Zespół zollingera-ellisona – Rokowania, prognozy i postęp choroby

Zespół zollingera-ellisona
Rokowania, prognozy i postęp choroby

Rokowanie w zespole Zollingera-Ellisona (ZES) jest zróżnicowane i zależy od charakterystyki guza oraz stopnia zaawansowania choroby. Wczesne rozpoznanie i całkowite usunięcie guza chirurgicznie zapewnia ponad 80% przeżywalność po 15 latach, a 5-10-letnie przeżycie przekracza 90%. Czynniki pogarszające rokowanie to m.in. guzy trzustkowe, większe rozmiary guza, obecność przerzutów do wątroby, węzłów chłonnych lub kości, ektopowy zespół Cushinga oraz wysokie stężenia gastryny w surowicy. Pacjenci z ZES powiązanym z zespołem MEN wykazują lepsze przeżycie niż osoby ze sporadycznym ZES. Współczesne leczenie inhibitorami pompy protonowej (PPI) skutecznie kontroluje nadmierne wydzielanie kwasu, co zmniejsza powikłania związane z nadkwaśnością, a główną przyczyną zgonów pozostaje progresja guza gastrinoma.

Prognoza zespołu Zollingera-Ellisona (Zespół Zollingera-Ellisona)

Czynniki prognostyczne
Wskaźniki przeżywalności
Przyczyny zgonów
Wpływ leczenia na rokowanie
Powikłania ZES
Zalecenia dla pacjentów
Kolejne rozdziały

Prognoza zespołu Zollingera-Ellisona (Zespół Zollingera-Ellisona)

Rokowanie w zespole Zollingera-Ellisona (ZES) nie jest jednoznacznie określone i zależy od wielu czynników, w tym głównie od charakterystyki guza oraz stopnia zaawansowania choroby w momencie diagnozy. Długoterminowe rokowanie w przypadku odpowiedniego leczenia jest generalnie dobre, szczególnie u pacjentów bez choroby przerzutowej.¹² Wczesne rozpoznanie choroby zazwyczaj skutkuje ponad 80% przeżywalnością po 15 latach od diagnozy.³

Czynniki prognostyczne

Na rokowanie w zespole Zollingera-Ellisona wpływa szereg czynników, które determinują przebieg choroby i skuteczność leczenia:

Rodzaj guza – guzy niezłośliwe (łagodne gastrinoma) nie są zagrażające życiu, ale mogą wymagać długotrwałego leczenia w celu kontroli nadmiernego wydzielania kwasu żołądkowego⁴
Lokalizacja pierwotnego guza – guzy zlokalizowane w trzustce mają gorsze rokowanie niż guzy dwunastnicy⁵
Wielkość guza – większe guzy pierwotne wiążą się z gorszym rokowaniem⁶
Obecność przerzutów – szczególnie przerzuty do wątroby, węzłów chłonnych czy kości znacząco pogarszają rokowanie⁷
Zakres przerzutów do wątroby – stopień zajęcia wątroby przez przerzuty jest skorelowany ze wskaźnikiem przeżywalności⁸
Współistniejący zespół Cushinga – ektopowy zespół Cushinga znacząco pogarsza rokowanie⁹
Poziom gastryny – wyższe stężenia gastryny w surowicy krwi wiążą się z gorszym rokowaniem¹⁰

Wskaźniki przeżywalności

Wskaźniki przeżywalności w zespole Zollingera-Ellisona są zróżnicowane i zależą głównie od skuteczności leczenia chirurgicznego oraz stopnia zaawansowania choroby:

Ponad 90% pacjentów przeżywa 5-10 lat po operacji całkowitego usunięcia guzów gastrinoma¹¹
5-letni wskaźnik przeżycia spada do około 43% w przypadkach, gdy operacja nie usunie wszystkich guzów¹²
U pacjentów bez przerzutów, u których możliwa jest resekcja chirurgiczna, 15-letni wskaźnik przeżycia wynosi około 80%¹³
Pacjenci z ZES powiązanym z zespołem mnogiej gruczolakowatości wewnątrzwydzielniczej (MEN) mają dłuższe przeżycie w porównaniu z pacjentami ze sporadycznym ZES¹⁴

Przyczyny zgonów

W nowoczesnej erze leczenia, dzięki skutecznej kontroli wydzielania kwasu żołądkowego za pomocą inhibitorów pompy protonowej (PPI), główną przyczyną zgonów wśród pacjentów z ZES nie są już powikłania związane z nadmiernym wydzielaniem kwasu, ale wzrost guza gastrinoma:¹⁵

Około 31% pacjentów z długotrwałym ZES umiera, przy czym wszystkie zgony są związane z przyczynami nie związanymi z nadkwaśnością¹⁶
Połowa zgonów jest bezpośrednio związana z ZES (głównie z progresją guza), a druga połowa z przyczynami niezwiązanymi z ZES¹⁷
Pacjenci umierający z powodu ZES częściej mają duże guzy pierwotne, częściej guzy trzustkowe, przerzuty do węzłów chłonnych, wątroby lub kości, ektopowy zespół Cushinga lub wyższe poziomy gastryny¹⁸

Wpływ leczenia na rokowanie

Odpowiednie leczenie znacząco wpływa na poprawę rokowania w zespole Zollingera-Ellisona:

Leczenie chirurgiczne – chirurgiczne usunięcie guza pierwotnego (i ewentualnie jego przerzutów) z zamiarem wyleczenia powinno być zawsze przeprowadzane, jeśli jest to możliwe¹⁹
Inhibitory pompy protonowej (PPI) – są lekami z wyboru do hamowania wydzielania kwasu i kontroli objawów zespołu²⁰
Diagnostyka obrazowa – badania PET z użyciem galu-68 powinny być zawsze uwzględniane w diagnostyce gastrinoma, zarówno w celu identyfikacji guza pierwotnego, jak i oceny stopnia zaawansowania choroby²¹

Warto podkreślić, że całkowite wyleczenie chirurgiczne ZES jest rzadkie.²² Niestety, diagnoza często jest stawiana, gdy choroba jest zbyt zaawansowana dla skutecznego podejścia chirurgicznego.²³ W takich przypadkach pierwszym krokiem jest kontrola objawów zespołu, oparta głównie na stosowaniu inhibitorów pompy protonowej.

Powikłania ZES

Dzięki postępom w medycznym i chirurgicznym leczeniu zespołu Zollingera-Ellisona, zachorowalność i śmiertelność związana z tą chorobą jest obecnie niska. Mniej niż 5% pacjentów rozwija powikłania, takie jak:²⁴

Perforacja przewodu pokarmowego
Niedrożność odźwiernika
Zwężenie przełyku

Warto odnotować, że średni czas między wystąpieniem objawów a ostateczną diagnozą często przekracza 5 lat, a prawie 25% pacjentów ma przerzuty w momencie pierwszej diagnozy, co wiąże się z gorszym rokowaniem w porównaniu z pacjentami bez przerzutów.²⁵

Zalecenia dla pacjentów

Dla pacjentów z zespołem Zollingera-Ellisona ważne jest, aby pamiętać, że wskaźniki przeżywalności w raku są jedynie szacunkami. Pacjenci powinni konsultować się ze swoim lekarzem prowadzącym w celu uzyskania indywidualnej oceny rokowania i oczekiwanych wyników leczenia.²⁶ Regularne badania kontrolne i długoterminowa opieka medyczna są niezbędne dla optymalizacji wyników leczenia i jakości życia.

Kolejne rozdziały

Zapraszamy do dalszego czytania naszego leksykonu.

Wybierz kolejny rozdział z menu poniżej, aby otworzyć nową podstronę kompedium wiedzy i uzyskać szczegółowe informację o leku, substancji lub chorobie.

Materiały źródłowe

#1 Long-term prognosis of Zollinger-Ellison syndrome in multiple endocrine neoplasia – PubMed
https://pubmed.ncbi.nlm.nih.gov/7903006/
The long-term prognosis of Zollinger-Ellison Syndrome (ZES) is not well defined. […] Long-term prognosis is good. Survival is longer for patients with ZES and MEN compared with patients with sporadic ZES. […] Surgical cure is rare.
#2 Zollinger-Ellison Syndrome: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/183555-overview
Prognosis is excellent in patients with Zollinger-Ellison syndrome (ZES) without metastatic disease. Early recognition generally results in more than 80% survival at 15 years. […] In general, the morbidity and mortality of ZES is low because of improved medical and surgical management of the disease. Fewer than 5% of patients develop a complication, such as abdominal perforation, gastric outlet obstruction, or esophageal stricture.
#3 Zollinger-Ellison Syndrome: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/183555-overview
Prognosis is excellent in patients with Zollinger-Ellison syndrome (ZES) without metastatic disease. Early recognition generally results in more than 80% survival at 15 years. […] In general, the morbidity and mortality of ZES is low because of improved medical and surgical management of the disease. Fewer than 5% of patients develop a complication, such as abdominal perforation, gastric outlet obstruction, or esophageal stricture.
#4 Zollinger-Ellison Syndrome: Symptoms & Treatment
https://my.clevelandclinic.org/health/diseases/17960-zollinger-ellison-syndrome
Zollinger-Ellison syndrome can be life-threatening. […] Your prognosis, or what you can expect after treatment, depends on the tumor type. Noncancerous gastrinoma tumors aren’t life-threatening. You may need ongoing treatment to manage stomach acid levels. […] The situation changes if you have cancerous tumors. In that case, you may need surgery and follow-up cancer treatment. […] Zollinger-Ellison survival rates vary. Experts estimate more than 90% of people will live between five and 10 years after surgery removes all gastrinoma tumors. The five-year survival rate estimate drops to 43% in cases where surgery doesn’t remove all tumors. It’s important to remember that cancer survival rates are estimates. If you have ZES, ask your healthcare provider what you can expect.
#5 Prospective study of the clinical course, prognostic factors, causes of death, and survival in patients with long-standing Zollinger-Ellison syndrome – PubMed
https://pubmed.ncbi.nlm.nih.gov/10080607/
Thirty-one percent of patients died, all of non-acid-related causes. […] One half died of a ZES-related cause; they differed from those who died of non-ZES deaths by having a large primary tumor, more frequently a pancreatic tumor; lymph node, liver, or bone metastases; ectopic Cushing’s syndrome; or higher gastrin levels. […] The extent of liver metastases correlated with survival rate. […] In ZES, gastrinoma growth is now the main single determinant of long-term survival, with one half of patients dying a gastrinoma-related death and none an acid-related death. […] Large primary tumors that are pancreatic in location, the development of liver metastases, (especially if associated with bone metastases or Cushing’s syndrome), and the extent of liver metastases are all important prognostic factors.
#6 Prospective study of the clinical course, prognostic factors, causes of death, and survival in patients with long-standing Zollinger-Ellison syndrome – PubMed
https://pubmed.ncbi.nlm.nih.gov/10080607/
Thirty-one percent of patients died, all of non-acid-related causes. […] One half died of a ZES-related cause; they differed from those who died of non-ZES deaths by having a large primary tumor, more frequently a pancreatic tumor; lymph node, liver, or bone metastases; ectopic Cushing’s syndrome; or higher gastrin levels. […] The extent of liver metastases correlated with survival rate. […] In ZES, gastrinoma growth is now the main single determinant of long-term survival, with one half of patients dying a gastrinoma-related death and none an acid-related death. […] Large primary tumors that are pancreatic in location, the development of liver metastases, (especially if associated with bone metastases or Cushing’s syndrome), and the extent of liver metastases are all important prognostic factors.
#7 Prospective study of the clinical course, prognostic factors, causes of death, and survival in patients with long-standing Zollinger-Ellison syndrome – PubMed
https://pubmed.ncbi.nlm.nih.gov/10080607/
Thirty-one percent of patients died, all of non-acid-related causes. […] One half died of a ZES-related cause; they differed from those who died of non-ZES deaths by having a large primary tumor, more frequently a pancreatic tumor; lymph node, liver, or bone metastases; ectopic Cushing’s syndrome; or higher gastrin levels. […] The extent of liver metastases correlated with survival rate. […] In ZES, gastrinoma growth is now the main single determinant of long-term survival, with one half of patients dying a gastrinoma-related death and none an acid-related death. […] Large primary tumors that are pancreatic in location, the development of liver metastases, (especially if associated with bone metastases or Cushing’s syndrome), and the extent of liver metastases are all important prognostic factors.
#8 Prospective study of the clinical course, prognostic factors, causes of death, and survival in patients with long-standing Zollinger-Ellison syndrome – PubMed
https://pubmed.ncbi.nlm.nih.gov/10080607/
Thirty-one percent of patients died, all of non-acid-related causes. […] One half died of a ZES-related cause; they differed from those who died of non-ZES deaths by having a large primary tumor, more frequently a pancreatic tumor; lymph node, liver, or bone metastases; ectopic Cushing’s syndrome; or higher gastrin levels. […] The extent of liver metastases correlated with survival rate. […] In ZES, gastrinoma growth is now the main single determinant of long-term survival, with one half of patients dying a gastrinoma-related death and none an acid-related death. […] Large primary tumors that are pancreatic in location, the development of liver metastases, (especially if associated with bone metastases or Cushing’s syndrome), and the extent of liver metastases are all important prognostic factors.
#9 Prospective study of the clinical course, prognostic factors, causes of death, and survival in patients with long-standing Zollinger-Ellison syndrome – PubMed
https://pubmed.ncbi.nlm.nih.gov/10080607/
Thirty-one percent of patients died, all of non-acid-related causes. […] One half died of a ZES-related cause; they differed from those who died of non-ZES deaths by having a large primary tumor, more frequently a pancreatic tumor; lymph node, liver, or bone metastases; ectopic Cushing’s syndrome; or higher gastrin levels. […] The extent of liver metastases correlated with survival rate. […] In ZES, gastrinoma growth is now the main single determinant of long-term survival, with one half of patients dying a gastrinoma-related death and none an acid-related death. […] Large primary tumors that are pancreatic in location, the development of liver metastases, (especially if associated with bone metastases or Cushing’s syndrome), and the extent of liver metastases are all important prognostic factors.
#10 Prospective study of the clinical course, prognostic factors, causes of death, and survival in patients with long-standing Zollinger-Ellison syndrome – PubMed
https://pubmed.ncbi.nlm.nih.gov/10080607/
Thirty-one percent of patients died, all of non-acid-related causes. […] One half died of a ZES-related cause; they differed from those who died of non-ZES deaths by having a large primary tumor, more frequently a pancreatic tumor; lymph node, liver, or bone metastases; ectopic Cushing’s syndrome; or higher gastrin levels. […] The extent of liver metastases correlated with survival rate. […] In ZES, gastrinoma growth is now the main single determinant of long-term survival, with one half of patients dying a gastrinoma-related death and none an acid-related death. […] Large primary tumors that are pancreatic in location, the development of liver metastases, (especially if associated with bone metastases or Cushing’s syndrome), and the extent of liver metastases are all important prognostic factors.
#11 Zollinger-Ellison Syndrome: Symptoms & Treatment
https://my.clevelandclinic.org/health/diseases/17960-zollinger-ellison-syndrome
Zollinger-Ellison syndrome can be life-threatening. […] Your prognosis, or what you can expect after treatment, depends on the tumor type. Noncancerous gastrinoma tumors aren’t life-threatening. You may need ongoing treatment to manage stomach acid levels. […] The situation changes if you have cancerous tumors. In that case, you may need surgery and follow-up cancer treatment. […] Zollinger-Ellison survival rates vary. Experts estimate more than 90% of people will live between five and 10 years after surgery removes all gastrinoma tumors. The five-year survival rate estimate drops to 43% in cases where surgery doesn’t remove all tumors. It’s important to remember that cancer survival rates are estimates. If you have ZES, ask your healthcare provider what you can expect.
#12 Zollinger-Ellison Syndrome: Symptoms & Treatment
https://my.clevelandclinic.org/health/diseases/17960-zollinger-ellison-syndrome
Zollinger-Ellison syndrome can be life-threatening. […] Your prognosis, or what you can expect after treatment, depends on the tumor type. Noncancerous gastrinoma tumors aren’t life-threatening. You may need ongoing treatment to manage stomach acid levels. […] The situation changes if you have cancerous tumors. In that case, you may need surgery and follow-up cancer treatment. […] Zollinger-Ellison survival rates vary. Experts estimate more than 90% of people will live between five and 10 years after surgery removes all gastrinoma tumors. The five-year survival rate estimate drops to 43% in cases where surgery doesn’t remove all tumors. It’s important to remember that cancer survival rates are estimates. If you have ZES, ask your healthcare provider what you can expect.
#13 Gastrinoma and Zollinger Ellison syndrome: A roadmap for the management between new and old therapies
https://www.wjgnet.com/1007-9327/full/v27/i35/5890.htm
Zollinger-Ellison syndrome (ZES) associated with pancreatic or duodenal gastrinoma is characterized by gastric acid hypersecretion, which typically leads to gastroesophageal reflux disease, recurrent peptic ulcers, and chronic diarrhea. […] The average time between the onset of symptoms and the final diagnosis is often longer than 5 years, and nearly 25% of patients are metastatic at the first diagnosis and show a worse prognosis when compared to non-metastatic patients in whom the surgical management is associated with a promising 15-year survival rate of 80%. […] The role of surgery in the treatment of gastrinoma has changed completely from the introduction of PPIs in the 1980s. […] Surgical removal of the primary tumor (and possibly its metastases) with curative intent should be, indeed, always performed.
#14 Long-term prognosis of Zollinger-Ellison syndrome in multiple endocrine neoplasia – PubMed
https://pubmed.ncbi.nlm.nih.gov/7903006/
The long-term prognosis of Zollinger-Ellison Syndrome (ZES) is not well defined. […] Long-term prognosis is good. Survival is longer for patients with ZES and MEN compared with patients with sporadic ZES. […] Surgical cure is rare.
#15 Prospective study of the clinical course, prognostic factors, causes of death, and survival in patients with long-standing Zollinger-Ellison syndrome – PubMed
https://pubmed.ncbi.nlm.nih.gov/10080607/
Thirty-one percent of patients died, all of non-acid-related causes. […] One half died of a ZES-related cause; they differed from those who died of non-ZES deaths by having a large primary tumor, more frequently a pancreatic tumor; lymph node, liver, or bone metastases; ectopic Cushing’s syndrome; or higher gastrin levels. […] The extent of liver metastases correlated with survival rate. […] In ZES, gastrinoma growth is now the main single determinant of long-term survival, with one half of patients dying a gastrinoma-related death and none an acid-related death. […] Large primary tumors that are pancreatic in location, the development of liver metastases, (especially if associated with bone metastases or Cushing’s syndrome), and the extent of liver metastases are all important prognostic factors.
#16 Prospective study of the clinical course, prognostic factors, causes of death, and survival in patients with long-standing Zollinger-Ellison syndrome – PubMed
https://pubmed.ncbi.nlm.nih.gov/10080607/
Thirty-one percent of patients died, all of non-acid-related causes. […] One half died of a ZES-related cause; they differed from those who died of non-ZES deaths by having a large primary tumor, more frequently a pancreatic tumor; lymph node, liver, or bone metastases; ectopic Cushing’s syndrome; or higher gastrin levels. […] The extent of liver metastases correlated with survival rate. […] In ZES, gastrinoma growth is now the main single determinant of long-term survival, with one half of patients dying a gastrinoma-related death and none an acid-related death. […] Large primary tumors that are pancreatic in location, the development of liver metastases, (especially if associated with bone metastases or Cushing’s syndrome), and the extent of liver metastases are all important prognostic factors.
#17 Prospective study of the clinical course, prognostic factors, causes of death, and survival in patients with long-standing Zollinger-Ellison syndrome – PubMed
https://pubmed.ncbi.nlm.nih.gov/10080607/
Thirty-one percent of patients died, all of non-acid-related causes. […] One half died of a ZES-related cause; they differed from those who died of non-ZES deaths by having a large primary tumor, more frequently a pancreatic tumor; lymph node, liver, or bone metastases; ectopic Cushing’s syndrome; or higher gastrin levels. […] The extent of liver metastases correlated with survival rate. […] In ZES, gastrinoma growth is now the main single determinant of long-term survival, with one half of patients dying a gastrinoma-related death and none an acid-related death. […] Large primary tumors that are pancreatic in location, the development of liver metastases, (especially if associated with bone metastases or Cushing’s syndrome), and the extent of liver metastases are all important prognostic factors.
#18 Prospective study of the clinical course, prognostic factors, causes of death, and survival in patients with long-standing Zollinger-Ellison syndrome – PubMed
https://pubmed.ncbi.nlm.nih.gov/10080607/
Thirty-one percent of patients died, all of non-acid-related causes. […] One half died of a ZES-related cause; they differed from those who died of non-ZES deaths by having a large primary tumor, more frequently a pancreatic tumor; lymph node, liver, or bone metastases; ectopic Cushing’s syndrome; or higher gastrin levels. […] The extent of liver metastases correlated with survival rate. […] In ZES, gastrinoma growth is now the main single determinant of long-term survival, with one half of patients dying a gastrinoma-related death and none an acid-related death. […] Large primary tumors that are pancreatic in location, the development of liver metastases, (especially if associated with bone metastases or Cushing’s syndrome), and the extent of liver metastases are all important prognostic factors.
#19 Gastrinoma and Zollinger Ellison syndrome: A roadmap for the management between new and old therapies
https://www.wjgnet.com/1007-9327/full/v27/i35/5890.htm
Zollinger-Ellison syndrome (ZES) associated with pancreatic or duodenal gastrinoma is characterized by gastric acid hypersecretion, which typically leads to gastroesophageal reflux disease, recurrent peptic ulcers, and chronic diarrhea. […] The average time between the onset of symptoms and the final diagnosis is often longer than 5 years, and nearly 25% of patients are metastatic at the first diagnosis and show a worse prognosis when compared to non-metastatic patients in whom the surgical management is associated with a promising 15-year survival rate of 80%. […] The role of surgery in the treatment of gastrinoma has changed completely from the introduction of PPIs in the 1980s. […] Surgical removal of the primary tumor (and possibly its metastases) with curative intent should be, indeed, always performed.
#20 Gastrinoma and Zollinger Ellison syndrome: A roadmap for the management between new and old therapies
https://www.wjgnet.com/1007-9327/full/v27/i35/5890.htm
Unfortunately, the diagnosis is often made when the disease is too advanced for a surgical approach. […] The first step, again, is represented by syndrome control, based on PPIs, which are considered to be the drugs of choice for suppressing acid secretion. […] Given the high expression of STTRs in gastrinomas, gallium-68PET-scan should be always included in the diagnostic pathway of all NENs, including gastrinoma, in order to both identify the primary tumor and to stage the disease.
#21 Gastrinoma and Zollinger Ellison syndrome: A roadmap for the management between new and old therapies
https://www.wjgnet.com/1007-9327/full/v27/i35/5890.htm
Unfortunately, the diagnosis is often made when the disease is too advanced for a surgical approach. […] The first step, again, is represented by syndrome control, based on PPIs, which are considered to be the drugs of choice for suppressing acid secretion. […] Given the high expression of STTRs in gastrinomas, gallium-68PET-scan should be always included in the diagnostic pathway of all NENs, including gastrinoma, in order to both identify the primary tumor and to stage the disease.
#22 Long-term prognosis of Zollinger-Ellison syndrome in multiple endocrine neoplasia – PubMed
https://pubmed.ncbi.nlm.nih.gov/7903006/
The long-term prognosis of Zollinger-Ellison Syndrome (ZES) is not well defined. […] Long-term prognosis is good. Survival is longer for patients with ZES and MEN compared with patients with sporadic ZES. […] Surgical cure is rare.
#23 Gastrinoma and Zollinger Ellison syndrome: A roadmap for the management between new and old therapies
https://www.wjgnet.com/1007-9327/full/v27/i35/5890.htm
Unfortunately, the diagnosis is often made when the disease is too advanced for a surgical approach. […] The first step, again, is represented by syndrome control, based on PPIs, which are considered to be the drugs of choice for suppressing acid secretion. […] Given the high expression of STTRs in gastrinomas, gallium-68PET-scan should be always included in the diagnostic pathway of all NENs, including gastrinoma, in order to both identify the primary tumor and to stage the disease.
#24 Zollinger-Ellison Syndrome: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/183555-overview
Prognosis is excellent in patients with Zollinger-Ellison syndrome (ZES) without metastatic disease. Early recognition generally results in more than 80% survival at 15 years. […] In general, the morbidity and mortality of ZES is low because of improved medical and surgical management of the disease. Fewer than 5% of patients develop a complication, such as abdominal perforation, gastric outlet obstruction, or esophageal stricture.
#25 Gastrinoma and Zollinger Ellison syndrome: A roadmap for the management between new and old therapies
https://www.wjgnet.com/1007-9327/full/v27/i35/5890.htm
Zollinger-Ellison syndrome (ZES) associated with pancreatic or duodenal gastrinoma is characterized by gastric acid hypersecretion, which typically leads to gastroesophageal reflux disease, recurrent peptic ulcers, and chronic diarrhea. […] The average time between the onset of symptoms and the final diagnosis is often longer than 5 years, and nearly 25% of patients are metastatic at the first diagnosis and show a worse prognosis when compared to non-metastatic patients in whom the surgical management is associated with a promising 15-year survival rate of 80%. […] The role of surgery in the treatment of gastrinoma has changed completely from the introduction of PPIs in the 1980s. […] Surgical removal of the primary tumor (and possibly its metastases) with curative intent should be, indeed, always performed.
#26 Zollinger-Ellison Syndrome: Symptoms & Treatment
https://my.clevelandclinic.org/health/diseases/17960-zollinger-ellison-syndrome
Zollinger-Ellison syndrome can be life-threatening. […] Your prognosis, or what you can expect after treatment, depends on the tumor type. Noncancerous gastrinoma tumors aren’t life-threatening. You may need ongoing treatment to manage stomach acid levels. […] The situation changes if you have cancerous tumors. In that case, you may need surgery and follow-up cancer treatment. […] Zollinger-Ellison survival rates vary. Experts estimate more than 90% of people will live between five and 10 years after surgery removes all gastrinoma tumors. The five-year survival rate estimate drops to 43% in cases where surgery doesn’t remove all tumors. It’s important to remember that cancer survival rates are estimates. If you have ZES, ask your healthcare provider what you can expect.