcinoid syndrome: a systematic review and meta-analysis. Endocr Relat Cancer. 2019;1 A literature review and meta-analysis regarding pharmacological therapies in CS, focusing on treatment efficacy in terms of symptomatic and biochemical response. […] Clinical benefits of above-standard dose of octreotide LAR in patients with neuroendocrine tumors for control of carcinoid syndrome symptoms: a multicenter retrospective chart review study.
  • #81 Carcinoid tumors – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/carcinoid-tumors/diagnosis-treatment/drc-20351044
    Treatment for a carcinoid tumor depends on the tumor’s location, whether cancer has spread to other areas of the body, the types of hormones the tumor secretes, your overall health and your own preferences. […] Carcinoid tumor treatment options may include: […] Surgery. When detected early, a carcinoid tumor may be removed completely using surgery. If carcinoid tumors are advanced when discovered, complete removal may not be possible. In some situations, surgeons may try to remove as much of the tumor as possible, to help control signs and symptoms. […] Medications to control excess hormones. Using medications to block hormones secreted by the tumor may reduce the signs and symptoms of carcinoid syndrome and slow tumor growth. Octreotide (Sandostatin, Bynfezia Pen) and lanreotide (Somatuline Depot) are given as injections under the skin. Side effects from either medication may include abdominal pain, bloating and diarrhea. Telotristat (Xermelo) is a pill that is sometimes used in combination with octreotide or lanreotide to further try to improve the symptoms of carcinoid syndrome.
  • #82 Carcinoid syndrome: update on the pathophysiology and treatment | Clinics
    https://www.elsevier.es/en-revista-clinics-22-articulo-carcinoid-syndrome-update-on-pathophysiology-S1807593222011310
    Approximately 30-40% of patients with well-differentiated neuroendocrine tumors present with carcinoid syndrome, which is a paraneoplastic syndrome associated with the secretion of several humoral factors. […] For several decades, patients with neuroendocrine tumors and carcinoid syndrome have been treated with somatostatin analogues as the first-line treatment. While these agents provide significant relief from carcinoid syndrome symptoms, there is inevitable clinical progression, and new therapeutic interventions are needed. […] Given the complexity involving the origin, diagnosis and management of patients with carcinoid syndrome, we have undertaken a comprehensive review to update information about the pathophysiology, diagnostic tools and treatment sequence of this syndrome, which currently comprises a multidisciplinary approach.
  • #83
    https://link.springer.com/article/10.1007/s11864-019-0671-0
    Carcinoid syndrome (CS) is a complex disorder caused by functional neuroendocrine tumors (NETs). This debilitating disease is characterized by hyper-secretion of biologically active substances eliciting major hormonal symptoms burden and fibrotic changes that are often challenging for management. […] There have been a number of insights that have substantially advanced treatments since the introduction of somatostatin analogs (SSAs). Second-line treatments are needed in a substantial proportion of patients with advanced disease that have uncontrolled hormone secretion on the highest labeled doses of SSAs. International guidelines suggest several available options including dose escalation of SSAs, interferon alpha, everolimus, radionuclide therapy, liver-directed therapies, and the novel tryptophan hydroxylase 1 inhibitor, telotristat ethyl.
  • #84 Carcinoid syndrome: update on the pathophysiology and treatment | Clinics
    https://www.elsevier.es/en-revista-clinics-22-articulo-carcinoid-syndrome-update-on-pathophysiology-S1807593222011310
    The main pillar of treatment for CS is the use of somatostatin analogues, such as octreotide and lanreotide. Approximately 80% of well-differentiated tumors express somatostatin receptors in the NET cell surface. Octreotide and lanreotide bind to somatostatin receptors and inhibit the secretion of several hormones and vasoactive substances, thus improving flushing and diarrhea symptoms in over 80% patients with CS. […] Unfortunately, all patients experience symptomatic progression of CS after a median of several months to years. In this scenario, several therapeutic options have been successfully tested and are discussed below. […] Telotristat ethyl was recently approved by the US Food and Drug Administration. Together with octreotide, telotristat reduces the frequency of diarrhea. […] An alternative to somatostatin analogues for the treatment of refractory diarrhea and flushing is interferon alpha, which, in retrospective studies, has been shown to provide symptom relief in up to 40-50% patients.
  • #85 What is Carcinoid Syndrome, and How Can Functional Medicine Help?
    https://www.rupahealth.com/post/what-is-carcinoid-syndrome-and-how-can-functional-medicine-help
    Certain dietary and lifestyle habits may worsen or precipitate the symptoms of carcinoid syndrome. Therefore, nutrition and lifestyle approaches can help manage hormonal imbalances and support the immune system. […] A diet for carcinoid syndrome is usually made up of several smaller meals that are rich in proteins like nuts, beans, lentils, and legumes and do not have too much fat at one time since large fatty meals can worsen symptoms. […] People with carcinoid syndrome should avoid alcohol, which can trigger flushing and other symptoms since it also contains amines. […] Integrating treatments for carcinoid syndrome by bringing together both conventional and functional medicine strategies allows for the most effective and comprehensive care.