Feochromocytoma – Zapobieganie i profilaktyka

Feochromocytoma
Zapobieganie i profilaktyka

Feochromocytoma to rzadki, katecholaminoaktywny nowotwór chromochłonny wywodzący się z rdzenia nadnerczy, z etiologią często nieznaną, choć w 25-40% przypadków związany z mutacjami genetycznymi (MEN2, VHL, NF1, dziedziczny zespół przyzwojaków, diada i triada Carneya). Diagnostyka obejmuje badania biochemiczne i genetyczne, szczególnie u pacjentów z wywiadem rodzinnym lub potwierdzonymi mutacjami germinalnymi, z zaleceniem corocznego monitorowania od 5. roku życia u chorych z zespołem VHL. Profilaktyka wtórna opiera się na wczesnym wykrywaniu i stałym nadzorze endokrynologicznym, co pozwala na zapobieganie powikłaniom sercowo-naczyniowym i transformacji złośliwej poprzez odpowiednie przygotowanie przedoperacyjne, w tym stosowanie antagonistów alfa (nieselektywnych lub selektywnych), blokerów kanału wapniowego oraz metyroksyny, a także rehydratację i kontrolę lęku.

Profilaktyka Feochromocytoma

Genetyczne uwarunkowania i badania przesiewowe
Wskazania do badań genetycznych

Profilaktyka wtórna feochromocytoma

Przygotowanie przedoperacyjne

Postępowanie kliniczne w feochromocytoma

Operacja oszczędzająca korę nadnerczy
Algorytm postępowania
Kolejne rozdziały

Profilaktyka Feochromocytoma

Feochromocytoma to rzadki nowotwór chromochłonny wywodzący się z rdzenia nadnerczy, produkujący katecholaminy. Niestety, nie istnieją znane metody zapobiegania rozwojowi feochromocytoma ¹²³. W wielu przypadkach przyczyny powstania tego guza pozostają nieznane, zwłaszcza w przypadkach sporadycznych ⁴. Jednak istnieją strategie ograniczające ryzyko powikłań u osób predysponowanych genetycznie oraz działania profilaktyczne mające na celu wczesne wykrycie i odpowiednie przygotowanie do leczenia.

Genetyczne uwarunkowania i badania przesiewowe

Czynniki dziedziczne odgrywają istotną rolę w około 25-40% przypadków feochromocytoma ⁵. Osoby zagrożone rozwojem guza z powodu określonych dziedzicznych zespołów i genów mogą skorzystać z poradnictwa genetycznego, które pomaga w badaniach przesiewowych w kierunku feochromocytoma i potencjalnie umożliwia wykrycie go we wczesnych fazach ⁶.

Należy skonsultować się z lekarzem prowadzącym, jeśli którykolwiek z krewnych pierwszego stopnia (rodzeństwo i rodzice) został zdiagnozowany z feochromocytoma i/lub posiada jedną z następujących chorób genetycznych ⁷⁸:

Zespół mnogiej gruczolakowatości wewnątrzwydzielniczej typu 2 (MEN2)
Choroba von Hippla-Lindaua (VHL)
Neurofibromatoza typu 1 (NF1)
Dziedziczny zespół przyzwojaków
Diada Carneya-Stratakisa
Triada Carneya

⁹¹⁰

Towarzystwo Endokrynologiczne zaleca przeprowadzanie badań biochemicznych w kierunku feochromocytoma u pacjentów z zespołem VHL, rozpoczynając je w 5. roku życia, z kontynuacją kontroli biochemicznej co roku przez całe życie ¹¹.

Wskazania do badań genetycznych

Badania genetyczne powinny być wykonywane u ¹²:

Pacjentów z wywiadem rodzinnym feochromocytoma
Osób z potwierdzoną mutacją germinalną genu RET u krewnych pierwszego stopnia

¹³

Osoby z kliniczną lub rodzinną historią dziedzicznego zespołu przyzwojaków i guzów chromochłonnych (PGL/PCC) powinny być badane wcześnie. Wczesna diagnoza może poprawić rokowanie pacjenta poprzez regularne badania przesiewowe i leczenie nowotworów o wczesnym początku. Wczesne wykrycie poprzez nadzór i usunięcie guzów może zapobiec lub zminimalizować powikłania związane z efektami masy guza, nadmierną sekrecją katecholamin i transformacją złośliwą ¹⁴.

Profilaktyka wtórna feochromocytoma

Skuteczne środki profilaktyki wtórnej feochromocytoma obejmują obowiązkowe badania biochemiczne dla członków rodzin pacjentów z MEN2 oraz badania genetyczne u krewnych pierwszego stopnia pacjenta z potwierdzoną mutacją germinalną genu RET ¹⁵.

Jeśli osoba jest w grupie ryzyka, ale nie ma guzów, wskazane jest zapewnienie stałego monitorowania w celu sprawdzenia wczesnych objawów feochromocytoma ¹⁶. Jeśli istnieje znana choroba genetyczna, taka jak neurofibromatoza typu 1, mnoga gruczolakowatość wewnątrzwydzielnicza typu 2 a/b, zespół von Hippla-Lindaua lub zespół przyzwojaków, choroba musi być ściśle i regularnie monitorowana przez endokrynologa. W ten sposób feochromocytoma może zostać wykryte i leczone na wczesnym etapie ¹⁷.

Przygotowanie przedoperacyjne

Odpowiednie przygotowanie przedoperacyjne jest niezbędne do zmniejszenia chorobowości i śmiertelności okołooperacyjnej w przypadku feochromocytoma ¹⁸. Przedoperacyjna ocena powinna koncentrować się na ocenie uszkodzeń narządów końcowych spowodowanych podwyższonym poziomem katecholamin ¹⁹.

Celem opieki przedoperacyjnej jest zapobieganie nagłemu uwolnieniu katecholamin lub złagodzenie odpowiedzi na uwolnienie katecholamin w okresie okołooperacyjnym ²⁰. Przygotowanie przedoperacyjne powinno być rozpoczęte nawet u pacjentów z prawidłowym ciśnieniem tętniczym lub bezobjawowych ²¹.

Zalecane strategie przedoperacyjne obejmują ²²:

Nieselektywne antagonisty alfa – stosowane do momentu osiągnięcia hipotonii ortostatycznej
Selektywne antagonisty alfa takie jak urapidil, prazosyna, terazosyna i doksazosyna – mają mniejszą kontrolę hemodynamiczną śródoperacyjną, ale również mniejszą hipotonię pooperacyjną i zmniejszony profil działań niepożądanych
Blokery kanału wapniowego dihydropirydynowe (np. amlodypina, nifedypina i nikardypina) – są szeroko dostępne i skuteczne w kontrolowaniu hemodynamiki śródoperacyjnej, szczególnie w przypadku mniejszych i mniej aktywnych guzów
Metyroksyna – nie powinna być stosowana jako monoterapia, ale jest doskonałą terapią dodatkową, która wykazała zmniejszenie powikłań
Rehydratacja i wyrównanie elektrolitów – choć nie ma badań specjalnie odnoszących się do tej praktyki w przygotowaniu do operacji, jest ona powszechnie zalecana
Zmniejszenie lęku przed indukcją jest kluczowe, aby zapobiec wzrostowi katecholamin z powodu obaw

²³

Przedoperacyjna blokada hormonalnie czynnych guzów chromochłonnych w celu zapobiegania powikłaniom sercowo-naczyniowym jest zalecana, wraz z przedoperacyjnym leczeniem mającym na celu normalizację ciśnienia krwi i częstości akcji serca, w połączeniu z dietą wysokosodową z odpowiednią podażą płynów, aby zapobiec ciężkiemu niedociśnieniu po usunięciu guza ²⁴.

Postępowanie kliniczne w feochromocytoma

Resekcja chirurgiczna guza jest leczeniem z wyboru i zwykle prowadzi do wyleczenia nadciśnienia. Staranne przedoperacyjne postępowanie jest wymagane do kontroli ciśnienia krwi, częstości akcji serca, objętości płynów i zapobiegania śródoperacyjnym kryzysom nadciśnieniowym ²⁵.

Ze względu na rzadkość tej choroby, wskazane jest leczenie w wyspecjalizowanym ośrodku. W ramach konsultacji nadnerczowych pacjent powinien otrzymać poradę na temat niezbędnych badań (badanie genetyczne) oraz późniejszej opieki (regularne obrazowanie, badania przesiewowe w kierunku innych guzów w zależności od choroby podstawowej) ²⁶.

Operacja oszczędzająca korę nadnerczy

Obecnym standardem leczenia feochromocytoma obejmującego oba nadnercza jest usunięcie obu nadnerczy (całkowita adrenalektomia) ²⁷. Jednak ostatnie dane sugerują, że zachowanie jak największej części kory nadnerczy (adrenalektomia oszczędzająca korę) może zmniejszyć potrzebę terapii zastępczej hormonami, która sama w sobie stanowi unikalne zagrożenie dla zdrowia ²⁸.

Adrenalektomia oszczędzająca korę jest często idealna dla osób z dziedzicznym feochromocytoma, ponieważ są one bardziej narażone na rozwój nowych guzów, które wymagałyby dodatkowych operacji ²⁹. W związku z tym zespół badawczy zaleca, aby wszystkim pacjentom z feochromocytoma oferować analizę genetyczną w celu kierowania decyzjami dotyczącymi postępowania chirurgicznego i medycznego ³⁰.

Multidyscyplinarny zespół badaczy doszedł do wniosku, że operacja oszczędzająca korę nadnerczy może być odpowiednia i bezpieczna w niektórych przypadkach obustronnego feochromocytoma, szczególnie gdy obecna jest predysponująca mutacja ³¹. Badanie to podkreśla znaczenie testów genetycznych w diagnostyce i leczeniu feochromocytoma ³².

Algorytm postępowania

Feochromocytoma może wpływać na wiele narządów i może wymagać wielu metod leczenia. Podejście do pacjenta powinno obejmować zespół multidyscyplinarny, w tym chirurgów, onkologów, urologów, otolaryngologów i innych specjalistów ³³.

Algorytm postępowania w feochromocytoma ³⁴:

Jeśli pacjent jest w grupie ryzyka, ale nie ma guzów – zapewnienie ciągłego monitorowania w celu sprawdzenia wczesnych objawów
Jeśli obecne są guzy i pacjent kwalifikuje się do operacji – przeprowadzenie zabiegu usunięcia guzów
Jeśli obecne są guzy, a pacjent nie kwalifikuje się do operacji – zastosowanie innych metod leczenia, takich jak chemioterapia lub badania kliniczne
Jeśli guzy nawracają po leczeniu – rozważenie dodatkowych opcji leczenia, które mogą obejmować badania kliniczne

³⁵

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Materiały źródłowe

#1 Pheochromocytoma Causes, Symptoms, and Treatments | UPMC
https://www.upmc.com/services/endocrinology/conditions/pheochromocytoma
There are no known ways to prevent PCC. […] But if you have the tumor, you should have genetic counseling. This can help you find out your risk of getting other cancers. […] Your doctors may suggest genetic testing for close family members. They may be at risk for PCC or other tumors.
#2 Pheochromocytoma: Symptoms, Causes, Diagnosis, and Treatment
https://www.webmd.com/cancer/what_is_pheochromocytoma?page=3
There’s no way to prevent a pheochromocytoma from developing. But those at risk of developing the tumor due to certain inherited conditions or genes can seek genetic testing to screen for it. Talk to your doctor if you have a parent or sibling with paraganglioma or pheochromocytoma or one of the following conditions: […] Multiple endocrine neoplasia, type 2 (MEN2) […] Von Hippel-Lindau disease […] Neurofibromatosis 1 (NF1) […] Carney-Stratakis dyad […] Carney triad.
#3 Pheochromocytoma – USZ
https://www.usz.ch/en/disease/pheochromocytoma/
The causes of pheochromocytoma are unknown in many cases. Therefore, you cannot prevent the tumor in the adrenal medulla in sporadic cases. There are also no special early detection measures for pheochromocytoma. […] However, hereditary factors play a role in around 25 to 40 percent of people with a pheochromocytoma. Regular check-ups are advisable, for example for healthy family members with the corresponding genetic changes: […] If you have a known genetic disease such as neurofibromatosis type 1, multiple endocrine neoplasia type 2 a/b, Von Hippel-Lindau syndrome or paraganglioma syndrome, you must have your disease monitored closely and regularly by an endocrinologist. In this way, a pheochromocytoma can be detected and treated at an early stage. […] As pheochromocytoma is a rare disease, treatment in a specialized center is advisable. In our adrenal consultation at the USZ, you will be advised on the necessary clarifications (genetic examination) and the subsequent follow-up care (regular imaging, screening for other tumors depending on the underlying disease).
#4 Pheochromocytoma – USZ
https://www.usz.ch/en/disease/pheochromocytoma/
The causes of pheochromocytoma are unknown in many cases. Therefore, you cannot prevent the tumor in the adrenal medulla in sporadic cases. There are also no special early detection measures for pheochromocytoma. […] However, hereditary factors play a role in around 25 to 40 percent of people with a pheochromocytoma. Regular check-ups are advisable, for example for healthy family members with the corresponding genetic changes: […] If you have a known genetic disease such as neurofibromatosis type 1, multiple endocrine neoplasia type 2 a/b, Von Hippel-Lindau syndrome or paraganglioma syndrome, you must have your disease monitored closely and regularly by an endocrinologist. In this way, a pheochromocytoma can be detected and treated at an early stage. […] As pheochromocytoma is a rare disease, treatment in a specialized center is advisable. In our adrenal consultation at the USZ, you will be advised on the necessary clarifications (genetic examination) and the subsequent follow-up care (regular imaging, screening for other tumors depending on the underlying disease).
#5 Pheochromocytoma – USZ
https://www.usz.ch/en/disease/pheochromocytoma/
The causes of pheochromocytoma are unknown in many cases. Therefore, you cannot prevent the tumor in the adrenal medulla in sporadic cases. There are also no special early detection measures for pheochromocytoma. […] However, hereditary factors play a role in around 25 to 40 percent of people with a pheochromocytoma. Regular check-ups are advisable, for example for healthy family members with the corresponding genetic changes: […] If you have a known genetic disease such as neurofibromatosis type 1, multiple endocrine neoplasia type 2 a/b, Von Hippel-Lindau syndrome or paraganglioma syndrome, you must have your disease monitored closely and regularly by an endocrinologist. In this way, a pheochromocytoma can be detected and treated at an early stage. […] As pheochromocytoma is a rare disease, treatment in a specialized center is advisable. In our adrenal consultation at the USZ, you will be advised on the necessary clarifications (genetic examination) and the subsequent follow-up care (regular imaging, screening for other tumors depending on the underlying disease).
#6 Pheochromocytoma: Causes, Symptoms & Treatment
https://my.clevelandclinic.org/health/diseases/23373-pheochromocytoma
Unfortunately, you cant prevent developing a pheochromocytoma. However, if youre at risk for developing a pheochromocytoma due to certain inherited syndromes and genes, genetic counseling can help screen for pheochromocytoma and potentially help you catch it in its early phases. […] Talk to your healthcare provider if you have any first-degree relatives (siblings and parents) that have been diagnosed with pheochromocytoma and/or any of the following genetic conditions: Multiple endocrine neoplasia 2 syndrome, Von Hippel-Lindau (VHL) disease, Neurofibromatosis type 1 (NF1), Hereditary paraganglioma syndrome, Carney-Stratakis dyad, Carney triad.
#7 Pheochromocytoma: Causes, Symptoms & Treatment
https://my.clevelandclinic.org/health/diseases/23373-pheochromocytoma
Unfortunately, you cant prevent developing a pheochromocytoma. However, if youre at risk for developing a pheochromocytoma due to certain inherited syndromes and genes, genetic counseling can help screen for pheochromocytoma and potentially help you catch it in its early phases. […] Talk to your healthcare provider if you have any first-degree relatives (siblings and parents) that have been diagnosed with pheochromocytoma and/or any of the following genetic conditions: Multiple endocrine neoplasia 2 syndrome, Von Hippel-Lindau (VHL) disease, Neurofibromatosis type 1 (NF1), Hereditary paraganglioma syndrome, Carney-Stratakis dyad, Carney triad.
#8 Pheochromocytoma: Symptoms, Causes, Diagnosis, and Treatment
https://www.webmd.com/cancer/what_is_pheochromocytoma?page=3
There’s no way to prevent a pheochromocytoma from developing. But those at risk of developing the tumor due to certain inherited conditions or genes can seek genetic testing to screen for it. Talk to your doctor if you have a parent or sibling with paraganglioma or pheochromocytoma or one of the following conditions: […] Multiple endocrine neoplasia, type 2 (MEN2) […] Von Hippel-Lindau disease […] Neurofibromatosis 1 (NF1) […] Carney-Stratakis dyad […] Carney triad.
#9 Pheochromocytoma: Causes, Symptoms & Treatment
https://my.clevelandclinic.org/health/diseases/23373-pheochromocytoma
Unfortunately, you cant prevent developing a pheochromocytoma. However, if youre at risk for developing a pheochromocytoma due to certain inherited syndromes and genes, genetic counseling can help screen for pheochromocytoma and potentially help you catch it in its early phases. […] Talk to your healthcare provider if you have any first-degree relatives (siblings and parents) that have been diagnosed with pheochromocytoma and/or any of the following genetic conditions: Multiple endocrine neoplasia 2 syndrome, Von Hippel-Lindau (VHL) disease, Neurofibromatosis type 1 (NF1), Hereditary paraganglioma syndrome, Carney-Stratakis dyad, Carney triad.
#10 Pheochromocytoma: Symptoms, Causes, Diagnosis, and Treatment
https://www.webmd.com/cancer/what_is_pheochromocytoma?page=3
There’s no way to prevent a pheochromocytoma from developing. But those at risk of developing the tumor due to certain inherited conditions or genes can seek genetic testing to screen for it. Talk to your doctor if you have a parent or sibling with paraganglioma or pheochromocytoma or one of the following conditions: […] Multiple endocrine neoplasia, type 2 (MEN2) […] Von Hippel-Lindau disease […] Neurofibromatosis 1 (NF1) […] Carney-Stratakis dyad […] Carney triad.
#11 Pheochromocytoma secondary prevention – wikidoc
https://www.wikidoc.org/index.php/Pheochromocytoma_secondary_prevention
Effective measures for the secondary prevention of pheochromocytoma include biochemical screening for family members of MEN2 patients is mandatory and Genetic testing in first-degree relatives of a patient with proven germline RET mutation. […] According to the Endocrine Society, biochemical screening for pheochromocytoma in recommended among patients with: VHL syndrome- started at 5 years of age with biochemical surveillance every year for the rest of life. […] Genetic testing should be performed in: Patients with a family history of pheochromocytoma.
#12 Pheochromocytoma secondary prevention – wikidoc
https://www.wikidoc.org/index.php/Pheochromocytoma_secondary_prevention
Effective measures for the secondary prevention of pheochromocytoma include biochemical screening for family members of MEN2 patients is mandatory and Genetic testing in first-degree relatives of a patient with proven germline RET mutation. […] According to the Endocrine Society, biochemical screening for pheochromocytoma in recommended among patients with: VHL syndrome- started at 5 years of age with biochemical surveillance every year for the rest of life. […] Genetic testing should be performed in: Patients with a family history of pheochromocytoma.
#13 Pheochromocytoma secondary prevention – wikidoc
https://www.wikidoc.org/index.php/Pheochromocytoma_secondary_prevention
Effective measures for the secondary prevention of pheochromocytoma include biochemical screening for family members of MEN2 patients is mandatory and Genetic testing in first-degree relatives of a patient with proven germline RET mutation. […] According to the Endocrine Society, biochemical screening for pheochromocytoma in recommended among patients with: VHL syndrome- started at 5 years of age with biochemical surveillance every year for the rest of life. […] Genetic testing should be performed in: Patients with a family history of pheochromocytoma.
#14 Hereditary Paraganglioma and Pheochromocytoma Panel Test – PreventionGenetics
https://www.preventiongenetics.com/testInfo?val=Hereditary-Paraganglioma-and-Pheochromocytoma-Panel
Individuals with a clinical or family history of hereditary PGL/PCC syndrome should be tested early. Early diagnosis may improve patient prognosis through regular screening and treatment for early-onset malignancies. Early detection through surveillance and removal of tumors may prevent or minimize complications related to mass effects, catecholamine hypersecretion, and malignant transformation. […] Early diagnosis may improve patient prognosis through regular screening and treatment for early-onset malignancies. Early detection through surveillance and removal of tumors may prevent or minimize complications related to mass effects, catecholamine hypersecretion, and malignant transformation.
#15 Pheochromocytoma secondary prevention – wikidoc
https://www.wikidoc.org/index.php/Pheochromocytoma_secondary_prevention
Effective measures for the secondary prevention of pheochromocytoma include biochemical screening for family members of MEN2 patients is mandatory and Genetic testing in first-degree relatives of a patient with proven germline RET mutation. […] According to the Endocrine Society, biochemical screening for pheochromocytoma in recommended among patients with: VHL syndrome- started at 5 years of age with biochemical surveillance every year for the rest of life. […] Genetic testing should be performed in: Patients with a family history of pheochromocytoma.
#16 Pheochromocytoma & Paraganglioma | Mays Cancer Center
https://cancer.uthscsa.edu/cancer-care/types-and-treatments/pheochromocytoma-and-paraganglioma
Pheo para can affect many organs and may require multiple treatments. Mays Cancer Center is home to the experts you need in one program. Our team includes surgeons, medical oncologists (doctors who treat cancer with medications), urologists, otolaryngologists (ear, nose and throat doctors) and others. […] If you are at risk but do not have tumors, we provide ongoing monitoring to check for the early signs of pheo para. […] If there are tumors, and you can have surgery, you undergo a procedure to remove them. […] If there are tumors and you are not eligible for surgery, you may receive other treatments, such as chemotherapy or clinical trials. […] If tumors come back after treatment, we explore additional treatment options, which may include clinical trials.
#17 Pheochromocytoma – USZ
https://www.usz.ch/en/disease/pheochromocytoma/
The causes of pheochromocytoma are unknown in many cases. Therefore, you cannot prevent the tumor in the adrenal medulla in sporadic cases. There are also no special early detection measures for pheochromocytoma. […] However, hereditary factors play a role in around 25 to 40 percent of people with a pheochromocytoma. Regular check-ups are advisable, for example for healthy family members with the corresponding genetic changes: […] If you have a known genetic disease such as neurofibromatosis type 1, multiple endocrine neoplasia type 2 a/b, Von Hippel-Lindau syndrome or paraganglioma syndrome, you must have your disease monitored closely and regularly by an endocrinologist. In this way, a pheochromocytoma can be detected and treated at an early stage. […] As pheochromocytoma is a rare disease, treatment in a specialized center is advisable. In our adrenal consultation at the USZ, you will be advised on the necessary clarifications (genetic examination) and the subsequent follow-up care (regular imaging, screening for other tumors depending on the underlying disease).
#18 Perioperative Management of Pheochromocytoma – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK589634/
Pre-op preparation and optimization are essential to decrease peri-operative morbidity and mortality in pheochromocytoma. […] Adequate pre-op optimization is mandatory to decrease perioperative morbidity and mortality. […] Pre-operative evaluation should focus on assessing end-organ damage due to elevated catecholamine levels. […] Pre-op preparation with alpha-adrenergic and beta blockade is recommended to control blood pressure and restore intravascular volume.
#19 Perioperative Management of Pheochromocytoma – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK589634/
Pre-op preparation and optimization are essential to decrease peri-operative morbidity and mortality in pheochromocytoma. […] Adequate pre-op optimization is mandatory to decrease perioperative morbidity and mortality. […] Pre-operative evaluation should focus on assessing end-organ damage due to elevated catecholamine levels. […] Pre-op preparation with alpha-adrenergic and beta blockade is recommended to control blood pressure and restore intravascular volume.
#20
https://www.clinicsinsurgery.com/full-text/cis-v2-id1517.php
Pheochromocytomas and paragangliomas are catecholamine-producing neoplasms that can cause life-threatening hemodynamic instability, particularly intraoperatively, when the tumor is manipulated. Preoperative medical management reduces both preoperative morbidity and mortality. The current review discusses the latest literature on preoperative management. Preoperative strategies include a nonselective alpha-antagonist, selective alpha antagonists, calcium channel blockers, tyrosine hydroxylase inhibitors, and fluid and salt loading. […] The goal of preoperative care is to prevent a sudden release of catecholamines or attenuate the response to a release of catecholamines perioperatively. There are many ways to achieve this. […] Preoperative preparation should be initiated, even in normotensive or asymptomatic patients.
#21
https://www.clinicsinsurgery.com/full-text/cis-v2-id1517.php
Pheochromocytomas and paragangliomas are catecholamine-producing neoplasms that can cause life-threatening hemodynamic instability, particularly intraoperatively, when the tumor is manipulated. Preoperative medical management reduces both preoperative morbidity and mortality. The current review discusses the latest literature on preoperative management. Preoperative strategies include a nonselective alpha-antagonist, selective alpha antagonists, calcium channel blockers, tyrosine hydroxylase inhibitors, and fluid and salt loading. […] The goal of preoperative care is to prevent a sudden release of catecholamines or attenuate the response to a release of catecholamines perioperatively. There are many ways to achieve this. […] Preoperative preparation should be initiated, even in normotensive or asymptomatic patients.
#22
https://www.clinicsinsurgery.com/full-text/cis-v2-id1517.php
Pheochromocytomas and paragangliomas are catecholamine-producing neoplasms that can cause life-threatening hemodynamic instability, particularly intraoperatively, when the tumor is manipulated. Preoperative medical management reduces both preoperative morbidity and mortality. The current review discusses the latest literature on preoperative management. Preoperative strategies include a nonselective alpha-antagonist, selective alpha antagonists, calcium channel blockers, tyrosine hydroxylase inhibitors, and fluid and salt loading. […] The goal of preoperative care is to prevent a sudden release of catecholamines or attenuate the response to a release of catecholamines perioperatively. There are many ways to achieve this. […] Preoperative preparation should be initiated, even in normotensive or asymptomatic patients.
#23
https://www.clinicsinsurgery.com/full-text/cis-v2-id1517.php
Pheochromocytomas and paragangliomas are catecholamine-producing neoplasms that can cause life-threatening hemodynamic instability, particularly intraoperatively, when the tumor is manipulated. Preoperative medical management reduces both preoperative morbidity and mortality. The current review discusses the latest literature on preoperative management. Preoperative strategies include a nonselective alpha-antagonist, selective alpha antagonists, calcium channel blockers, tyrosine hydroxylase inhibitors, and fluid and salt loading. […] The goal of preoperative care is to prevent a sudden release of catecholamines or attenuate the response to a release of catecholamines perioperatively. There are many ways to achieve this. […] Preoperative preparation should be initiated, even in normotensive or asymptomatic patients.
#24 Treatment – SDHB Coalition
https://sdhbcoalition.org/pheochromocytoma/treatment/
Preoperative blockade of hormonally functional PPGL to prevent cardiovascular complications is recommended, along with preoperative medical treatment to normalize blood pressure and heart rate combined with a high-sodium diet with fluid intake to prevent severe hypotension after removal of the tumor. […] Surgical resection of a tumor is the treatment of choice and usually results in a cure of the hypertension. Careful preoperative management is required to control blood pressure, heart rate, fluid volume, and to prevent intraoperative hypertensive crises.
#25 Treatment – SDHB Coalition
https://sdhbcoalition.org/pheochromocytoma/treatment/
Preoperative blockade of hormonally functional PPGL to prevent cardiovascular complications is recommended, along with preoperative medical treatment to normalize blood pressure and heart rate combined with a high-sodium diet with fluid intake to prevent severe hypotension after removal of the tumor. […] Surgical resection of a tumor is the treatment of choice and usually results in a cure of the hypertension. Careful preoperative management is required to control blood pressure, heart rate, fluid volume, and to prevent intraoperative hypertensive crises.
#26 Pheochromocytoma – USZ
https://www.usz.ch/en/disease/pheochromocytoma/
The causes of pheochromocytoma are unknown in many cases. Therefore, you cannot prevent the tumor in the adrenal medulla in sporadic cases. There are also no special early detection measures for pheochromocytoma. […] However, hereditary factors play a role in around 25 to 40 percent of people with a pheochromocytoma. Regular check-ups are advisable, for example for healthy family members with the corresponding genetic changes: […] If you have a known genetic disease such as neurofibromatosis type 1, multiple endocrine neoplasia type 2 a/b, Von Hippel-Lindau syndrome or paraganglioma syndrome, you must have your disease monitored closely and regularly by an endocrinologist. In this way, a pheochromocytoma can be detected and treated at an early stage. […] As pheochromocytoma is a rare disease, treatment in a specialized center is advisable. In our adrenal consultation at the USZ, you will be advised on the necessary clarifications (genetic examination) and the subsequent follow-up care (regular imaging, screening for other tumors depending on the underlying disease).
#27 Genetic Status and Clinical Management of Bilateral Pheochromocytoma | Lerner Research Institute
https://www.lerner.ccf.org/news/article/?title=Genetic+Status+and+Clinical+Management+of+Bilateral+Pheochromocytoma&id=93db6174b323d65ed95331182272f53f2dbae244
The current standard treatment for pheochromocytoma affecting both adrenals, is removal of both adrenal glands (total adrenalectomy). […] Recent data suggest that preserving as much of the adrenal cortex as possible (cortical-sparing adrenalectomy) can reduce the need for hormone replacement therapy, which in itself presents unique health risks. […] Furthermore, cortical-sparing adrenalectomy is often ideal for individuals with hereditary pheochromocytoma as they are at higher risk for developing new tumors that would require additional surgeries. […] Therefore, the research team recommends that all patients presenting with pheochromocytomas should be offered genetic analysis to guide surgical and medical management decisions. […] In summary, the multidisciplinary group of researchers concluded that cortical-sparing surgery can be appropriate and safe for certain cases of bilateral pheochromocytoma, particularly when a predisposing mutation is present. […] this study underscores the importance of genetic testing in diagnosing and managing pheochromocytoma.
#28 Genetic Status and Clinical Management of Bilateral Pheochromocytoma | Lerner Research Institute
https://www.lerner.ccf.org/news/article/?title=Genetic+Status+and+Clinical+Management+of+Bilateral+Pheochromocytoma&id=93db6174b323d65ed95331182272f53f2dbae244
The current standard treatment for pheochromocytoma affecting both adrenals, is removal of both adrenal glands (total adrenalectomy). […] Recent data suggest that preserving as much of the adrenal cortex as possible (cortical-sparing adrenalectomy) can reduce the need for hormone replacement therapy, which in itself presents unique health risks. […] Furthermore, cortical-sparing adrenalectomy is often ideal for individuals with hereditary pheochromocytoma as they are at higher risk for developing new tumors that would require additional surgeries. […] Therefore, the research team recommends that all patients presenting with pheochromocytomas should be offered genetic analysis to guide surgical and medical management decisions. […] In summary, the multidisciplinary group of researchers concluded that cortical-sparing surgery can be appropriate and safe for certain cases of bilateral pheochromocytoma, particularly when a predisposing mutation is present. […] this study underscores the importance of genetic testing in diagnosing and managing pheochromocytoma.
#29 Genetic Status and Clinical Management of Bilateral Pheochromocytoma | Lerner Research Institute
https://www.lerner.ccf.org/news/article/?title=Genetic+Status+and+Clinical+Management+of+Bilateral+Pheochromocytoma&id=93db6174b323d65ed95331182272f53f2dbae244
The current standard treatment for pheochromocytoma affecting both adrenals, is removal of both adrenal glands (total adrenalectomy). […] Recent data suggest that preserving as much of the adrenal cortex as possible (cortical-sparing adrenalectomy) can reduce the need for hormone replacement therapy, which in itself presents unique health risks. […] Furthermore, cortical-sparing adrenalectomy is often ideal for individuals with hereditary pheochromocytoma as they are at higher risk for developing new tumors that would require additional surgeries. […] Therefore, the research team recommends that all patients presenting with pheochromocytomas should be offered genetic analysis to guide surgical and medical management decisions. […] In summary, the multidisciplinary group of researchers concluded that cortical-sparing surgery can be appropriate and safe for certain cases of bilateral pheochromocytoma, particularly when a predisposing mutation is present. […] this study underscores the importance of genetic testing in diagnosing and managing pheochromocytoma.
#30 Genetic Status and Clinical Management of Bilateral Pheochromocytoma | Lerner Research Institute
https://www.lerner.ccf.org/news/article/?title=Genetic+Status+and+Clinical+Management+of+Bilateral+Pheochromocytoma&id=93db6174b323d65ed95331182272f53f2dbae244
The current standard treatment for pheochromocytoma affecting both adrenals, is removal of both adrenal glands (total adrenalectomy). […] Recent data suggest that preserving as much of the adrenal cortex as possible (cortical-sparing adrenalectomy) can reduce the need for hormone replacement therapy, which in itself presents unique health risks. […] Furthermore, cortical-sparing adrenalectomy is often ideal for individuals with hereditary pheochromocytoma as they are at higher risk for developing new tumors that would require additional surgeries. […] Therefore, the research team recommends that all patients presenting with pheochromocytomas should be offered genetic analysis to guide surgical and medical management decisions. […] In summary, the multidisciplinary group of researchers concluded that cortical-sparing surgery can be appropriate and safe for certain cases of bilateral pheochromocytoma, particularly when a predisposing mutation is present. […] this study underscores the importance of genetic testing in diagnosing and managing pheochromocytoma.
#31 Genetic Status and Clinical Management of Bilateral Pheochromocytoma | Lerner Research Institute
https://www.lerner.ccf.org/news/article/?title=Genetic+Status+and+Clinical+Management+of+Bilateral+Pheochromocytoma&id=93db6174b323d65ed95331182272f53f2dbae244
The current standard treatment for pheochromocytoma affecting both adrenals, is removal of both adrenal glands (total adrenalectomy). […] Recent data suggest that preserving as much of the adrenal cortex as possible (cortical-sparing adrenalectomy) can reduce the need for hormone replacement therapy, which in itself presents unique health risks. […] Furthermore, cortical-sparing adrenalectomy is often ideal for individuals with hereditary pheochromocytoma as they are at higher risk for developing new tumors that would require additional surgeries. […] Therefore, the research team recommends that all patients presenting with pheochromocytomas should be offered genetic analysis to guide surgical and medical management decisions. […] In summary, the multidisciplinary group of researchers concluded that cortical-sparing surgery can be appropriate and safe for certain cases of bilateral pheochromocytoma, particularly when a predisposing mutation is present. […] this study underscores the importance of genetic testing in diagnosing and managing pheochromocytoma.
#32 Genetic Status and Clinical Management of Bilateral Pheochromocytoma | Lerner Research Institute
https://www.lerner.ccf.org/news/article/?title=Genetic+Status+and+Clinical+Management+of+Bilateral+Pheochromocytoma&id=93db6174b323d65ed95331182272f53f2dbae244
The current standard treatment for pheochromocytoma affecting both adrenals, is removal of both adrenal glands (total adrenalectomy). […] Recent data suggest that preserving as much of the adrenal cortex as possible (cortical-sparing adrenalectomy) can reduce the need for hormone replacement therapy, which in itself presents unique health risks. […] Furthermore, cortical-sparing adrenalectomy is often ideal for individuals with hereditary pheochromocytoma as they are at higher risk for developing new tumors that would require additional surgeries. […] Therefore, the research team recommends that all patients presenting with pheochromocytomas should be offered genetic analysis to guide surgical and medical management decisions. […] In summary, the multidisciplinary group of researchers concluded that cortical-sparing surgery can be appropriate and safe for certain cases of bilateral pheochromocytoma, particularly when a predisposing mutation is present. […] this study underscores the importance of genetic testing in diagnosing and managing pheochromocytoma.
#33 Pheochromocytoma & Paraganglioma | Mays Cancer Center
https://cancer.uthscsa.edu/cancer-care/types-and-treatments/pheochromocytoma-and-paraganglioma
Pheo para can affect many organs and may require multiple treatments. Mays Cancer Center is home to the experts you need in one program. Our team includes surgeons, medical oncologists (doctors who treat cancer with medications), urologists, otolaryngologists (ear, nose and throat doctors) and others. […] If you are at risk but do not have tumors, we provide ongoing monitoring to check for the early signs of pheo para. […] If there are tumors, and you can have surgery, you undergo a procedure to remove them. […] If there are tumors and you are not eligible for surgery, you may receive other treatments, such as chemotherapy or clinical trials. […] If tumors come back after treatment, we explore additional treatment options, which may include clinical trials.
#34 Pheochromocytoma & Paraganglioma | Mays Cancer Center
https://cancer.uthscsa.edu/cancer-care/types-and-treatments/pheochromocytoma-and-paraganglioma
Pheo para can affect many organs and may require multiple treatments. Mays Cancer Center is home to the experts you need in one program. Our team includes surgeons, medical oncologists (doctors who treat cancer with medications), urologists, otolaryngologists (ear, nose and throat doctors) and others. […] If you are at risk but do not have tumors, we provide ongoing monitoring to check for the early signs of pheo para. […] If there are tumors, and you can have surgery, you undergo a procedure to remove them. […] If there are tumors and you are not eligible for surgery, you may receive other treatments, such as chemotherapy or clinical trials. […] If tumors come back after treatment, we explore additional treatment options, which may include clinical trials.
#35 Pheochromocytoma & Paraganglioma | Mays Cancer Center
https://cancer.uthscsa.edu/cancer-care/types-and-treatments/pheochromocytoma-and-paraganglioma
Pheo para can affect many organs and may require multiple treatments. Mays Cancer Center is home to the experts you need in one program. Our team includes surgeons, medical oncologists (doctors who treat cancer with medications), urologists, otolaryngologists (ear, nose and throat doctors) and others. […] If you are at risk but do not have tumors, we provide ongoing monitoring to check for the early signs of pheo para. […] If there are tumors, and you can have surgery, you undergo a procedure to remove them. […] If there are tumors and you are not eligible for surgery, you may receive other treatments, such as chemotherapy or clinical trials. […] If tumors come back after treatment, we explore additional treatment options, which may include clinical trials.