Zespół rakowiczy – Rokowania, prognozy i postęp choroby

Zespół rakowiczy
Rokowania, prognozy i postęp choroby

Rokowanie u pacjentów z zespołem rakowiczym jest relatywnie korzystne w porównaniu z innymi nowotworami złośliwymi, z medianą 5-letniego przeżycia wynoszącą 30-67%. Obecność zespołu rakowiczego, zwłaszcza w przebiegu choroby serca (CHD), stanowi negatywny czynnik prognostyczny, z 3-letnim wskaźnikiem przeżycia na poziomie 31% w porównaniu do 68% u pacjentów bez zajęcia serca. Wiek ≥50 lat, przerzuty do wątroby lub węzłów chłonnych oraz płeć męska są niezależnymi czynnikami ryzyka zgonu. Przerzuty korelują z wielkością guza pierwotnego: guzy <1 cm dają przerzuty w 2%, 1-2 cm w 50%, a >2 cm w 85% przypadków. Lokalizacja przerzutów obejmuje głównie węzły chłonne i wątrobę, rzadziej kości i płuca. Leczenie chirurgiczne guzów przewodu pokarmowego wiąże się z 5-letnim przeżyciem na poziomie 83%, a agresywna cytoredukcja przerzutów wątrobowych może wydłużyć medianę przeżycia do ponad 100 miesięcy.

Zespół rakowiczy – Rokowanie (prognozowanie wyniku)

Czynniki prognostyczne wpływające na przeżycie
Wpływ przerzutów na rokowanie
Wskaźniki przeżycia w zależności od lokalizacji i stadium guza
Inne czynniki prognostyczne
Wpływ wczesnej diagnozy i leczenia na przeżycie
Podsumowanie czynników prognostycznych
Kolejne rozdziały

Zespół rakowiczy – Rokowanie (prognozowanie wyniku)

Rokowanie u pacjentów z zespołem rakowiczym jest stosunkowo dobre w porównaniu z rokowaniem w przypadku innych nowotworów złośliwych, choć prognoza dla każdego pacjenta z postępującą, nawracającą lub nawrotową chorobą jest niekorzystna. Mediana 5-letniego wskaźnika przeżycia wynosi 30-67%. Odnotowano przypadki przeżycia nawet do 23 lat¹. Warto jednak pamiętać, że wskaźniki przeżycia są jedynie szacunkami opartymi na wcześniejszych wynikach dużej liczby osób z określonym typem nowotworu i nie mogą przewidzieć, co stanie się w konkretnym przypadku².

Czynniki prognostyczne wpływające na przeżycie

Obecność zespołu rakowiczego jest negatywnym wskaźnikiem prognostycznym, co prawdopodobnie wynika z jego związku z masą guza. W tym spektrum choroba serca w przebiegu zespołu rakowiczego (CHD) również wiąże się ze zmniejszonym przeżyciem pacjentów w analizach jednowymiarowych³. Choroba serca w przebiegu zespołu rakowiczego jest rzadką chorobą, która rozwija się u pacjentów z funkcjonalnymi guzami neuroendokrynnymi w zaawansowanym stadium nowotworu. Pacjenci z rozpoznaniem choroby serca w przebiegu zespołu rakowiczego mają złe rokowanie długoterminowe w odniesieniu do chorobowości i śmiertelności⁴.

Do 70% pacjentów z NET powodującym zespół rakowiczy ostatecznie rozwinie CHD, która wiąże się ze złym rokowaniem długoterminowym, z szacowanym 3-letnim wskaźnikiem przeżycia wynoszącym 31% w porównaniu do 68% obserwowanym u pacjentów z NET powodującym zespół rakowiczy bez zajęcia serca⁵. Jedyną definitywną interwencją łagodzącą objawy CHD jest wymiana zastawki trójdzielnej i/lub płucnej, co zwiększa medianę przeżycia do 6-11 lat⁶.

W wielowariantowej analizie przeżycia wiek ≥50 lat (p=0,005, współczynnik ryzyka=7,8), przerzuty do wątroby lub węzłów chłonnych (p=0,027, współczynnik ryzyka=2,1) oraz płeć męska (p=0,064, współczynnik ryzyka=1,8) zostały zidentyfikowane jako statystycznie istotne niezależne czynniki predykcyjne zgonu⁷.

Wpływ przerzutów na rokowanie

Obecność choroby przerzutowej znacząco zmniejsza przeżywalność. Nawet pacjenci z przerzutowymi guzami rakowiczymi mogą mieć przedłużone przeżycie ze względu na często powolny charakter tego nowotworu. Ogólna mediana przeżycia pacjentów z chorobą przerzutową wynosi nadal 8 lat⁸.

Ryzyko przerzutów koreluje z wielkością guza pierwotnego:

Guzy <1 cm dają przerzuty tylko w 2% przypadków
Guzy 1-2 cm dają przerzuty w 50% przypadków
Guzy >2 cm dają przerzuty w 85% przypadków

⁹

Węzły chłonne i wątroba są najczęstszymi miejscami przerzutów, podczas gdy kości i płuca są rzadziej dotknięte. Zajęcie odległych miejsc (np. jajnika, oczodołu) jest bardzo rzadkie¹⁰.

Wskaźniki przeżycia w zależności od lokalizacji i stadium guza

W przypadku guzów rakowiczych przewodu pokarmowego, leczeni chirurgicznie pacjenci mają ogólnie korzystny 83% wskaźnik 5-letniego przeżycia¹¹. Badania krajowych baz danych wykazały medianę przeżycia wynoszącą 170 miesięcy dla pacjentów z zlokalizowanymi guzami neuroendokrynnymi jelita cienkiego, 145 miesięcy dla choroby regionalnej i 70 miesięcy dla osób z chorobą przerzutową. Przy agresywnej cytoredukcji wątrobowej przerzutów do wątroby, mediana przeżycia może być poprawiona do ponad 100 miesięcy¹².

Guzy rakowicze płuc: Długoterminowe wskaźniki przeżycia dla pacjentów z typowymi guzami rakowiczymi płuc lub oskrzeli po resekcji chirurgicznej przekraczają 85%. Wskaźniki długoterminowego przeżycia są znacznie krótsze dla pacjentów poddawanych resekcji z powodu atypowych rakowiaków – około 52% według McCaughana i wsp.¹³ Pięcioletnie przeżycie dla guzów rakowiczych płuc zostało zgłoszone jako 96% w jednym badaniu (14% miało zajęcie węzłów chłonnych, a żaden nie miał zespołu rakowiczego)¹⁴.

Guzy rakowicze wyrostka robaczkowego: Dziewięćdziesiąt pięć procent guzów rakowiczych wyrostka robaczkowego ma średnicę mniejszą niż 2 cm. Częstość przerzutów w tych przypadkach jest niska. W przeciwieństwie do tego, około 33% pacjentów z guzami rakowiczymi wyrostka robaczkowego o średnicy większej niż 2 cm ma przerzuty węzłowe lub odległe. Gdy choroba jest zlokalizowana, rokowanie rakowiaków wyrostka robaczkowego jest dobre. Wskaźnik 5-letniego przeżycia wynosi 94% dla choroby miejscowej, 85% dla choroby regionalnej i 34% w przypadku obecności przerzutów odległych¹⁵.

Ogólne wskaźniki 5-letniego przeżycia:

60% dla pacjentów z chorobą zlokalizowaną
73% dla osób z przerzutami regionalnymi
36% dla pacjentów z przerzutami odległymi

¹⁶

W jednym z badań retrospektywnych przeżycie pacjentów z guzem okrężnicy esowatej wynosiło tylko 33%, podczas gdy 99% pacjentów z guzem wyrostka robaczkowego przeżyło¹⁷.

Inne czynniki prognostyczne

U dorosłych pacjentów czynniki związane ze złym przeżyciem obejmują utrzymywanie się neurokininy A (NKA) w osoczu, wydalanie kwasu 5-hydroksyindolooctowego z moczem, wiek oraz 5 lub więcej przerzutów do wątroby¹⁸. Wzrost NKA w osoczu wydaje się być niezależny od innych czynników prognostycznych i stanowi najsilniejszy wskaźnik¹⁹.

Stopień zróżnicowania jest ważnym czynnikiem prognostycznym dla NET. Dobrze zróżnicowane NET mają lepsze rokowanie niż słabo zróżnicowane raki neuroendokrynne²⁰. NET z niskim indeksem Ki-67 mają bardziej korzystne rokowanie i dłuższe przeżycie niż te z wysokim indeksem Ki-67²¹.

Osoby z wysokim poziomem CgA we krwi lub 5-HIAA w moczu mają gorsze rokowanie w porównaniu do osób z normalnymi poziomami²². Osoby z chorobą serca w przebiegu zespołu rakowiczego mają mniej korzystne rokowanie²³. Osoby z zespołem Cushinga również mają tendencję do mniej korzystnego rokowania²⁴.

Wpływ wczesnej diagnozy i leczenia na przeżycie

Zaobserwowano, że wczesna diagnoza z monitorowaniem echokardiograficznym choroby serca w przebiegu zespołu rakowiczego podczas przebiegu choroby guza neuroendokrynnego była korzystna dla ogólnego przeżycia pacjentów²⁵. Wskaźniki przeżycia pacjentów z CHD wzrosły w okresie 20 lat z 18 miesięcy w latach 80. do 52 miesięcy pod koniec lat 90., podczas gdy w badanej kohorcie wskaźniki przeżycia po wstępnej diagnozie CHD wynosiły 24 miesiące (zakres 0-144 miesiące)²⁶.

Po wykluczeniu pacjentów, którzy otrzymali diagnozę w końcowym stadium choroby, jedynym istotnym parametrem między pacjentami ze słabym (średnio 6 miesięcy) lub długim (średnio 50 miesięcy) przeżyciem po wstępnej diagnozie CHD był czas do diagnozy CHD po wstępnej diagnozie NET²⁷.

Postępy w metodach diagnostycznych i technikach chirurgicznych pozwoliły na bardziej aktywne zarządzanie i poprawę rokowania²⁸. Najlepsze leczenie choroby przerzutowej pozostaje kontrowersyjne. Większość pacjentów z przerzutami jest bezobjawowa i ma powolny przebieg. Jeśli pacjenci pozostają nieleczeni, przeżycie 5-letnie wynosi 30%²⁹.

Dla wielu pacjentów opcje leczenia zespołów rakowiczych przedłużyły życie, ale bardzo ważne jest zrozumienie postrzegania objawów przez pacjenta i wpływu leczenia, które może wpływać na jakość życia³⁰.

Podsumowanie czynników prognostycznych

Ogólnie rzecz biorąc, zlokalizowany guz rakowiczy, który jest całkowicie wycięty, ma doskonałe rokowanie; wynik dla pacjentów z przerzutami pozostaje jednak niekorzystny³¹. Resekcja chirurgiczna jest leczeniem z wyboru dla poprawy przeżycia³².

U pacjentów z guzami rakowiczymi zlokalizowanymi w wyrostku robaczkowym, wiek, wielkość guza pierwotnego, cechy histologiczne, zajęcie węzłów chłonnych i przerzuty odległe są istotnymi czynnikami w przewidywaniu przeżycia³³. Guzy większe niż 2 cm, dodatnie węzły chłonne i atypowe cechy histologiczne są często związane ze złym rokowaniem³⁴.

Osoby, u których obecnie diagnozuje się guz rakowiczy przewodu pokarmowego, mogą mieć lepsze rokowanie niż pokazują te liczby. Leczenie ulega poprawie z czasem, a te liczby są oparte na osobach, które zostały zdiagnozowane i leczone co najmniej pięć lat wcześniej³⁵.

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Materiały źródłowe

#1 Malignant Carcinoid Syndrome: Practice Essentials, Background, Pathophysiology
https://emedicine.medscape.com/article/282515-overview
The prognosis for patients with malignant carcinoid syndrome is relatively good compared with the prognosis for patients with other malignancies, but the prognosis for any treated patient with progressing, recurring, or relapsing disease is poor. The median 5-year survival rate is 30-67%. Survival for as long as 23 years has been reported. […] For many patients, the treatment options for carcinoid syndromes have prolonged life, but it is very important to understand the patients perception of symptoms and the influence of treatment, which may affect quality of life. […] The incidence of metastasis is estimated at 1-2 cases per 100,000 affected people. Lymph nodes and liver are the most common sites of metastasis, while bone and lung are less commonly affected. Involvement of distant sites (eg, ovary, orbit) is very rare. Risk of metastasis correlates with size of primary tumor, as follows: Tumors 1 cm metastasize in only 2% of cases; Tumors 1-2 cm metastasize in 50% of cases; Tumors 2 cm metastasize in 85% of cases. […] The best treatment for metastatic disease remains controversial. Most patients with metastases are asymptomatic and have an indolent course. If patients are left untreated, survival at 5 years is 30%.
#2 Survival Rates for Gastrointestinal Carcinoid Tumors | American Cancer Society
https://www.cancer.org/cancer/types/gastrointestinal-carcinoid-tumor/detection-diagnosis-staging/survival-rates.html
Survival rates can give you an idea of what percentage of people with the same type and stage of cancer are still alive a certain amount of time (usually 5 years) after they were diagnosed. […] Keep in mind that survival rates are estimates and are often based on previous outcomes of large numbers of people who had a specific cancer, but they cant predict what will happen in any particular persons case. […] People now being diagnosed with a GI carcinoid tumor may have a better outlook than these numbers show. Treatments improve over time, and these numbers are based on people who were diagnosed and treated at least five years earlier.
#3 Gastrointestinal Neuroendocrine Tumors and the Carcinoid Syndrome – Endotext – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK279162/
The presence of carcinoid syndrome is a negative prognostic indicator, which is likely caused by its association with tumor bulk. Within this spectrum, CHD is also associated with decreased survival in patients in univariate analyses. Because of these features carcinoid syndrome should be diligently investigated in all patients with NET and actively managed alongside antiproliferative therapy. […] Resection is the only potential cure for gastrointestinal NET. Recurrence is however frequently observed in NET patients operated on with curative intent. Exceptions that are associated with excellent curation rates after local resection include T1-T2 appendiceal, gastric, duodenal, or rectal NET. Long-term imaging follow-up is mandated for the other subtypes of gastrointestinal NET after resection of localized, locoregional, or oligometastatic disease.
#4 Outcome of carcinoid heart syndrome in patients enrolled in the SwissNet cohort | BMC Cancer | Full Text
https://bmccancer.biomedcentral.com/articles/10.1186/s12885-023-10739-z
Carcinoid heart disease is a rare disease which develops in patients with functional neuroendocrine tumors in an advanced tumor state. Patients diagnosed with carcinoid heart disease have a poor longtime prognosis with respect to morbidity and mortality and long-term data on patient outcomes are lacking. […] We observed that early diagnosis with echocardiographic surveillance of carcinoid heart disease during the course of the neuroendocrine tumor disease was beneficial to overall survival of patients. […] Up to 70% of patients with a NET causing carcinoid syndrome will eventually develop CHD which is associated with a poor long-term prognosis with an estimated 3-year survival rate of 31% vs 68% seen in patients with a NET causing carcinoid syndrome without heart involvement. […] The only definite intervention relieving CHD symptoms is TV and/ or PV valve replacement which increases the median survival to 6-11 years.
#5 Outcome of carcinoid heart syndrome in patients enrolled in the SwissNet cohort | BMC Cancer | Full Text
https://bmccancer.biomedcentral.com/articles/10.1186/s12885-023-10739-z
Carcinoid heart disease is a rare disease which develops in patients with functional neuroendocrine tumors in an advanced tumor state. Patients diagnosed with carcinoid heart disease have a poor longtime prognosis with respect to morbidity and mortality and long-term data on patient outcomes are lacking. […] We observed that early diagnosis with echocardiographic surveillance of carcinoid heart disease during the course of the neuroendocrine tumor disease was beneficial to overall survival of patients. […] Up to 70% of patients with a NET causing carcinoid syndrome will eventually develop CHD which is associated with a poor long-term prognosis with an estimated 3-year survival rate of 31% vs 68% seen in patients with a NET causing carcinoid syndrome without heart involvement. […] The only definite intervention relieving CHD symptoms is TV and/ or PV valve replacement which increases the median survival to 6-11 years.
#6 Outcome of carcinoid heart syndrome in patients enrolled in the SwissNet cohort | BMC Cancer | Full Text
https://bmccancer.biomedcentral.com/articles/10.1186/s12885-023-10739-z
Carcinoid heart disease is a rare disease which develops in patients with functional neuroendocrine tumors in an advanced tumor state. Patients diagnosed with carcinoid heart disease have a poor longtime prognosis with respect to morbidity and mortality and long-term data on patient outcomes are lacking. […] We observed that early diagnosis with echocardiographic surveillance of carcinoid heart disease during the course of the neuroendocrine tumor disease was beneficial to overall survival of patients. […] Up to 70% of patients with a NET causing carcinoid syndrome will eventually develop CHD which is associated with a poor long-term prognosis with an estimated 3-year survival rate of 31% vs 68% seen in patients with a NET causing carcinoid syndrome without heart involvement. […] The only definite intervention relieving CHD symptoms is TV and/ or PV valve replacement which increases the median survival to 6-11 years.
#7 Prognosis and Survival in Patients With Gastrointestinal Tract Carcinoid Tumors
https://pmc.ncbi.nlm.nih.gov/articles/PMC1420828/
Gastrointestinal tract carcinoid tumors have a nonspecific clinical presentation, except in the case of the carcinoid syndrome. Surgical resection is the treatment of choice for improving survival. Surgically treated patients with carcinoid tumor have an overall favorable 83% 5-year survival rate. […] By multivariate analysis, age 50 years, metastases, and male gender were statistically significant predictors of death. […] The presence of metastatic disease significantly decreased survival, as depicted in Figure 2. […] Even patients with metastatic carcinoid tumors could have prolonged survival because of the often indolent nature of this tumor. The overall median survival of patients with metastatic disease was still 8 years. […] A multivariate survival model including all variables selected age 50 years or older (p = 0.005, risk ratio = 7.8), liver or lymph node metastases (p = 0.027, risk ratio = 2.1), and male gender (p = 0.064, risk ratio = 1.8) as statistically significant independent predictors of death. […] Patients with rectal carcinoids have an early drop in survival and do worse than those with ileojejunal carcinoid in the first 4 years, but ultimately the tumors stabilize and behave similarly to the nonileojejunal sites.
#8 Prognosis and Survival in Patients With Gastrointestinal Tract Carcinoid Tumors
https://pmc.ncbi.nlm.nih.gov/articles/PMC1420828/
Gastrointestinal tract carcinoid tumors have a nonspecific clinical presentation, except in the case of the carcinoid syndrome. Surgical resection is the treatment of choice for improving survival. Surgically treated patients with carcinoid tumor have an overall favorable 83% 5-year survival rate. […] By multivariate analysis, age 50 years, metastases, and male gender were statistically significant predictors of death. […] The presence of metastatic disease significantly decreased survival, as depicted in Figure 2. […] Even patients with metastatic carcinoid tumors could have prolonged survival because of the often indolent nature of this tumor. The overall median survival of patients with metastatic disease was still 8 years. […] A multivariate survival model including all variables selected age 50 years or older (p = 0.005, risk ratio = 7.8), liver or lymph node metastases (p = 0.027, risk ratio = 2.1), and male gender (p = 0.064, risk ratio = 1.8) as statistically significant independent predictors of death. […] Patients with rectal carcinoids have an early drop in survival and do worse than those with ileojejunal carcinoid in the first 4 years, but ultimately the tumors stabilize and behave similarly to the nonileojejunal sites.
#9 Malignant Carcinoid Syndrome: Practice Essentials, Background, Pathophysiology
https://emedicine.medscape.com/article/282515-overview
The prognosis for patients with malignant carcinoid syndrome is relatively good compared with the prognosis for patients with other malignancies, but the prognosis for any treated patient with progressing, recurring, or relapsing disease is poor. The median 5-year survival rate is 30-67%. Survival for as long as 23 years has been reported. […] For many patients, the treatment options for carcinoid syndromes have prolonged life, but it is very important to understand the patients perception of symptoms and the influence of treatment, which may affect quality of life. […] The incidence of metastasis is estimated at 1-2 cases per 100,000 affected people. Lymph nodes and liver are the most common sites of metastasis, while bone and lung are less commonly affected. Involvement of distant sites (eg, ovary, orbit) is very rare. Risk of metastasis correlates with size of primary tumor, as follows: Tumors 1 cm metastasize in only 2% of cases; Tumors 1-2 cm metastasize in 50% of cases; Tumors 2 cm metastasize in 85% of cases. […] The best treatment for metastatic disease remains controversial. Most patients with metastases are asymptomatic and have an indolent course. If patients are left untreated, survival at 5 years is 30%.
#10 Malignant Carcinoid Syndrome: Practice Essentials, Background, Pathophysiology
https://emedicine.medscape.com/article/282515-overview
The prognosis for patients with malignant carcinoid syndrome is relatively good compared with the prognosis for patients with other malignancies, but the prognosis for any treated patient with progressing, recurring, or relapsing disease is poor. The median 5-year survival rate is 30-67%. Survival for as long as 23 years has been reported. […] For many patients, the treatment options for carcinoid syndromes have prolonged life, but it is very important to understand the patients perception of symptoms and the influence of treatment, which may affect quality of life. […] The incidence of metastasis is estimated at 1-2 cases per 100,000 affected people. Lymph nodes and liver are the most common sites of metastasis, while bone and lung are less commonly affected. Involvement of distant sites (eg, ovary, orbit) is very rare. Risk of metastasis correlates with size of primary tumor, as follows: Tumors 1 cm metastasize in only 2% of cases; Tumors 1-2 cm metastasize in 50% of cases; Tumors 2 cm metastasize in 85% of cases. […] The best treatment for metastatic disease remains controversial. Most patients with metastases are asymptomatic and have an indolent course. If patients are left untreated, survival at 5 years is 30%.
#11 Prognosis and Survival in Patients With Gastrointestinal Tract Carcinoid Tumors
https://pmc.ncbi.nlm.nih.gov/articles/PMC1420828/
Gastrointestinal tract carcinoid tumors have a nonspecific clinical presentation, except in the case of the carcinoid syndrome. Surgical resection is the treatment of choice for improving survival. Surgically treated patients with carcinoid tumor have an overall favorable 83% 5-year survival rate. […] By multivariate analysis, age 50 years, metastases, and male gender were statistically significant predictors of death. […] The presence of metastatic disease significantly decreased survival, as depicted in Figure 2. […] Even patients with metastatic carcinoid tumors could have prolonged survival because of the often indolent nature of this tumor. The overall median survival of patients with metastatic disease was still 8 years. […] A multivariate survival model including all variables selected age 50 years or older (p = 0.005, risk ratio = 7.8), liver or lymph node metastases (p = 0.027, risk ratio = 2.1), and male gender (p = 0.064, risk ratio = 1.8) as statistically significant independent predictors of death. […] Patients with rectal carcinoids have an early drop in survival and do worse than those with ileojejunal carcinoid in the first 4 years, but ultimately the tumors stabilize and behave similarly to the nonileojejunal sites.
#12 Carcinoid Tumours: Causes, Symptoms and Treatment | Doctor
https://patient.info/doctor/carcinoid-tumours
Advances in diagnostic methods and surgical techniques have allowed more active management and improved prognosis. […] Surveys of national databases have shown a median survival of 170 months for patients with localised small bowel neuroendocrine tumours, 145 months for regional disease, and 70 months for those with metastatic disease. With aggressive hepatic cytoreduction of liver metastases, median survival may be improved to well over 100 months. […] Five-year survival for carcinoid tumours of the lung has been reported as 96% in one study (14% had lymph node involvement and none had carcinoid syndrome).
#13 Neuro-Endocrine/Endocrine system: Carcinoid tumors
https://atlasgeneticsoncology.org/solid-tumor/5523/neuro-endocrine-endocrine-system-carcinoid-tumors
Long-term survival rates for patients with typical lung or bronchial carcinoid tumors following surgical resection exceed 85%. […] Long-term survival rates are significantly shorter for patients who undergo resection for atypical carcinoids – about 52% according to McCaughan et al. […] The 5-year survival rate is 60% for patients with localized disease. It is 73% for those with regional metastases, and 36% for patients with distant metastases. […] The size of the primary lesion is predictive of metastatic disease. Metastases occur in less than 5% of tumors that are less 1 cm in diameter but at a much higher rate when the primary lesions is greater than 2 cm in diameter. […] The 5-year survival rate is 81% for local disease, 47% when regional metastases are present, and 18% with distant metastases.
#14 Carcinoid Tumours: Causes, Symptoms and Treatment | Doctor
https://patient.info/doctor/carcinoid-tumours
Advances in diagnostic methods and surgical techniques have allowed more active management and improved prognosis. […] Surveys of national databases have shown a median survival of 170 months for patients with localised small bowel neuroendocrine tumours, 145 months for regional disease, and 70 months for those with metastatic disease. With aggressive hepatic cytoreduction of liver metastases, median survival may be improved to well over 100 months. […] Five-year survival for carcinoid tumours of the lung has been reported as 96% in one study (14% had lymph node involvement and none had carcinoid syndrome).
#15 Neuro-Endocrine/Endocrine system: Carcinoid tumors
https://atlasgeneticsoncology.org/solid-tumor/5523/neuro-endocrine-endocrine-system-carcinoid-tumors
Ninety-five percent of appendiceal carcinoid tumors are less than 2 cm in diameter. The incidence of metastases in these cases is low. In contrast, about 33% of patients who have appendiceal carcinoid tumors measuring more than 2 cm in diameter have either nodal or distant metastases. When disease is localized, the prognosis of appendiceal carcinoids is good. The 5-year survival rate is 94% for local disease, 85% for regional disease, and 34% when distant metastases are present.
#16 Neuro-Endocrine/Endocrine system: Carcinoid tumors
https://atlasgeneticsoncology.org/solid-tumor/5523/neuro-endocrine-endocrine-system-carcinoid-tumors
Long-term survival rates for patients with typical lung or bronchial carcinoid tumors following surgical resection exceed 85%. […] Long-term survival rates are significantly shorter for patients who undergo resection for atypical carcinoids – about 52% according to McCaughan et al. […] The 5-year survival rate is 60% for patients with localized disease. It is 73% for those with regional metastases, and 36% for patients with distant metastases. […] The size of the primary lesion is predictive of metastatic disease. Metastases occur in less than 5% of tumors that are less 1 cm in diameter but at a much higher rate when the primary lesions is greater than 2 cm in diameter. […] The 5-year survival rate is 81% for local disease, 47% when regional metastases are present, and 18% with distant metastases.
#17 Carcinoid Tumor: Practice Essentials, Background, Pathophysiology
https://emedicine.medscape.com/article/986050-overview
No systematic data for survival of children with carcinoid tumors is currently available. […] The prognosis for patients with completely resected localized disease is excellent. […] Tumors larger than 2 cm, positive lymph nodes, and atypical histologic features are often associated with a poor prognosis. […] In patients with carcinoid tumors located in the appendix, age, primary tumor size, histologic features, lymph node involvement, and distant metastasis are significant factors in predicting survival. […] In one retroactive study, survival of patients with sigmoid colon was only 33% while 99% of those with appendiceal tumor had survived. […] Second primary malignancies of gastrointestinal tract which occurred in up to 33% of cases in adults, are unusual in children and adolescents.
#18 Carcinoid Tumor: Practice Essentials, Background, Pathophysiology
https://emedicine.medscape.com/article/986050-overview
In adult patients, factors associated with poor survival include persistence of plasma neurokinin A (NKA), urinary 50-hydroxyindolacetic acid output, age, and 5 or more liver metastases. […] Rise in plasma NKA appears to be independent of other prognostic factors and constitutes the strongest indication. […] Overall, localized carcinoid tumor which is completely resected has an excellent prognosis; the outcome for patients with metastasis, however, remains poor.
#19 Carcinoid Tumor: Practice Essentials, Background, Pathophysiology
https://emedicine.medscape.com/article/986050-overview
In adult patients, factors associated with poor survival include persistence of plasma neurokinin A (NKA), urinary 50-hydroxyindolacetic acid output, age, and 5 or more liver metastases. […] Rise in plasma NKA appears to be independent of other prognostic factors and constitutes the strongest indication. […] Overall, localized carcinoid tumor which is completely resected has an excellent prognosis; the outcome for patients with metastasis, however, remains poor.
#20 Prognosis and survival for neuroendocrine tumours (NETs) | Canadian Cancer Society
https://cancer.ca/en/cancer-information/cancer-types/neuroendocrine-tumours/prognosis-and-survival
The level of differentiation is an important prognostic factor for NETs. Well-differentiated NETs have a better prognosis than poorly differentiated neuroendocrine carcinomas. […] NETs with a low Ki-67 index have a more favourable prognosis and longer survival than those with a high Ki-67 index. […] People with high levels of CgA in the blood or 5-HIAA in the urine have a poorer prognosis compared to people with normal levels. […] People with carcinoid heart disease have a less favourable prognosis. […] People with Cushing syndrome also tend to have a less favourable prognosis.
#21 Prognosis and survival for neuroendocrine tumours (NETs) | Canadian Cancer Society
https://cancer.ca/en/cancer-information/cancer-types/neuroendocrine-tumours/prognosis-and-survival
The level of differentiation is an important prognostic factor for NETs. Well-differentiated NETs have a better prognosis than poorly differentiated neuroendocrine carcinomas. […] NETs with a low Ki-67 index have a more favourable prognosis and longer survival than those with a high Ki-67 index. […] People with high levels of CgA in the blood or 5-HIAA in the urine have a poorer prognosis compared to people with normal levels. […] People with carcinoid heart disease have a less favourable prognosis. […] People with Cushing syndrome also tend to have a less favourable prognosis.
#22 Prognosis and survival for neuroendocrine tumours (NETs) | Canadian Cancer Society
https://cancer.ca/en/cancer-information/cancer-types/neuroendocrine-tumours/prognosis-and-survival
The level of differentiation is an important prognostic factor for NETs. Well-differentiated NETs have a better prognosis than poorly differentiated neuroendocrine carcinomas. […] NETs with a low Ki-67 index have a more favourable prognosis and longer survival than those with a high Ki-67 index. […] People with high levels of CgA in the blood or 5-HIAA in the urine have a poorer prognosis compared to people with normal levels. […] People with carcinoid heart disease have a less favourable prognosis. […] People with Cushing syndrome also tend to have a less favourable prognosis.
#23 Prognosis and survival for neuroendocrine tumours (NETs) | Canadian Cancer Society
https://cancer.ca/en/cancer-information/cancer-types/neuroendocrine-tumours/prognosis-and-survival
The level of differentiation is an important prognostic factor for NETs. Well-differentiated NETs have a better prognosis than poorly differentiated neuroendocrine carcinomas. […] NETs with a low Ki-67 index have a more favourable prognosis and longer survival than those with a high Ki-67 index. […] People with high levels of CgA in the blood or 5-HIAA in the urine have a poorer prognosis compared to people with normal levels. […] People with carcinoid heart disease have a less favourable prognosis. […] People with Cushing syndrome also tend to have a less favourable prognosis.
#24 Prognosis and survival for neuroendocrine tumours (NETs) | Canadian Cancer Society
https://cancer.ca/en/cancer-information/cancer-types/neuroendocrine-tumours/prognosis-and-survival
The level of differentiation is an important prognostic factor for NETs. Well-differentiated NETs have a better prognosis than poorly differentiated neuroendocrine carcinomas. […] NETs with a low Ki-67 index have a more favourable prognosis and longer survival than those with a high Ki-67 index. […] People with high levels of CgA in the blood or 5-HIAA in the urine have a poorer prognosis compared to people with normal levels. […] People with carcinoid heart disease have a less favourable prognosis. […] People with Cushing syndrome also tend to have a less favourable prognosis.
#25 Outcome of carcinoid heart syndrome in patients enrolled in the SwissNet cohort | BMC Cancer | Full Text
https://bmccancer.biomedcentral.com/articles/10.1186/s12885-023-10739-z
Carcinoid heart disease is a rare disease which develops in patients with functional neuroendocrine tumors in an advanced tumor state. Patients diagnosed with carcinoid heart disease have a poor longtime prognosis with respect to morbidity and mortality and long-term data on patient outcomes are lacking. […] We observed that early diagnosis with echocardiographic surveillance of carcinoid heart disease during the course of the neuroendocrine tumor disease was beneficial to overall survival of patients. […] Up to 70% of patients with a NET causing carcinoid syndrome will eventually develop CHD which is associated with a poor long-term prognosis with an estimated 3-year survival rate of 31% vs 68% seen in patients with a NET causing carcinoid syndrome without heart involvement. […] The only definite intervention relieving CHD symptoms is TV and/ or PV valve replacement which increases the median survival to 6-11 years.
#26 Outcome of carcinoid heart syndrome in patients enrolled in the SwissNet cohort | BMC Cancer | Full Text
https://bmccancer.biomedcentral.com/articles/10.1186/s12885-023-10739-z
Survival rates of patients with CHD increased over a time period of 20 years from 18 months in the 1980s to 52 months in the late 1990s while in our cohort, survival rates following initial CHD diagnosis were 24 months ranging from 0-144 months. […] When excluding patients who received their diagnoses at end stage disease, the only significant parameter between patients with poor (average of 6 months) or long (average of 50 months) survival rates following initial CHD diagnosis was the time to CHD diagnosis following initial NET diagnosis. […] Therefore, large long-term observational clinical trials are needed to further study markers influencing occurrence, severity, progression and long-term survival of patients with CHD to gain a better understanding of the disease and decrease morbidity and mortality among patients diagnosed with CHD.
#27 Outcome of carcinoid heart syndrome in patients enrolled in the SwissNet cohort | BMC Cancer | Full Text
https://bmccancer.biomedcentral.com/articles/10.1186/s12885-023-10739-z
Survival rates of patients with CHD increased over a time period of 20 years from 18 months in the 1980s to 52 months in the late 1990s while in our cohort, survival rates following initial CHD diagnosis were 24 months ranging from 0-144 months. […] When excluding patients who received their diagnoses at end stage disease, the only significant parameter between patients with poor (average of 6 months) or long (average of 50 months) survival rates following initial CHD diagnosis was the time to CHD diagnosis following initial NET diagnosis. […] Therefore, large long-term observational clinical trials are needed to further study markers influencing occurrence, severity, progression and long-term survival of patients with CHD to gain a better understanding of the disease and decrease morbidity and mortality among patients diagnosed with CHD.
#28 Carcinoid Tumours: Causes, Symptoms and Treatment | Doctor
https://patient.info/doctor/carcinoid-tumours
Advances in diagnostic methods and surgical techniques have allowed more active management and improved prognosis. […] Surveys of national databases have shown a median survival of 170 months for patients with localised small bowel neuroendocrine tumours, 145 months for regional disease, and 70 months for those with metastatic disease. With aggressive hepatic cytoreduction of liver metastases, median survival may be improved to well over 100 months. […] Five-year survival for carcinoid tumours of the lung has been reported as 96% in one study (14% had lymph node involvement and none had carcinoid syndrome).
#29 Malignant Carcinoid Syndrome: Practice Essentials, Background, Pathophysiology
https://emedicine.medscape.com/article/282515-overview
The prognosis for patients with malignant carcinoid syndrome is relatively good compared with the prognosis for patients with other malignancies, but the prognosis for any treated patient with progressing, recurring, or relapsing disease is poor. The median 5-year survival rate is 30-67%. Survival for as long as 23 years has been reported. […] For many patients, the treatment options for carcinoid syndromes have prolonged life, but it is very important to understand the patients perception of symptoms and the influence of treatment, which may affect quality of life. […] The incidence of metastasis is estimated at 1-2 cases per 100,000 affected people. Lymph nodes and liver are the most common sites of metastasis, while bone and lung are less commonly affected. Involvement of distant sites (eg, ovary, orbit) is very rare. Risk of metastasis correlates with size of primary tumor, as follows: Tumors 1 cm metastasize in only 2% of cases; Tumors 1-2 cm metastasize in 50% of cases; Tumors 2 cm metastasize in 85% of cases. […] The best treatment for metastatic disease remains controversial. Most patients with metastases are asymptomatic and have an indolent course. If patients are left untreated, survival at 5 years is 30%.
#30 Malignant Carcinoid Syndrome: Practice Essentials, Background, Pathophysiology
https://emedicine.medscape.com/article/282515-overview
The prognosis for patients with malignant carcinoid syndrome is relatively good compared with the prognosis for patients with other malignancies, but the prognosis for any treated patient with progressing, recurring, or relapsing disease is poor. The median 5-year survival rate is 30-67%. Survival for as long as 23 years has been reported. […] For many patients, the treatment options for carcinoid syndromes have prolonged life, but it is very important to understand the patients perception of symptoms and the influence of treatment, which may affect quality of life. […] The incidence of metastasis is estimated at 1-2 cases per 100,000 affected people. Lymph nodes and liver are the most common sites of metastasis, while bone and lung are less commonly affected. Involvement of distant sites (eg, ovary, orbit) is very rare. Risk of metastasis correlates with size of primary tumor, as follows: Tumors 1 cm metastasize in only 2% of cases; Tumors 1-2 cm metastasize in 50% of cases; Tumors 2 cm metastasize in 85% of cases. […] The best treatment for metastatic disease remains controversial. Most patients with metastases are asymptomatic and have an indolent course. If patients are left untreated, survival at 5 years is 30%.
#31 Carcinoid Tumor: Practice Essentials, Background, Pathophysiology
https://emedicine.medscape.com/article/986050-overview
In adult patients, factors associated with poor survival include persistence of plasma neurokinin A (NKA), urinary 50-hydroxyindolacetic acid output, age, and 5 or more liver metastases. […] Rise in plasma NKA appears to be independent of other prognostic factors and constitutes the strongest indication. […] Overall, localized carcinoid tumor which is completely resected has an excellent prognosis; the outcome for patients with metastasis, however, remains poor.
#32 Prognosis and Survival in Patients With Gastrointestinal Tract Carcinoid Tumors
https://pmc.ncbi.nlm.nih.gov/articles/PMC1420828/
Gastrointestinal tract carcinoid tumors have a nonspecific clinical presentation, except in the case of the carcinoid syndrome. Surgical resection is the treatment of choice for improving survival. Surgically treated patients with carcinoid tumor have an overall favorable 83% 5-year survival rate. […] By multivariate analysis, age 50 years, metastases, and male gender were statistically significant predictors of death. […] The presence of metastatic disease significantly decreased survival, as depicted in Figure 2. […] Even patients with metastatic carcinoid tumors could have prolonged survival because of the often indolent nature of this tumor. The overall median survival of patients with metastatic disease was still 8 years. […] A multivariate survival model including all variables selected age 50 years or older (p = 0.005, risk ratio = 7.8), liver or lymph node metastases (p = 0.027, risk ratio = 2.1), and male gender (p = 0.064, risk ratio = 1.8) as statistically significant independent predictors of death. […] Patients with rectal carcinoids have an early drop in survival and do worse than those with ileojejunal carcinoid in the first 4 years, but ultimately the tumors stabilize and behave similarly to the nonileojejunal sites.
#33 Carcinoid Tumor: Practice Essentials, Background, Pathophysiology
https://emedicine.medscape.com/article/986050-overview
No systematic data for survival of children with carcinoid tumors is currently available. […] The prognosis for patients with completely resected localized disease is excellent. […] Tumors larger than 2 cm, positive lymph nodes, and atypical histologic features are often associated with a poor prognosis. […] In patients with carcinoid tumors located in the appendix, age, primary tumor size, histologic features, lymph node involvement, and distant metastasis are significant factors in predicting survival. […] In one retroactive study, survival of patients with sigmoid colon was only 33% while 99% of those with appendiceal tumor had survived. […] Second primary malignancies of gastrointestinal tract which occurred in up to 33% of cases in adults, are unusual in children and adolescents.
#34 Carcinoid Tumor: Practice Essentials, Background, Pathophysiology
https://emedicine.medscape.com/article/986050-overview
No systematic data for survival of children with carcinoid tumors is currently available. […] The prognosis for patients with completely resected localized disease is excellent. […] Tumors larger than 2 cm, positive lymph nodes, and atypical histologic features are often associated with a poor prognosis. […] In patients with carcinoid tumors located in the appendix, age, primary tumor size, histologic features, lymph node involvement, and distant metastasis are significant factors in predicting survival. […] In one retroactive study, survival of patients with sigmoid colon was only 33% while 99% of those with appendiceal tumor had survived. […] Second primary malignancies of gastrointestinal tract which occurred in up to 33% of cases in adults, are unusual in children and adolescents.
#35 Survival Rates for Gastrointestinal Carcinoid Tumors | American Cancer Society
https://www.cancer.org/cancer/types/gastrointestinal-carcinoid-tumor/detection-diagnosis-staging/survival-rates.html
Survival rates can give you an idea of what percentage of people with the same type and stage of cancer are still alive a certain amount of time (usually 5 years) after they were diagnosed. […] Keep in mind that survival rates are estimates and are often based on previous outcomes of large numbers of people who had a specific cancer, but they cant predict what will happen in any particular persons case. […] People now being diagnosed with a GI carcinoid tumor may have a better outlook than these numbers show. Treatments improve over time, and these numbers are based on people who were diagnosed and treated at least five years earlier.