Zespół zollingera-ellisona
Etiologia i przyczyny

Zespół Zollingera-Ellisona (ZES) to rzadkie schorzenie neuroendokrynne, charakteryzujące się obecnością gastrinoma – guzów wydzielających nadmierne ilości gastryny, najczęściej zlokalizowanych w dwunastnicy (50-85%) lub trzustce (25-30%). Nadprodukcja gastryny prowadzi do hipersekrecji kwasu solnego, co skutkuje owrzodzeniami przewodu pokarmowego, refluksem żołądkowo-przełykowym oraz biegunką. Około 20-30% przypadków ZES jest związanych z zespołem MEN-1, dziedzicznym zaburzeniem autosomalnym dominującym, obejmującym guzy przytarczyc, przysadki i innych narządów endokrynnych. Diagnostyka powinna uwzględniać wywiad rodzinny i badania genetyczne w kierunku mutacji genu MEN1 (11q13). W pozostałych 70-80% przypadków ZES występuje sporadycznie, prawdopodobnie w wyniku somatycznych mutacji genetycznych.

Etiologia Zespołu Zollingera-Ellisona

Zespół Zollingera-Ellisona (ZES) to rzadkie schorzenie wywołane przez obecność guzów zwanych gastrinoma, które wydzielają nadmierne ilości hormonu gastryny. Guzy te najczęściej rozwijają się w trzustce lub dwunastnicy, rzadziej w innych lokalizacjach. Podwyższone stężenie gastryny prowadzi do nadmiernej produkcji kwasu żołądkowego, co skutkuje rozwojem owrzodzeń, refluksu żołądkowo-przełykowego oraz biegunki.12

Podstawowe mechanizmy rozwoju zespołu

Sekwencja zdarzeń w zespole Zollingera-Ellisona zazwyczaj przebiega według określonego schematu. Zespół rozpoczyna się, gdy w trzustce lub dwunastnicy powstaje jeden lub więcej guzów. Guzy te są zbudowane z komórek wydzielających duże ilości hormonu gastryny, dlatego nazywane są gastrinoma. Podwyższony poziom gastryny powoduje, że żołądek produkuje zbyt dużo kwasu, co prowadzi do uszkodzenia błony śluzowej przewodu pokarmowego.12

Gastrinoma to guzy neuroendokrynne, które powstają najczęściej w następujących lokalizacjach:

  • Dwunastnica (50-85% przypadków)
  • Trzustka (25-30% przypadków)
  • Rzadziej w innych lokalizacjach: węzły chłonne, wątroba, śledziona, jajniki, serce, żołądek, płuca12

Zwiększone wydzielanie gastryny stymuluje komórki okładzinowe żołądka do maksymalnej aktywności, co prowadzi do hipersekreji kwasu solnego. Nadprodukcja kwasu powoduje uszkodzenie błony śluzowej przewodu pokarmowego, a w konsekwencji owrzodzenia, które najczęściej lokalizują się w dwunastnicy i bliższej części jelita czczego.12

Potencjał złośliwy gastrinoma

Gastrinoma mogą występować jako pojedyncze guzy lub mnogie zmiany. Około połowa do dwóch trzecich pojedynczych gastrinoma to guzy złośliwe (nowotwory). Te złośliwe zmiany często dają przerzuty do wątroby i okolicznych węzłów chłonnych. Jeśli nowotwór się rozprzestrzenia, najczęściej zajmuje węzły chłonne w pobliżu guza pierwotnego, a następnie wątrobę i kości.12

W przypadku przerzutów do wątroby należy rozważyć operację, jeśli wszystkie zidentyfikowane guzy można bezpiecznie usunąć. Zabieg chirurgiczny w celu usunięcia złośliwych gastrinoma może wyleczyć chorobę, jednak wyleczenie zależy od usunięcia wszystkich komórek nowotworowych w organizmie. Nawet po skutecznym zabiegu pacjent będzie musiał przyjmować leki kontrolujące wydzielanie kwasu żołądkowego przez całe życie.12

Czynniki genetyczne w zespole Zollingera-Ellisona

Związek z zespołem MEN-1

Około 20-30% przypadków zespołu Zollingera-Ellisona występuje w ramach dziedzicznego zaburzenia zwanego zespołem wielogruczołowej neoplazji wewnątrzwydzielniczej typu 1 (MEN-1, Multiple Endocrine Neoplasia type 1). Jest to rzadkie schorzenie dziedziczone autosomalnie dominująco, charakteryzujące się rozwojem guzów w różnych gruczołach endokrynnych.12

U pacjentów z zespołem MEN-1 gastrinoma występują obok guzów innych gruczołów:

  • Przytarczyc (najczęściej)
  • Przysadki mózgowej
  • Innych narządów endokrynnych12

W przypadku podejrzenia zespołu Zollingera-Ellisona należy przeprowadzić diagnostykę w kierunku zespołu MEN-1, w tym zebrać wywiad rodzinny. Objawy MEN-1 mogą obejmować podwyższone poziomy hormonów we krwi, kamienie nerkowe, cukrzycę, osłabienie mięśni, osłabienie kości i złamania.12

Mutacje genetyczne

Zespół MEN-1 jest spowodowany mutacjami w genie MEN1 (11q13) kodującym meninę, białko, które wiąże i reguluje aktywność licznych czynników transkrypcyjnych. Menina jest białkiem supresorowym guza, które pomaga zapobiegać rozwojowi guzów w trzustce i innych narządach. Gdy gen ten ulega mutacji, może prowadzić do rozwoju trzustkowego gastrinoma i innych typów guzów.12

Osoby, których rodzic ma zespół MEN-1, mają 50% szans na odziedziczenie tej mutacji i rozwój zespołu Zollingera-Ellisona. Jeśli pacjent ma krewnego pierwszego stopnia (rodzica lub rodzeństwo) z MEN-1, istnieje większe prawdopodobieństwo wystąpienia u niego zespołu Zollingera-Ellisona.12

Poradnictwo genetyczne jest zalecane dla rodzin z historią zaburzeń endokrynologicznych, zwłaszcza gdy występuje podejrzenie zespołu MEN-1. Badanie genetyczne może pomóc zidentyfikować osoby z podwyższonym ryzykiem rozwoju gastrinoma i innych zaburzeń endokrynologicznych.1

Przypadki sporadyczne zespołu Zollingera-Ellisona

W około 70-80% przypadków zespół Zollingera-Ellisona występuje sporadycznie, czyli bez wykrywalnej przyczyny genetycznej lub rodzinnej. W tych przypadkach dokładna etiologia powstawania gastrinoma pozostaje nieznana.12

Badacze sugerują, że przyczyną mogą być somatyczne mutacje genetyczne prowadzące do niekontrolowanego podziału i rozmnażania komórek produkujących gastrynę, jednak badania mające na celu zidentyfikowanie dokładnej przyczyny tego zjawiska nadal trwają.12

Leczenie przypadków sporadycznych różni się od podejścia do pacjentów z zespołem MEN-1. W przypadku sporadycznych gastrinoma stosuje się zarówno supresję kwasu, jak i chirurgiczne usunięcie guza, podczas gdy w przypadku zespołu MEN-1 leczenie często ogranicza się do samej supresji kwasu.1

Epidemiologia i czynniki ryzyka

Zespół Zollingera-Ellisona jest rzadkim schorzeniem. Szacuje się, że gastrinoma dotykają od 1 do 2 osób na milion rocznie. Większość lekarzy nigdy nie spotyka się z przypadkiem tego zespołu w swojej praktyce klinicznej.1

Do czynników ryzyka rozwoju zespołu Zollingera-Ellisona należą:

  • Zespół MEN-1 w wywiadzie rodzinnym
  • Historia rodzinna owrzodzeń przewodu pokarmowego
  • Płeć męska (ZES występuje częściej u mężczyzn)
  • Wiek między 20 a 50 lat12

Osoby z historią rodzinną zespołu ZES lub MEN-1 powinny być świadome zwiększonego ryzyka i omówić odpowiednie badania przesiewowe z lekarzem. Wczesne wykrycie zespołu Zollingera-Ellisona jest kluczowe dla skutecznego leczenia i zapobiegania powikłaniom.1

Patofizjologia zespołu Zollingera-Ellisona

Patofizjologia zespołu Zollingera-Ellisona opiera się na nadmiernym wydzielaniu gastryny przez guzy neuroendokrynne, co prowadzi do kaskady zdarzeń w przewodzie pokarmowym.1

Mechanizm działania gastryny

W warunkach fizjologicznych gastryna jest wydzielana w małych ilościach po spożyciu posiłku i stymuluje produkcję kwasu żołądkowego. W zespole Zollingera-Ellisona gastrinoma wydzielają duże ilości gastryny, co prowadzi do:12

  • Hipertrofii błony śluzowej żołądka
  • Zwiększenia liczby komórek okładzinowych
  • Zwiększenia podstawowego wydzielania kwasu
  • Nadprodukcji kwasu żołądkowego1

Nadmierna produkcja kwasu prowadzi do uszkodzenia błony śluzowej przewodu pokarmowego, powodując owrzodzenia, krwawienia i ciężki refluks żołądkowo-przełykowy. Dodatkowo prowadzi do biegunki i zaburzeń wchłaniania.12

Zaburzenia wchłaniania

Zaburzenia wchłaniania w zespole Zollingera-Ellisona wynikają z kilku mechanizmów:1

Te mechanizmy przyczyniają się do przewlekłej biegunki, która jest jednym z charakterystycznych objawów zespołu Zollingera-Ellisona.1

Historia odkrycia zespołu

Zespół Zollingera-Ellisona został po raz pierwszy opisany w 1955 roku. Doktorzy Zollinger i Ellison teoretyzowali, że hormon wydzielany z guzów trzustki powoduje nadprodukcję kwasu żołądkowego, co prowadzi do zespołu owrzodzeń. W 1960 roku, Dr Roderic Gregory i Dr Hilda Tracy wyizolowali substancję podobną do gastryny z trzustki pacjenta z zespołem Zollingera-Ellisona, ustalając gastrynę jako hormon odpowiedzialny za ten zespół.1

Zrozumienie patofizjologii zespołu Zollingera-Ellisona doprowadziło do opracowania skutecznych metod leczenia, w tym inhibitorów pompy protonowej, które są bardzo efektywne w kontrolowaniu wydzielania kwasu żołądkowego.1

Leczenie zespołu Zollingera-Ellisona obejmuje zarówno odpowiednią terapię medyczną w celu złagodzenia objawów, jak i interwencję chirurgiczną z zamiarem wyleczenia, gdy jest to możliwe. Szczególnie ważne jest podejście multidyscyplinarne, z ścisłą współpracą między klinicystami a chirurgami, co pozostaje podstawą właściwego postępowania w tej złożonej chorobie.12

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

Materiały źródłowe

  • #1 Zollinger-Ellison syndrome – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/zollinger-ellison-syndrome/symptoms-causes/syc-20379042
    Zollinger-Ellison syndrome is a condition in which one or more tumors grow in the pancreas or small intestine. The tumors, called gastrinomas, produce large amounts of the hormone gastrin. Gastrin causes the stomach to produce too much acid, which leads to peptic ulcers. High gastrin levels also can cause diarrhea, belly pain and other symptoms. […] The exact cause of Zollinger-Ellison syndrome isn’t known. But the pattern of events that occurs in Zollinger-Ellison syndrome typically follows the same sequence. The syndrome begins when one or more tumors form in the pancreas or a part of the small intestine called the duodenum. […] The tumors that form with Zollinger-Ellison syndrome are made up of cells that secrete large amounts of the hormone gastrin. For this reason, they are sometimes called gastrinomas. Increased gastrin causes the stomach to make far too much acid. The excess acid then leads to peptic ulcers and sometimes to diarrhea.
  • #1 Orphanet: Zollinger-Ellison syndrome
    https://www.orpha.net/en/disease/detail/913
    ZES is caused by a gastrin-secreting tumor (gastrinoma), usually located in the duodenum (50-85%), pancreas, abdominal lymph nodes or, in rare cases, ectopic locations (heart, ovary, liver, mesentery, etc.). […] MEN1 is caused by mutations in MEN1 (11q13) encoding menin, a protein which binds and regulates the activity of numerous transcription factors.
  • #1 Zollinger-Ellison Syndrome: Background, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/183555-overview
    Zollinger-Ellison syndrome (ZES) is caused by a nonbeta islet cell, gastrin-secreting tumor of the pancreas that stimulates the acid-secreting cells of the stomach to maximal activity, with consequent gastrointestinal mucosal ulceration. […] ZES may occur sporadically or as part of multiple endocrine neoplasia-type 1 (MEN 1).
  • #1 Zollinger-Ellison syndrome Information | Mount Sinai – New York
    https://www.mountsinai.org/health-library/diseases-conditions/zollinger-ellison-syndrome
    Zollinger-Ellison syndrome is a condition in which the body produces too much of the hormone gastrin. Most of the time, a small tumor (gastrinoma) in the pancreas or small intestine is the source of the extra gastrin in the blood. […] Zollinger-Ellison syndrome is caused by tumors called gastrinomas. These growths are most often found in the head of the pancreas and the upper small intestine. High levels of gastrin cause production of too much stomach acid. […] Gastrinomas may occur as single tumors or several tumors. One half to two thirds of single gastrinomas are cancerous (malignant) tumors. These tumors often spread to the liver and nearby lymph nodes. […] Many people with gastrinomas have several tumors as part of a condition called multiple endocrine neoplasia type I (MEN I). Tumors may develop in the pituitary gland (brain) and parathyroid gland (neck) as well as in the pancreas.
  • #1 Zollinger-Ellison Syndrome: Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/17960-zollinger-ellison-syndrome
    Zollinger-Ellison syndrome is a rare condition that causes tumors called gastrinomas to grow in your pancreas or intestine. Gastrinomas release the hormone gastrin, which triggers your stomach to produce gastric acid. Too much stomach acid leads to ulcers and other complications. Some gastrinomas are also cancerous. […] Most cases of Zollinger-Ellison syndrome happen sporadically (for no known reason). About 25% of people with this condition have MEN Type 1. MEN stands for multiple endocrine neoplasia. MEN is a rare inherited disorder that affects glands in your endocrine system. […] Zollinger-Ellison syndrome can be life-threatening. For example, a gastrinoma tumor that blocks your duodenum can make it rupture. The tumor can spread to your liver. Large amounts of gastric acid may lead to severe gastrointestinal bleeding. […] Surgery to remove cancerous gastrinomas may cure the condition. But a cure depends on removing all cancerous cells in your body. If surgery is successful, you’ll need to take medication to manage gastric acid for the rest of your life.
  • #1 Zollinger-Ellison Syndrome – NIDDK
    https://www.niddk.nih.gov/health-information/digestive-diseases/zollinger-ellison-syndrome
    Tumors, called gastrinomas, that form mainly in the pancreas and duodenum cause Zollinger-Ellison syndrome. Gastrinomas release large amounts of a hormone called gastrin. Normally, your body releases a small amount of gastrin after you eat, and gastrin triggers your stomach to make acid. When gastrinomas release large amounts of gastrin, your stomach makes too much acid, which leads to Zollinger-Ellison syndrome. […] In most cases of Zollinger-Ellison syndrome, experts dont know what causes the gastrinomas to form. In about 20% to 25% of cases, a rare genetic disorder called MEN1 causes gastrinomas to form.
  • #1 Zollinger-Ellison syndrome – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/zollinger-ellison-syndrome/symptoms-causes/syc-20379042
    Zollinger-Ellison syndrome may be caused by an inherited condition called multiple endocrine neoplasia, type 1 (MEN 1). People with MEN 1 also have tumors in the parathyroid glands. They may have tumors in their pituitary glands as well. […] About 25% of people who have gastrinomas have them as part of MEN 1. They also may have tumors in the pancreas and other organs.
  • #1 Zollinger-Ellison Syndrome: Symptoms and Treatment
    https://patient.info/doctor/zollinger-ellison-syndrome
    Zollinger-Ellison syndrome (ZES) is an endocrinopathy characterised by gastrin-secreting tumours, which cause multiple, refractory and recurrent peptic ulcers in the distal duodenum and proximal jejunum. There are two main variants: Sporadic (isolated) and Associated with parathyroid and pituitary tumours as part of the genetic disorder multiple endocrine neoplasia type 1 (MEN1). […] The tumour (gastrinoma) is usually in the duodenum (60-65%) or the pancreas (30%). In rare cases, gastrinomas occur in other abdominal locations (eg, the stomach, liver, bile duct, ovary) and also extra-abdominal locations (eg, the heart, lung – small cell lung cancer). […] 20-30% of patients have ZES as part of MEN1, an autosomal dominant disorder. […] Gastrinomas are the most common functioning tumour of the pancreas. In addition to secreting high levels of gastrin, these tumours may produce other hormones such as adrenocorticotrophic hormone (ACTH), vasoactive intestinal polypeptide (VIP), and glucagon.
  • #1 Zollinger-Ellison syndrome: Symptoms, diagnosis, and treatment
    https://www.medicalnewstoday.com/articles/186793
    Zollinger-Ellison syndrome is a rare disorder that usually occurs when a tumor called a gastrinoma develops in the pancreas or duodenum. […] Gastrinomas that result in Zollinger-Ellison syndrome may originate from the pancreas or, less likely, from the small intestine. They occasionally stem from other organs in the body, for example, the lymph nodes, stomach, liver, and ovaries. […] It is not clear exactly what causes Zollinger-Ellison syndrome or the gastrinomas that develop. […] Most people develop Zollinger-Ellison syndrome for no apparent reason. However, a genetic condition called multiple endocrine neoplasia type 1 (MEN1) is responsible for 25 to 30 percent of cases. […] A person has a 50 percent chance of developing Zollinger-Ellison syndrome from a parent who has the syndrome. […] An individual may be at risk for developing a gastrinoma if they have had several family members with endocrine cancers, or if they have a family member with MEN1.
  • #1 Zollinger-Ellison Syndrome: Causes, Symptoms, and Treatment
    https://www.medicoverhospitals.in/diseases/zollingerellison-syndrome/
    Zollinger-Ellison Syndrome (ZES) is a rare but serious condition characterized by the formation of one or more tumors known as gastrinomas in the pancreas or duodenum. […] In some cases, Zollinger-Ellison Syndrome is associated with a genetic disorder called Multiple Endocrine Neoplasia type 1 (MEN1). […] The majority of Zollinger-Ellison Syndrome cases are sporadic, meaning they occur in individuals without a family history of the condition. […] The pathophysiology of Zollinger-Ellison Syndrome involves the overproduction of gastrin by gastrinomas. […] Elevated gastrin levels suggest the presence of gastrinomas. […] Given the association between Zollinger-Ellison Syndrome and MEN1, genetic counseling is recommended for families with a history of endocrine disorders.
  • #1 Zollinger–Ellison syndrome – Wikipedia
    https://en.wikipedia.org/wiki/Zollinger%E2%80%93Ellison_syndrome
    ZollingerEllison syndrome (Z-E syndrome) is a rare disease in which tumors cause the stomach to produce too much acid, resulting in peptic ulcers. […] The syndrome is caused by the formation of a gastrinoma, a neuroendocrine tumor that secretes a hormone called gastrin. […] In 75% of cases, ZollingerEllison syndrome occurs sporadically, while the remaining 25% of cases are due to an autosomal dominant syndrome called multiple endocrine neoplasia type 1 (MEN 1). […] Gastrinomas most commonly arise in the duodenum, pancreas or stomach.
  • #1 Gastrinomas and Zollinger-Ellison Syndrome
    https://www.cancercenter.com/cancer-types/pancreatic-cancer/types/gastrinomas
    Gastrinomas are a rare form of pancreatic neuroendocrine tumor (NET) that begins in the neuroendocrine cells of the pancreas. […] Different types of neuroendocrine cells release different types of hormones. For example, gastrinomas release gastrin. […] When working correctly, gastrin is released in small amounts after you eat, but gastrinomas may trigger a condition called Zollinger-Ellison syndrome, in which the stomach produces excess acid, ulcers, bleeding and severe reflux. […] Zollinger-Ellison syndrome (ZES) occurs when gastrinomas cause the stomach to produce too much acid. […] Approximately 20 percent of patients diagnosed with Zollinger-Ellison syndrome also have multiple endocrine neoplasia type 1 (MEN1), a rare genetic condition, according to NIDDK. […] While the uncontrolled division and reproduction of gastrin-producing cells contributes to the occurrence of gastrinomas, research to identify their exact cause is ongoing. […] However, its known that an inherited genetic condition called multiple endocrine neoplasia type 1 (MEN1) syndrome causes 25 percent to 30 percent of all gastrinomas, according to The National Pancreas Foundation.
  • #1 Zollinger-Ellison Syndrome Causes, Treatments, Symptoms, Prognosis, and More
    https://www.webmd.com/digestive-disorders/zollinger-ellison-syndrome
    Zollinger-Ellison syndrome (ZES) is a disease of the gastrointestinal system. People who have ZES develop tumors known as gastrinomas in the pancreas and duodenum (the first section of the small intestine). The gastrinomas caused by ZES secrete the hormone gastrin. […] Approximately 25% to 30% of ZES patients also have a genetic (inherited) disorder known as „multiple endocrine neoplasia type 1,” which also causes tumors in the pituitary and parathyroid glands. […] Another complication of ZES is that more than half of single gastrinomas are malignant (cancerous). These malignant gastrinomas can spread to other parts of the body, including the liver, lymph nodes, spleen, bones, or skin. […] Treatment of ZES depends on whether the gastrinoma is sporadic or part of the inherited MEN I syndrome. While the latter is usually treated with acid suppression alone, sporadic gastrinomas are treated with acid suppression and surgical removal of the tumor.
  • #1 Zollinger-Ellison Syndrome | Symptoms & Causes | Freedmans Health
    https://freedmanshealth.org/disease-conditions/symptoms-causes/zollinger-ellison-syndrome/
    Zollinger-Ellison syndrome (ZES) is a very rare condition that affects your digestive system. It results from a type of tumor called a gastrinoma which may develop in your pancreas and/or duodenum (the top of your small intestine). […] Gastrinomas are estimated to affect between 1 to 2 people per million every year. Most physicians have never seen a case. You’re more likely to get them if you have a family history of multiple endocrine neoplasia (MEN) type 1. About 25% of people with Zollinger-Ellison syndrome have this rare genetic disorder, which causes tumors in various glands throughout your endocrine system. However, most people who get gastrinomas get them for unknown reasons. They’re usually diagnosed in middle age.
  • #1 Zollinger-Ellison Syndrome
    https://healthlibrary.somc.org/Library/DiseasesConditions/Pediatric/Blood/134,209
    Zollinger-Ellison syndrome (ZES) is a rare digestive disorder that occurs when one or more tumors called gastrinomas located in your pancreas or duodenum cause your stomach to make too much acid. […] The exact cause of this disorder is unknown. Tumors called gastrinomas release large amounts of a hormone called gastrin, causing your stomach to make too much acid. […] Just about anyone can get ZES. But some people with the condition may have a rare genetic problem known as multiple endocrine neoplasia type 1 (MEN1). Children of adults with MEN1 are at a greater risk of getting the disease. ZES is more common in men, often those 20 to 50 years old. […] The tumors that cause Zollinger-Ellison syndrome are sometimes cancerous and may spread to other parts of your body. If the cancer spreads, it most often spreads to lymph nodes near the tumor and later to the liver and bones.
  • #1 Zollinger-Ellison Syndrome: Symptoms, Causes, Diagnosis & Treatment | Medanta
    https://www.medanta.org/hospitals-near-me/gurugram-hospital/speciality/gi-surgery/disease/zollinger-ellison-syndrome-symptoms-causes-treatment-diagnosis
    ZES is primarily caused by the growth of gastrinomas, usually benign, non-cancerous tumours that develop in the pancreas. […] The excessive gastrin production caused by these tumours is responsible for developing peptic ulcers and other gastrointestinal complications. […] In some cases, the cause of ZES is unknown. However, research suggests that genetic mutations may play a crucial role in the spreading the condition. In particular, mutations in a gene called MEN1 have been associated with the development of ZES. MEN1 is a tumour suppressor gene that helps prevent the growth of tumours in the pancreas and other organs. When this gene is mutated, it can lead to the development of pancreatic gastrinoma and other types of tumours. […] Zollinger-Ellison Syndrome (ZES) is a rare condition caused by the formation of tumours that produce excessive amounts of gastrin, a hormone that stimulates acid production in the stomach. While the exact cause of ZES is unknown, certain factors can raise a person’s risk of developing the condition.
  • #1 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 critical step for the diagnosis of ZES is represented by the initial clinical suspicion. Hypergastrinemia is the hallmark of ZES; however, hypergastrinemia might recognize several causes, which should be ruled out in order to make a final diagnosis. Gastrin levels 1000 pg/mL and a gastric pH below 2 are considered to be diagnostic for gastrinoma; some specific tests, including esophageal pH-recording and secretin test, might be useful in selected cases, although they are not widely available. […] Zollinger-Ellison syndrome (ZES) was firstly described in 1955 as associated with a neuroendocrine neoplasm (NEN) capable of ectopic gastrin secretion (namely gastrinoma), resulting in gastric acid hypersecretion, which typically leads to gastroesophageal reflux disease (GERD), recurrent peptic ulcers, and chronic diarrhea.
  • #1 Gastrinoma | Endocrine Conditions
    https://www.yourhormones.info/endocrine-conditions/gastrinoma/
    Gastrinomas are tumours which produce an excessive amount of the hormone called gastrin. These tumours originate from pancreas, stomach and the small intestine. […] Gastrinomas are neuroendocrine tumours that produce large quantities of the hormone gastrin, which stimulates the production of excess gastric acid from the stomach. […] Gastrinomas are overgrowth of G cells, which lead to production of excessive amounts of gastrin The increased acid production causes erosions or ulcers in the stomach and small intestine. […] Gastrinomas often develop spontaneously (sporadic tumours) However, in approximately 20-30% of patients these tumours may develop as part of a syndrome called multiple endocrine neoplasia type 1 (MEN1). […] Zollinger-Ellison syndrome is a rare syndrome caused by a gastrinoma, which is characterised by severe, recurrent, and multiple peptic ulcers in the first part of the small intestine (such as the duodenum) or pancreas.
  • #1 Zollinger-Ellison syndrome pathophysiology – Creative Med Doses
    https://creativemeddoses.com/topics-list/zollinger-ellison-syndrome-pathophysiology/
    Zollinger-Ellison syndrome (ZES) is caused by a nonbeta islet cell, gastrin-secreting tumor of the pancreas that stimulates the acid-secreting cells of the stomach to maximal activity. […] The symptoms of Zollinger-Ellison syndrome (ZES) are due to hypergastrinemia, it causes hypertrophy of the gastric mucosa, leading to increased numbers of parietal cells and increased acid output. […] Gastrin also stimulates acid secretion, resulting in increased basal acid secretion. This excessive acid secretion leads to gastrointestinal mucosal ulceration. It also causes diarrhea and malabsorption. […] Malabsorption in ZES usually due to Direct mucosal damage due to acid, Inactivation of pancreatic enzymes due to acidic pH (pancreatic enzyme works in Alkaline pH), Precipitation of bile salts due to acidic pH.
  • #1 Zollinger-Ellison Syndrome | 5-Minute Clinical Consult
    https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688838/all/Zollinger_Ellison_Syndrome
    Zollinger-Ellison syndrome (ZES) triad: Markedly elevated gastric acid secretion, Peptic ulcer disease, A gastrinoma or non-islet cell tumor of the pancreas or duodenal wall that produces gastrin (hypergastrinemia). Gastrinomas (at the time of diagnosis) may be single or multiple (1/2 to 2/3), large or small, benign or malignant (2/3), sporadic (7075%) or associated with multiple endocrine neoplasia type 1 (MEN1) (2530%). […] Gastrinoma is found the head of the pancreas (2030%) and the first or second portion of the duodenum (7080%); if in the pancreas, the lesion is more likely to metastasize to the liver. Hypergastrinemia results in gastric mucosal hypertrophy and increased acid production. Increased acid production causes mucosal ulceration. Diarrhea (60%) and malabsorption are also common in ZES. […] ~2530% of cases occur in association with the autosomal dominant MEN1 syndrome tumors of pancreas, pituitary, and parathyroid. Can occur sporadically as well. […] Risk Factors: MEN1, Family history of ulcer disease.
  • #1 Eponyms in Oncology: Zollinger-Ellison Syndrome- Cancer Therapy Advisor
    https://www.cancertherapyadvisor.com/features/eponyms-oncology-zollinger-ellison-syndrome/
    Zollinger-Ellison syndrome is a condition in which neuroendocrine gastrinomas form in the pancreas or duodenum. […] About 60% of gastrinomas are malignant. The gastrinomas secrete gastrin, which causes the stomach to produce too much acid, and this leads to peptic ulcers in the stomach and duodenum. […] Drs Zollinger and Ellison theorized that a hormone secreted from these tumors was causing the gastric acid output that resulted in the peptic ulcer syndromes. […] In 1960, Dr Roderic Gregory and Dr Hilda Tracy extracted a gastrin-like substance from the pancreas of a patient with Zollinger-Ellison syndrome, establishing gastrin as the hormone that causes the syndrome.
  • #1 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
    The management of gastrinoma and ZES includes both a proper medical treatment for symptoms relief and surgery with curative intent whenever feasible. […] 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. […] The therapy for syndrome control is based on PPI, which are highly effective drugs and considered the drugs of choice for suppressing acid secretion.
  • #1 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
    Gastrinomas are NENs located in the duodenum (70%), pancreas (25%), and rarely (5%), in other sites, including stomach, liver, ovary, and lung. Gastrinoma is the most frequent functioning duodenal NEN and the second most frequently occurring functional pancreatic NEN (pNEN), following insulinoma; in turn, 15% of functioning pNENs is represented by gastrinoma. […] The diagnosis of ZES is not always straightforward due to both non-specific symptoms and confounding factors including proton pump inhibitor (PPI) therapy, which might temporarily relieve symptoms. […] Given that gastrinoma and ZES need both a proper medical treatment for symptom relief and a surgical procedure whenever feasible, the multidisciplinary approach, with close cooperation between clinicians and surgeons, remains the cornerstone for proper management of this composite disease, which should be always referred to tertiary centers.
  • #2 Zollinger-Ellison Syndrome – NIDDK
    https://www.niddk.nih.gov/health-information/digestive-diseases/zollinger-ellison-syndrome
    Tumors, called gastrinomas, that form mainly in the pancreas and duodenum cause Zollinger-Ellison syndrome. Gastrinomas release large amounts of a hormone called gastrin. Normally, your body releases a small amount of gastrin after you eat, and gastrin triggers your stomach to make acid. When gastrinomas release large amounts of gastrin, your stomach makes too much acid, which leads to Zollinger-Ellison syndrome. […] In most cases of Zollinger-Ellison syndrome, experts dont know what causes the gastrinomas to form. In about 20% to 25% of cases, a rare genetic disorder called MEN1 causes gastrinomas to form.
  • #2 Mayo Clinic Health Library – Zollinger-Ellison syndrome | Swiss Medical Network
    https://www.swissmedical.net/en/healtcare-library/con-20379029
    Zollinger-Ellison syndrome is a condition in which one or more tumors grow in the pancreas or small intestine. The tumors, called gastrinomas, produce large amounts of the hormone gastrin. Gastrin causes the stomach to produce too much acid, which leads to peptic ulcers. […] The exact cause of Zollinger-Ellison syndrome isn’t known. But the pattern of events that occurs in Zollinger-Ellison syndrome typically follows the same sequence. The syndrome begins when one or more tumors form in the pancreas or a part of the small intestine called the duodenum. […] The tumors that form with Zollinger-Ellison syndrome are made up of cells that secrete large amounts of the hormone gastrin. For this reason, they are sometimes called gastrinomas. Increased gastrin causes the stomach to make far too much acid. […] Zollinger-Ellison syndrome may be caused by an inherited condition called multiple endocrine neoplasia, type 1 (MEN 1).
  • #2 Zollinger-Ellison syndrome: Symptoms, diagnosis, and treatment
    https://www.medicalnewstoday.com/articles/186793
    Zollinger-Ellison syndrome is a rare disorder that usually occurs when a tumor called a gastrinoma develops in the pancreas or duodenum. […] Gastrinomas that result in Zollinger-Ellison syndrome may originate from the pancreas or, less likely, from the small intestine. They occasionally stem from other organs in the body, for example, the lymph nodes, stomach, liver, and ovaries. […] It is not clear exactly what causes Zollinger-Ellison syndrome or the gastrinomas that develop. […] Most people develop Zollinger-Ellison syndrome for no apparent reason. However, a genetic condition called multiple endocrine neoplasia type 1 (MEN1) is responsible for 25 to 30 percent of cases. […] A person has a 50 percent chance of developing Zollinger-Ellison syndrome from a parent who has the syndrome. […] An individual may be at risk for developing a gastrinoma if they have had several family members with endocrine cancers, or if they have a family member with MEN1.
  • #2 Zollinger-Ellison Syndrome: Symptoms and Treatment
    https://patient.info/doctor/zollinger-ellison-syndrome
    Zollinger-Ellison syndrome (ZES) is an endocrinopathy characterised by gastrin-secreting tumours, which cause multiple, refractory and recurrent peptic ulcers in the distal duodenum and proximal jejunum. There are two main variants: Sporadic (isolated) and Associated with parathyroid and pituitary tumours as part of the genetic disorder multiple endocrine neoplasia type 1 (MEN1). […] The tumour (gastrinoma) is usually in the duodenum (60-65%) or the pancreas (30%). In rare cases, gastrinomas occur in other abdominal locations (eg, the stomach, liver, bile duct, ovary) and also extra-abdominal locations (eg, the heart, lung – small cell lung cancer). […] 20-30% of patients have ZES as part of MEN1, an autosomal dominant disorder. […] Gastrinomas are the most common functioning tumour of the pancreas. In addition to secreting high levels of gastrin, these tumours may produce other hormones such as adrenocorticotrophic hormone (ACTH), vasoactive intestinal polypeptide (VIP), and glucagon.
  • #2 Zollinger-Ellison Syndrome
    https://healthlibrary.ecuhealth.org/Library/Wellness/Parenting/134,209
    Zollinger-Ellison syndrome (ZES) is a rare digestive disorder that occurs when one or more tumors called gastrinomas located in your pancreas or duodenum cause your stomach to make too much acid. […] The exact cause of this disorder is unknown. Tumors called gastrinomas release large amounts of a hormone called gastrin, causing your stomach to make too much acid. […] Just about anyone can get ZES. But some people with the condition may have a rare genetic problem known as multiple endocrine neoplasia type 1 (MEN1). Children of adults with MEN1 are at a greater risk of getting the disease. ZES is more common in men, often those 20 to 50 years old. […] The extra stomach acid caused by Zollinger-Ellison syndrome can lead to peptic ulcer disease or GERD. Without treatment, these diseases can lead to complications such as:
  • #2 Zollinger-Ellison Syndrome: Symptoms and Treatment
    https://patient.info/doctor/zollinger-ellison-syndrome
    If ZES is confirmed, other features of MEN1 should be sought, including a family history. […] Once the diagnosis has been made, management is within tertiary centres by multidisciplinary teams. Management includes medical suppression of gastric acid production and surgical resection of primary tumour for the prevention of malignant transformation and metastatic complications. […] In patients with liver metastases, surgery should be considered if all identifiable tumour can be safely removed. […] It has been argued that the widespread use of PPIs may delay the diagnosis of ZES so that presentation is later and more advanced.
  • #2 Zollinger-Ellison Syndrome Causes, Treatments, Symptoms, Prognosis, and More
    https://www.webmd.com/digestive-disorders/zollinger-ellison-syndrome
    Zollinger-Ellison syndrome (ZES) is a disease of the gastrointestinal system. People who have ZES develop tumors known as gastrinomas in the pancreas and duodenum (the first section of the small intestine). The gastrinomas caused by ZES secrete the hormone gastrin. […] Approximately 25% to 30% of ZES patients also have a genetic (inherited) disorder known as „multiple endocrine neoplasia type 1,” which also causes tumors in the pituitary and parathyroid glands. […] Another complication of ZES is that more than half of single gastrinomas are malignant (cancerous). These malignant gastrinomas can spread to other parts of the body, including the liver, lymph nodes, spleen, bones, or skin. […] Treatment of ZES depends on whether the gastrinoma is sporadic or part of the inherited MEN I syndrome. While the latter is usually treated with acid suppression alone, sporadic gastrinomas are treated with acid suppression and surgical removal of the tumor.
  • #2 What Causes Zollinger-Ellison Syndrome?
    https://www.icliniq.com/articles/gastro-health/zollinger-ellison-syndrome
    Zollinger-Ellison syndrome is caused by multiple tumors called Gastrinoma. Gastrinomas develop in the pancreas or duodenum. […] This syndrome is also known to be associated with a hereditary genetic disorder known as multiple endocrine neoplasia 1 (MEN 1). MEN 1 also produces a few hormones that are responsible for the formation of gastrinomas in the duodenum.
  • #2 Zollinger-Ellison Syndrome/Gastrinoma – National Pancreas Foundation
    https://pancreasfoundation.org/pancreas-disease/other-pancreatic-conditions/zollinger-ellison-syndrome-gastrinoma/
    Zollinger-Ellison syndrome is a rare disorder characterized by the development of a tumor called a Gastrinoma found in the pancreas and/or duodenum. […] Medical researchers are still studying the exact cause of Zollinger-Ellison syndrome. About 25 to 30 percent of Gastrinoma’s are caused by an inherited genetic disorder called multiple endocrine neoplasia type 1 (MEN1). […] Symptoms of MEN1 include increased hormone levels in the blood, kidney stones, diabetes, muscle weakness, weakened bones, and fractures.
  • #2 Zollinger-Ellison Syndrome: Symptoms, Causes, Diagnosis & Treatment | Medanta
    https://www.medanta.org/pillar/zollinger-ellison-syndrome-symptoms-causes-treatment-diagnosis
    ZES is primarily caused by the growth of gastrinomas, usually benign, non-cancerous tumours that develop in the pancreas. However, in rare cases, these tumours can become malignant and spread to other body parts. […] The excessive gastrin production caused by these tumours is responsible for developing peptic ulcers and other gastrointestinal complications. […] In some cases, the cause of ZES is unknown. However, research suggests that genetic mutations may play a crucial role in the spreading the condition. In particular, mutations in a gene called MEN1 have been associated with the development of ZES. MEN1 is a tumour suppressor gene that helps prevent the growth of tumours in the pancreas and other organs. When this gene is mutated, it can lead to the development of pancreatic gastrinoma and other types of tumours.
  • #2 Zollinger-Ellison syndrome | Altru Health System
    https://www.altru.org/health-library/conditions/zollinger-ellison-syndrome
    Zollinger-Ellison syndrome is a condition in which one or more tumors grow in the pancreas or small intestine. The tumors, called gastrinomas, produce large amounts of the hormone gastrin. […] The exact cause of Zollinger-Ellison syndrome isn’t known. But the pattern of events that occurs in Zollinger-Ellison syndrome typically follows the same sequence. The syndrome begins when one or more tumors form in the pancreas or a part of the small intestine called the duodenum. […] The tumors that form with Zollinger-Ellison syndrome are made up of cells that secrete large amounts of the hormone gastrin. For this reason, they are sometimes called gastrinomas. Increased gastrin causes the stomach to make far too much acid. […] Zollinger-Ellison syndrome may be caused by an inherited condition called multiple endocrine neoplasia, type 1 (MEN 1). […] About 25% of people who have gastrinomas have them as part of MEN 1. […] If you have a first-degree relative, such as a sibling or parent, with MEN 1, it’s more likely that you’ll have Zollinger-Ellison syndrome.
  • #2 Zollinger-Ellison Syndrome: Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/17960-zollinger-ellison-syndrome
    Zollinger-Ellison syndrome is a rare condition that causes tumors called gastrinomas to grow in your pancreas or intestine. Gastrinomas release the hormone gastrin, which triggers your stomach to produce gastric acid. Too much stomach acid leads to ulcers and other complications. Some gastrinomas are also cancerous. […] Most cases of Zollinger-Ellison syndrome happen sporadically (for no known reason). About 25% of people with this condition have MEN Type 1. MEN stands for multiple endocrine neoplasia. MEN is a rare inherited disorder that affects glands in your endocrine system. […] Zollinger-Ellison syndrome can be life-threatening. For example, a gastrinoma tumor that blocks your duodenum can make it rupture. The tumor can spread to your liver. Large amounts of gastric acid may lead to severe gastrointestinal bleeding. […] Surgery to remove cancerous gastrinomas may cure the condition. But a cure depends on removing all cancerous cells in your body. If surgery is successful, you’ll need to take medication to manage gastric acid for the rest of your life.
  • #2 Zollinger-Ellison Syndrome: Symptoms, Treatment, and More
    https://www.healthline.com/health/zollinger-ellison-syndrome
    ZES is a rare condition that affects the digestive tract. Its possible to only have one tumor, but more common for there to be multiple ones. About half of these tumors are noncancerous, or benign. Experts arent exactly sure what causes ZES. In most cases, the disorder appears for no obvious reason. In 25 percent of cases, its association with an inherited, tumor-causing condition called multiple endocrine neoplasia type 1 (MEN1). […] More research is needed to better understand this condition.
  • #2 Zollinger-Ellison Syndrome: Symptoms, Causes, Diagnosis & Treatment | Medanta
    https://www.medanta.org/hospitals-near-me/gurugram-hospital/speciality/gi-surgery/disease/zollinger-ellison-syndrome-symptoms-causes-treatment-diagnosis
    One of the common risk factors for ZES is having a family history of the condition or multiple endocrine neoplasia type 1 (MEN1), a genetic disorder that can cause tumours in various endocrine glands, including the pancreas. Individuals with a family history of these conditions should know the increased risk and discuss appropriate screening with their healthcare provider. […] In some cases, the cause of ZES is unknown. However, research suggests that genetic mutations may play a crucial role in the spreading the condition.
  • #2 Zollinger-Ellison Syndrome (ZES) vs. Gastrinoma
    https://www.healthline.com/health/zollinger-ellison-syndrome-vs-gastrinoma
    Zollinger-Ellison syndrome (ZES) is a health condition that results from having a gastrinoma, or gastric tumor, that increases the production of stomach acid. […] Gastrinoma can develop from a mutation in neuroendocrine cells. About 75% to 80% of people develop it sporadically, while 20% to 30% of people develop it due to a MEN-1 mutation. MEN-1 also makes it more likely a person will develop multiple tumors. […] A gastrinoma is a type of tumor that secretes gastric acid, which can cause chronic diarrhea and acid reflux symptoms.
  • #2 Zollinger-Ellison syndrome pathophysiology – Creative Med Doses
    https://creativemeddoses.com/topics-list/zollinger-ellison-syndrome-pathophysiology/
    Zollinger-Ellison syndrome (ZES) is caused by a nonbeta islet cell, gastrin-secreting tumor of the pancreas that stimulates the acid-secreting cells of the stomach to maximal activity. […] The symptoms of Zollinger-Ellison syndrome (ZES) are due to hypergastrinemia, it causes hypertrophy of the gastric mucosa, leading to increased numbers of parietal cells and increased acid output. […] Gastrin also stimulates acid secretion, resulting in increased basal acid secretion. This excessive acid secretion leads to gastrointestinal mucosal ulceration. It also causes diarrhea and malabsorption. […] Malabsorption in ZES usually due to Direct mucosal damage due to acid, Inactivation of pancreatic enzymes due to acidic pH (pancreatic enzyme works in Alkaline pH), Precipitation of bile salts due to acidic pH.
  • #2 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
    The management of gastrinoma and ZES includes both a proper medical treatment for symptoms relief and surgery with curative intent whenever feasible. […] 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. […] The therapy for syndrome control is based on PPI, which are highly effective drugs and considered the drugs of choice for suppressing acid secretion.