Materiały źródłowe

• #1 Zollinger-Ellison syndrome | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/zollinger-ellison-syndrome?content_id=CON-20379029

  Treatment of Zollinger-Ellison syndrome focuses on treating the hormone-secreting tumors as well as the ulcers they cause. […] An operation to remove the gastrinomas requires a skilled surgeon because the tumors are often small and difficult to find. If you have just one tumor, a medical professional may be able to remove it surgically. But surgery may not be an option if you have many tumors or tumors that have spread to your liver. On the other hand, even if you have multiple tumors, your surgeon still may recommend removing a single large tumor. […] In some cases, providers recommend other treatments to control tumor growth, including: Removing as much of a liver tumor as possible, a procedure known as debulking. Attempting to destroy the tumor by cutting off the blood supply, called embolization. Using heat to destroy cancer cells with a procedure called radiofrequency ablation. Injecting drugs into the tumor to relieve cancer symptoms. Using chemotherapy to try to slow tumor growth. Having a liver transplant.

• #2 Treatment of Zollinger-Ellison Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4320348/

  In this article, we have reviewed the main therapeutic measures for the treatment of Zollinger-Ellison syndrome (ZES). […] In ZES patients, the best therapeutic procedure is surgery which, if radical, can be curative. Medical treatment can be the best palliative therapy and should be used, when possible, in association with surgery, in a multimodal therapeutic approach. […] The goal of ZES therapy is to obtain improvement of the symptoms and healing of the ulcers by controlling the gastric hypersecretion. […] Omeprazole, the first proton pump inhibitor available, belongs to a new class of drugs which has proven to be safe and effective in controlling gastric hypersecretion in patients with ZES and has completely substituted the use of H2 antagonists. […] Somatostatin analogs can be useful in reducing gastric acid hypersecretion, serum gastrin and gastric ECL-cells, thus contributing to curing the disease more effectively.

• #3 Zollinger-Ellison syndrome | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/zollinger-ellison-syndrome?content_id=CON-20379029

  Excess acid production can almost always be controlled. Medicines known as proton pump inhibitors are the first line of treatment. These are effective medicines for controlling acid production in Zollinger-Ellison syndrome. […] Proton pump inhibitors are powerful acid-reducing medicines. They work by blocking the action of the tiny „pumps” within acid-secreting cells. Commonly prescribed medicines include lansoprazole (Prevacid), omeprazole (Prilosec, Zegerid), pantoprazole (Protonix), rabeprazole (Aciphex) and esomeprazole (Nexium). Talk with your care professional about the risks of taking these medicines long term. […] Octreotide (Sandostatin), a medicine similar to the hormone somatostatin, may counteract the effects of gastrin and be helpful for some people.

• #4 Treatment of Zollinger-Ellison Syndrome

  https://www.wjgnet.com/1007-9327/full/v11/i35/5423.htm

  Thus, at present, a large number of treatment options are available for the therapy of patients with ZES: control of the gastric acid hypersecretion; control of the gastrin hypersecretion; surgical resection or cytoreduction of the tumor; control of the tumor size by using chemotherapy or interferon alfa; control of the hepatic metastases with chemoembolization and/or embolization. […] If ZES is suspected, it is important, while waiting for the conclusive results of the diagnostic tests, to prevent the complications which could arise. For this reason, it is advised to rapidly start an antisecretory therapy which is usually well-tolerated and without particular contraindications. […] The goal of ZES therapy is to obtain improvement of the symptoms and healing of the ulcers by controlling the gastric hypersecretion.

• #5 Zollinger-Ellison syndrome | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/zollinger-ellison-syndrome?content_id=CON-20379029

  Excess acid production can almost always be controlled. Medicines known as proton pump inhibitors are the first line of treatment. These are effective medicines for controlling acid production in Zollinger-Ellison syndrome. […] Proton pump inhibitors are powerful acid-reducing medicines. They work by blocking the action of the tiny „pumps” within acid-secreting cells. Commonly prescribed medicines include lansoprazole (Prevacid), omeprazole (Prilosec, Zegerid), pantoprazole (Protonix), rabeprazole (Aciphex) and esomeprazole (Nexium). Talk with your care professional about the risks of taking these medicines long term. […] Octreotide (Sandostatin), a medicine similar to the hormone somatostatin, may counteract the effects of gastrin and be helpful for some people.

• #6 Zollinger-Ellison Syndrome Causes, Treatments, Symptoms, Prognosis, and More

  https://www.webmd.com/digestive-disorders/zollinger-ellison-syndrome

  ZES is treated by reducing the amount of acid your stomach produces. Medications called proton pump inhibitors are usually prescribed. These drugs, which include dexlansoprazole (Dexilant), esomeprazole (Nexium), lansoprazole (Prevacid), omeprazole (Prilosec, Zegerid), pantoprazole (Protonix), and rabeprazole (Aciphex), curb the production of stomach acid and allow the ulcers to heal. […] 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. Somatostatin analogs such as octreotide, which suppresses hormone production, are also very good at controlling symptoms. […] If there is metastatic disease, you may be offered a combination of therapies including surgery, chemotherapy, targeted drug therapy, or radiation.

• #7 Zollinger-Ellison syndrome | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/zollinger-ellison-syndrome?content_id=CON-20379029

  Excess acid production can almost always be controlled. Medicines known as proton pump inhibitors are the first line of treatment. These are effective medicines for controlling acid production in Zollinger-Ellison syndrome. […] Proton pump inhibitors are powerful acid-reducing medicines. They work by blocking the action of the tiny „pumps” within acid-secreting cells. Commonly prescribed medicines include lansoprazole (Prevacid), omeprazole (Prilosec, Zegerid), pantoprazole (Protonix), rabeprazole (Aciphex) and esomeprazole (Nexium). Talk with your care professional about the risks of taking these medicines long term. […] Octreotide (Sandostatin), a medicine similar to the hormone somatostatin, may counteract the effects of gastrin and be helpful for some people.

• #8 Zollinger-Ellison Syndrome Causes, Treatments, Symptoms, Prognosis, and More

  https://www.webmd.com/digestive-disorders/zollinger-ellison-syndrome

  ZES is treated by reducing the amount of acid your stomach produces. Medications called proton pump inhibitors are usually prescribed. These drugs, which include dexlansoprazole (Dexilant), esomeprazole (Nexium), lansoprazole (Prevacid), omeprazole (Prilosec, Zegerid), pantoprazole (Protonix), and rabeprazole (Aciphex), curb the production of stomach acid and allow the ulcers to heal. […] 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. Somatostatin analogs such as octreotide, which suppresses hormone production, are also very good at controlling symptoms. […] If there is metastatic disease, you may be offered a combination of therapies including surgery, chemotherapy, targeted drug therapy, or radiation.

• #9 Successful Lifetime/Long-Term Medical Treatment of Acid Hypersecretion in Zollinger-Ellison Syndrome (ZES): Myth or Fact? Insights from an Analysis of Results of NIH Long-Term Prospective Studies of ZES

  https://www.mdpi.com/2072-6694/15/5/1377

  Successful Lifetime/Long-Term Medical Treatment of Acid Hypersecretion in Zollinger-Ellison Syndrome (ZES): Myth or Fact? Insights from an Analysis of Results of NIH Long-Term Prospective Studies of ZES […] Zollinger-Ellison syndrome (ZES) has, since its original description, been characterized by extreme acid hypersecretion due to a neuroendocrine tumor ectopically secreting gastrin, resulting in severe, recalcitrant peptic ulcer disease/gastroesophageal reflux disease (GERD) that is refractory to standard anti-acid treatments. From the very beginning of its description in 1955, it has been well recognized that control of the acid hypersecretion, both acutely and long-term, is essential to all aspects of management of these patients. Originally, no medical treatment was effective at controlling the acid hypersecretion long-term, resulting in total gastrectomy being the initial treatment of choice. However, starting in the late 1970s with the discovery of histamine H2-receptor antagonists (H2Rs) and then in the 1980s with the widespread use of gastric H+K+ ATPase inhibitors (also called proton pump inhibitors [PPIs]), medical control of gastric acid hypersecretion became possible in almost all patients.

• #10 Successful Lifetime/Long-Term Medical Treatment of Acid Hypersecretion in Zollinger-Ellison Syndrome (ZES): Myth or Fact? Insights from an Analysis of Results of NIH Long-Term Prospective Studies of ZES

  https://www.mdpi.com/2072-6694/15/5/1377

  Long-term/lifelong acid antisecretory treatment with H2Rs/PPIs can be successfully carried out in all patients with both uncomplicated and complicated ZES (i.e., with MEN1/ZES, previous Billroth 2, severe GERD). This is only possible if drug doses are individually set by assessing acid secretory control to establish proven criteria, with regular reassessments and readjustments. […] The current study aimed to address these issues. The NIH prospective study on all aspects of ZES has been in effect since 1974, and part of the study involves the long-term medical management of the acid hypersecretion of all patients. In the current study, we analyzed data from this prospective database related to all issues of the long-term efficacy of both H2Rs and PPIs in these patients. […] The most important conclusion of the present study is that long-term/lifelong medical treatment of acid hypersecretion in ZES patients is possible and can be successful in controlling peptic acid symptoms and preventing the development of acid-related complications in all patients. While this statement includes long-term/lifelong treatment with either a PPI or H2R, for all practical purposes, the findings with PPIs will be of primary interest to most clinicians. Presently, because of the greater potency and longer duration of action of PPIs, which allows once or twice a day dosing, PPIs have become the drugs of choice for acid treatment in ZES.

• #11

  https://link.springer.com/article/10.1007/s11938-003-0017-6

  Zollinger-Ellison syndrome (ZES) is caused by a gastrin-producing tumor called a gastrinoma, which results in gastric acid hypersecretion. […] Initial treatment for ZES should be oral high-dose proton pump inhibitors. If parenteral therapy is needed, intermittent bolus injection of pantoprazole is recommended. […] Total gastrectomy and antisecretory surgery is rarely required. […] In patients without metastasis and without MEN-1, surgical cure is possible in 30%. It has been suggested that patients with gastrinomas larger than 2.5 cm, irrespective of whether they have MEN-1, should undergo surgical resection in an effort to decrease the risk for metastasis. […] Lew EA, Pisegna JR, Starr JA, et al.: Intravenous pantoprazole rapidly controls acid hypersecretion in patients with Zollinger-Ellison syndrome.

• #12 Zollinger-Ellison Syndrome Treatment & Management: Medical Care, Surgical Care

  https://emedicine.medscape.com/article/183555-treatment

  The goals of treatment in patients with Zollinger-Ellison syndrome (ZES) are medical control of gastric acid hypersecretion and surgical resection of the tumor. Inpatient care is aimed at first controlling the gastric acid hypersecretion. Once gastric acid hypersecretion is controlled, imaging studies should be obtained to localize the tumor and determine tumor extent. […] If the patient is acutely ill, immediate control of gastric acid hypersecretion can be achieved with intravenous proton pump inhibitors. […] Proton pump inhibitors are superior to H2 blockers for the control of gastric acid hypersecretion. […] Patients who are candidates for surgical resection should be referred for resection of the tumor. […] For patients with metastatic disease, chemotherapy, interferon, and octreotide may be helpful.

• #13 Zollinger-Ellison Syndrome Treatment & Management: Medical Care, Surgical Care

  https://emedicine.medscape.com/article/183555-treatment

  All patients with sporadic Zollinger-Ellison syndrome (ZES) without hepatic metastases or medical contraindications to surgery are advised to undergo surgical resection of the tumor because this decreases the risk of developing liver metastases, which can decrease the survival of these patients. […] An attempt at surgical resection has been recommended if the tumor is larger than 2.5 cm. […] After surgical resection of a gastrinoma, patients should be assessed for evidence of recurrence with serum fasting gastrin levels, a secretin test, and SRS. […] Proton pump inhibitors can be continued with the goal of maintaining the basal acid output (BAO) below 10 mEq/h before the next dose of the proton pump inhibitors.

• #14 Zollinger-Ellison syndrome | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/zollinger-ellison-syndrome?content_id=CON-20379029

  Treatment of Zollinger-Ellison syndrome focuses on treating the hormone-secreting tumors as well as the ulcers they cause. […] An operation to remove the gastrinomas requires a skilled surgeon because the tumors are often small and difficult to find. If you have just one tumor, a medical professional may be able to remove it surgically. But surgery may not be an option if you have many tumors or tumors that have spread to your liver. On the other hand, even if you have multiple tumors, your surgeon still may recommend removing a single large tumor. […] In some cases, providers recommend other treatments to control tumor growth, including: Removing as much of a liver tumor as possible, a procedure known as debulking. Attempting to destroy the tumor by cutting off the blood supply, called embolization. Using heat to destroy cancer cells with a procedure called radiofrequency ablation. Injecting drugs into the tumor to relieve cancer symptoms. Using chemotherapy to try to slow tumor growth. Having a liver transplant.

• #15 Zollinger-Ellison Syndrome: Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17960-zollinger-ellison-syndrome

  Treatment can ease Zollinger-Ellison symptoms. Surgery to remove gastrinoma tumors can cure it. But the tumors that cause it can come back. […] Your treatment will depend on your symptoms and whether the gastrinomas are cancerous or noncancerous. Treatment options include medication, surgery and cancer therapy. […] Proton pump inhibitors (PPIs) are the most common medication for Zollinger-Ellison syndrome. Proton pump inhibitors reduce the amount of gastric acid your stomach produces. […] Your provider may recommend surgery to remove the tumor. Surgeries to remove gastrinoma tumors include: Duodenectomy. Your surgeon makes a small incision in your duodenum to remove the tumor. Gastrectomy. This surgery removes all or part of your stomach. Liver surgery. Your surgeon removes tumors in your liver. Pancreatectomy. This surgery removes all or part of your pancreas. Tumor enucleation. You may have this surgery if there are tumors in the head of your pancreas.

• #16 Zollinger-Ellison Syndrome: Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17960-zollinger-ellison-syndrome

  Treatment can ease Zollinger-Ellison symptoms. Surgery to remove gastrinoma tumors can cure it. But the tumors that cause it can come back. […] Your treatment will depend on your symptoms and whether the gastrinomas are cancerous or noncancerous. Treatment options include medication, surgery and cancer therapy. […] Proton pump inhibitors (PPIs) are the most common medication for Zollinger-Ellison syndrome. Proton pump inhibitors reduce the amount of gastric acid your stomach produces. […] Your provider may recommend surgery to remove the tumor. Surgeries to remove gastrinoma tumors include: Duodenectomy. Your surgeon makes a small incision in your duodenum to remove the tumor. Gastrectomy. This surgery removes all or part of your stomach. Liver surgery. Your surgeon removes tumors in your liver. Pancreatectomy. This surgery removes all or part of your pancreas. Tumor enucleation. You may have this surgery if there are tumors in the head of your pancreas.

• #17 Zollinger-Ellison Syndrome: Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17960-zollinger-ellison-syndrome

  Treatment can ease Zollinger-Ellison symptoms. Surgery to remove gastrinoma tumors can cure it. But the tumors that cause it can come back. […] Your treatment will depend on your symptoms and whether the gastrinomas are cancerous or noncancerous. Treatment options include medication, surgery and cancer therapy. […] Proton pump inhibitors (PPIs) are the most common medication for Zollinger-Ellison syndrome. Proton pump inhibitors reduce the amount of gastric acid your stomach produces. […] Your provider may recommend surgery to remove the tumor. Surgeries to remove gastrinoma tumors include: Duodenectomy. Your surgeon makes a small incision in your duodenum to remove the tumor. Gastrectomy. This surgery removes all or part of your stomach. Liver surgery. Your surgeon removes tumors in your liver. Pancreatectomy. This surgery removes all or part of your pancreas. Tumor enucleation. You may have this surgery if there are tumors in the head of your pancreas.

• #18 Zollinger-Ellison Syndrome: Symptoms and Treatment

  https://patient.info/doctor/zollinger-ellison-syndrome

  Zollinger-Ellison syndrome treatment and management […] 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. Liver surgery is the best treatment for endocrine liver metastases, but it is often impossible due to diffuse disease. Systemic chemotherapy is poorly effective. Hepatic arterial embolisation and chemoembolisation may be considered. […] Oral PPIs will be effective in maintaining acid secretion at an acceptable level but a higher dose than usual, such as omeprazole 40 mg daily, is required. Oral doses of histamine H2 receptor antagonists can also be effective, but high, frequent dosing is required. Chemotherapy may be tried for metastatic disease.

• #19 Zollinger-Ellison Syndrome – Endocrine Surgery | UCLA Health

  https://www.uclahealth.org/medical-services/surgery/endocrine-surgery/patient-resources/patient-education/endocrine-surgery-encyclopedia/zollinger-ellison-syndrome

  A class of acid-suppressing medications called proton pump inhibitors (for example, omeprazole, lansoprazole) is now the first choice of treatment of Zollinger-Ellison syndrome. These drugs dramatically reduce acid production by the stomach, and promote healing of ulcers in the stomach and small bowel. They also provide relief of abdominal pain and diarrhea. […] Surgical removal of a single gastrinoma may be attempted if there is no evidence that it has spread to other organs (such as lymph nodes or the liver). Surgery on the stomach (gastrectomy) to control acid production is rarely necessary today. […] Early diagnosis and surgical removal of the tumor is associated with a cure rate of only 20% to 25%. However, gastrinomas grow slowly, and patients may live for many years after the tumor is discovered. Acid-suppressing medications are very effective at controlling the symptoms of acid overproduction.

• #20 Zollinger-Ellison syndrome | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/zollinger-ellison-syndrome?content_id=CON-20379029

  Treatment of Zollinger-Ellison syndrome focuses on treating the hormone-secreting tumors as well as the ulcers they cause. […] An operation to remove the gastrinomas requires a skilled surgeon because the tumors are often small and difficult to find. If you have just one tumor, a medical professional may be able to remove it surgically. But surgery may not be an option if you have many tumors or tumors that have spread to your liver. On the other hand, even if you have multiple tumors, your surgeon still may recommend removing a single large tumor. […] In some cases, providers recommend other treatments to control tumor growth, including: Removing as much of a liver tumor as possible, a procedure known as debulking. Attempting to destroy the tumor by cutting off the blood supply, called embolization. Using heat to destroy cancer cells with a procedure called radiofrequency ablation. Injecting drugs into the tumor to relieve cancer symptoms. Using chemotherapy to try to slow tumor growth. Having a liver transplant.

• #21 Zollinger-Ellison syndrome | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/zollinger-ellison-syndrome?content_id=CON-20379029

  Treatment of Zollinger-Ellison syndrome focuses on treating the hormone-secreting tumors as well as the ulcers they cause. […] An operation to remove the gastrinomas requires a skilled surgeon because the tumors are often small and difficult to find. If you have just one tumor, a medical professional may be able to remove it surgically. But surgery may not be an option if you have many tumors or tumors that have spread to your liver. On the other hand, even if you have multiple tumors, your surgeon still may recommend removing a single large tumor. […] In some cases, providers recommend other treatments to control tumor growth, including: Removing as much of a liver tumor as possible, a procedure known as debulking. Attempting to destroy the tumor by cutting off the blood supply, called embolization. Using heat to destroy cancer cells with a procedure called radiofrequency ablation. Injecting drugs into the tumor to relieve cancer symptoms. Using chemotherapy to try to slow tumor growth. Having a liver transplant.

• #22 Gastrinomas | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/pancreatic-nets/gastrinoma/treatment

  The main treatment to stop you from making too much acid are drugs called proton pump inhibitors (PPIs). You usually have 1 of the following drugs: omeprazole, lansoprazole, pantoprazole, esomeprazole. […] You might have other treatments if: you can’t have surgery to remove the NET, the NET has spread to another part of the body, the NET came back after the initial treatment. […] These treatments can control your symptoms and help you feel better but won’t get rid of the NET. There are a number of different treatment options: somatostatin analogues – drugs include octreotide and lanreotide, peptide receptor radionuclide therapy (PRRT) – this uses a radioactive medicine to treat the NET, chemotherapy drugs include temozolomide, fluorouracil, capecitabine, streptozotocin, targeted cancer drugs drugs include everolimus and sunitinib. […] For cancer that has spread to your liver you might have: radiofrequency ablation and microwave ablation, surgery to remove cancer from your liver, a liver transplant this is a rare treatment for NETs.

• #23 Zollinger-Ellison syndrome | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/zollinger-ellison-syndrome?content_id=CON-20379029

  Excess acid production can almost always be controlled. Medicines known as proton pump inhibitors are the first line of treatment. These are effective medicines for controlling acid production in Zollinger-Ellison syndrome. […] Proton pump inhibitors are powerful acid-reducing medicines. They work by blocking the action of the tiny „pumps” within acid-secreting cells. Commonly prescribed medicines include lansoprazole (Prevacid), omeprazole (Prilosec, Zegerid), pantoprazole (Protonix), rabeprazole (Aciphex) and esomeprazole (Nexium). Talk with your care professional about the risks of taking these medicines long term. […] Octreotide (Sandostatin), a medicine similar to the hormone somatostatin, may counteract the effects of gastrin and be helpful for some people.

• #24 Treatment of Zollinger-Ellison Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4320348/

  In this article, we have reviewed the main therapeutic measures for the treatment of Zollinger-Ellison syndrome (ZES). […] In ZES patients, the best therapeutic procedure is surgery which, if radical, can be curative. Medical treatment can be the best palliative therapy and should be used, when possible, in association with surgery, in a multimodal therapeutic approach. […] The goal of ZES therapy is to obtain improvement of the symptoms and healing of the ulcers by controlling the gastric hypersecretion. […] Omeprazole, the first proton pump inhibitor available, belongs to a new class of drugs which has proven to be safe and effective in controlling gastric hypersecretion in patients with ZES and has completely substituted the use of H2 antagonists. […] Somatostatin analogs can be useful in reducing gastric acid hypersecretion, serum gastrin and gastric ECL-cells, thus contributing to curing the disease more effectively.

• #25 Treatment of Zollinger-Ellison Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4320348/

  In this article, we have reviewed the main therapeutic measures for the treatment of Zollinger-Ellison syndrome (ZES). […] In ZES patients, the best therapeutic procedure is surgery which, if radical, can be curative. Medical treatment can be the best palliative therapy and should be used, when possible, in association with surgery, in a multimodal therapeutic approach. […] The goal of ZES therapy is to obtain improvement of the symptoms and healing of the ulcers by controlling the gastric hypersecretion. […] Omeprazole, the first proton pump inhibitor available, belongs to a new class of drugs which has proven to be safe and effective in controlling gastric hypersecretion in patients with ZES and has completely substituted the use of H2 antagonists. […] Somatostatin analogs can be useful in reducing gastric acid hypersecretion, serum gastrin and gastric ECL-cells, thus contributing to curing the disease more effectively.

• #26 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

  Gastrinoma and Zollinger Ellison syndrome: A roadmap for the management between new and old therapies. The proper management of Zollinger-Ellison syndrome due to a gastrinoma includes both the medical treatment for symptoms relief and surgery whenever feasible with curative intent; the multidisciplinary approach, with close cooperation between gastroenterologists and surgeons, and the referral to tertiary centers with great expertise in the neuroendocrine field are mandatory. 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 excision is generally recommended either for sporadic gastrinoma or for MEN-1 associated gastrinoma if complete tumor removal is possible. 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. Only when PPIs are unable to control gastric acid secretion, SSAs can be considered, as they reduce gastrin secretion, even if they do not represent a first-line treatment at least for symptom control. The multitargeted tyrosine kinase inhibitor, sunitinib, has demonstrated an improved progression-free survival from 5.5 mo to 11.4 mo in metastatic pNENs. Peptide receptor radionuclide therapy (PRRT) may be the most promising systemic therapy, and it has been repeatedly reported as particularly useful for symptom relief in functioning forms, even if this aspect might be less important in the setting of gastrinomas due to concomitant PPI treatment which is considered to be the first-line approach for symptoms control.

• #27 Gastrinoma Treatment & Management: Medical Care, Surgical Care, Long-Term Monitoring

  https://emedicine.medscape.com/article/184332-treatment

  Granberg et al described a patient with almost complete response to treatment with Sandostatin LAR, a long-acting somatostatin analog. […] Interferon or targeted radiotherapy may also be considered in patients who are not candidates for chemotherapy. Treatment of metastasized gastrinoma with targeted somatostatin-based radiotherapy with repeated cycles of 90yttrium-labeled tetraazacyclododecane-tetraacetic acid modified Tyr-octreotide ([90Y-DOTA]-TOC) or with cycles alternating between [90Y-DOTA]-TOC and 177lutetium-labeled DOTA-TOC ([177Lu-DOTA]-TOC) appears to improve the overall survival. […] Surgical care is indicated for localized disease; resection is the treatment of choice for primary pancreatic neuroendocrine tumors (PNETs), because it is associated with increased survival. […] Surgical resection of localized disease leads to a complete cure without any recurrence in 20-25% of patients with gastrinomas.

• #28 Gastrinomas | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/pancreatic-nets/gastrinoma/treatment

  The main treatment to stop you from making too much acid are drugs called proton pump inhibitors (PPIs). You usually have 1 of the following drugs: omeprazole, lansoprazole, pantoprazole, esomeprazole. […] You might have other treatments if: you can’t have surgery to remove the NET, the NET has spread to another part of the body, the NET came back after the initial treatment. […] These treatments can control your symptoms and help you feel better but won’t get rid of the NET. There are a number of different treatment options: somatostatin analogues – drugs include octreotide and lanreotide, peptide receptor radionuclide therapy (PRRT) – this uses a radioactive medicine to treat the NET, chemotherapy drugs include temozolomide, fluorouracil, capecitabine, streptozotocin, targeted cancer drugs drugs include everolimus and sunitinib. […] For cancer that has spread to your liver you might have: radiofrequency ablation and microwave ablation, surgery to remove cancer from your liver, a liver transplant this is a rare treatment for NETs.

• #29 Gastrinoma – Endocrine and Metabolic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/endocrine-and-metabolic-disorders/gastrointestinal-and-pancreatic-neuroendocrine-tumors-nets/gastrinoma

  A gastrinoma is a gastrin-producing tumor usually located in the pancreas or the duodenal wall. […] Treatment is proton pump inhibitors and surgical removal. […] Proton pump inhibitors are the medications of choice (eg, omeprazole or esomeprazole 40 mg orally 2 times a day). […] A maintenance dose is needed; patients need to take these medications indefinitely unless they undergo surgery. […] Octreotide injections, 100 to 500 mcg subcutaneously twice a day to 3 times a day, may also decrease gastric acid production and may be palliative in patients who are not responding well to proton pump inhibitors. […] Surgical removal should be attempted in patients without apparent metastases because of the high risk of underlying cancer. […] Surgical cure is possible in 20 to 25% of patients if the gastrinoma is localized and not part of a multiple endocrine neoplasia syndrome. […] In patients with metastatic disease, streptozocin in combination with 5-fluorouracil or doxorubicin is the preferred chemotherapy for islet cell tumors. […] Newer chemotherapeutic regimens may be helpful in patients with advanced metastatic disease.

• #30 Gastrinoma Treatment & Management: Medical Care, Surgical Care, Long-Term Monitoring

  https://emedicine.medscape.com/article/184332-treatment

  Individualize the selection of treatment. Base the treatment on factors related to ulcer disease, diarrhea, and malignant properties of the tumor. Antisecretory medications are helpful for controlling the manifestations of peptic acid disease and secretory diarrhea (secondary to hyperacidity). […] These are highly effective drugs and are the drugs of choice for suppressing acid secretion. Long duration of action, fewer adverse effects, and high potency make them superior to H2 blockers. […] Although a good success rate exists, this treatment has been reported to fail in 50% of patients. […] This is indicated in patients with metastatic disease and in patients who are not candidates for surgery; however, it is not indicated for metastatic disease confined to the lymph nodes. […] Chemotherapy reduces tumor size and improves the symptoms secondary to metastatic effects of the tumor.

• #31 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

  Gastrinoma and Zollinger Ellison syndrome: A roadmap for the management between new and old therapies. The proper management of Zollinger-Ellison syndrome due to a gastrinoma includes both the medical treatment for symptoms relief and surgery whenever feasible with curative intent; the multidisciplinary approach, with close cooperation between gastroenterologists and surgeons, and the referral to tertiary centers with great expertise in the neuroendocrine field are mandatory. 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 excision is generally recommended either for sporadic gastrinoma or for MEN-1 associated gastrinoma if complete tumor removal is possible. 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. Only when PPIs are unable to control gastric acid secretion, SSAs can be considered, as they reduce gastrin secretion, even if they do not represent a first-line treatment at least for symptom control. The multitargeted tyrosine kinase inhibitor, sunitinib, has demonstrated an improved progression-free survival from 5.5 mo to 11.4 mo in metastatic pNENs. Peptide receptor radionuclide therapy (PRRT) may be the most promising systemic therapy, and it has been repeatedly reported as particularly useful for symptom relief in functioning forms, even if this aspect might be less important in the setting of gastrinomas due to concomitant PPI treatment which is considered to be the first-line approach for symptoms control.

• #32 Gastrinomas | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/pancreatic-nets/gastrinoma/treatment

  The main treatment to stop you from making too much acid are drugs called proton pump inhibitors (PPIs). You usually have 1 of the following drugs: omeprazole, lansoprazole, pantoprazole, esomeprazole. […] You might have other treatments if: you can’t have surgery to remove the NET, the NET has spread to another part of the body, the NET came back after the initial treatment. […] These treatments can control your symptoms and help you feel better but won’t get rid of the NET. There are a number of different treatment options: somatostatin analogues – drugs include octreotide and lanreotide, peptide receptor radionuclide therapy (PRRT) – this uses a radioactive medicine to treat the NET, chemotherapy drugs include temozolomide, fluorouracil, capecitabine, streptozotocin, targeted cancer drugs drugs include everolimus and sunitinib. […] For cancer that has spread to your liver you might have: radiofrequency ablation and microwave ablation, surgery to remove cancer from your liver, a liver transplant this is a rare treatment for NETs.

• #33 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

  Gastrinoma and Zollinger Ellison syndrome: A roadmap for the management between new and old therapies. The proper management of Zollinger-Ellison syndrome due to a gastrinoma includes both the medical treatment for symptoms relief and surgery whenever feasible with curative intent; the multidisciplinary approach, with close cooperation between gastroenterologists and surgeons, and the referral to tertiary centers with great expertise in the neuroendocrine field are mandatory. 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 excision is generally recommended either for sporadic gastrinoma or for MEN-1 associated gastrinoma if complete tumor removal is possible. 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. Only when PPIs are unable to control gastric acid secretion, SSAs can be considered, as they reduce gastrin secretion, even if they do not represent a first-line treatment at least for symptom control. The multitargeted tyrosine kinase inhibitor, sunitinib, has demonstrated an improved progression-free survival from 5.5 mo to 11.4 mo in metastatic pNENs. Peptide receptor radionuclide therapy (PRRT) may be the most promising systemic therapy, and it has been repeatedly reported as particularly useful for symptom relief in functioning forms, even if this aspect might be less important in the setting of gastrinomas due to concomitant PPI treatment which is considered to be the first-line approach for symptoms control.

• #34 Gastrinomas | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/pancreatic-nets/gastrinoma/treatment

  The main treatment to stop you from making too much acid are drugs called proton pump inhibitors (PPIs). You usually have 1 of the following drugs: omeprazole, lansoprazole, pantoprazole, esomeprazole. […] You might have other treatments if: you can’t have surgery to remove the NET, the NET has spread to another part of the body, the NET came back after the initial treatment. […] These treatments can control your symptoms and help you feel better but won’t get rid of the NET. There are a number of different treatment options: somatostatin analogues – drugs include octreotide and lanreotide, peptide receptor radionuclide therapy (PRRT) – this uses a radioactive medicine to treat the NET, chemotherapy drugs include temozolomide, fluorouracil, capecitabine, streptozotocin, targeted cancer drugs drugs include everolimus and sunitinib. […] For cancer that has spread to your liver you might have: radiofrequency ablation and microwave ablation, surgery to remove cancer from your liver, a liver transplant this is a rare treatment for NETs.

• #35 Gastrinoma Treatment & Management: Medical Care, Surgical Care, Long-Term Monitoring

  https://emedicine.medscape.com/article/184332-treatment

  Granberg et al described a patient with almost complete response to treatment with Sandostatin LAR, a long-acting somatostatin analog. […] Interferon or targeted radiotherapy may also be considered in patients who are not candidates for chemotherapy. Treatment of metastasized gastrinoma with targeted somatostatin-based radiotherapy with repeated cycles of 90yttrium-labeled tetraazacyclododecane-tetraacetic acid modified Tyr-octreotide ([90Y-DOTA]-TOC) or with cycles alternating between [90Y-DOTA]-TOC and 177lutetium-labeled DOTA-TOC ([177Lu-DOTA]-TOC) appears to improve the overall survival. […] Surgical care is indicated for localized disease; resection is the treatment of choice for primary pancreatic neuroendocrine tumors (PNETs), because it is associated with increased survival. […] Surgical resection of localized disease leads to a complete cure without any recurrence in 20-25% of patients with gastrinomas.

• #36 Gastrinoma – Knowledge @ AMBOSS

  https://www.amboss.com/us/knowledge/gastrinoma/

  Gastrinomas are primarily managed by specialists (e.g., gastroenterology, oncology, surgery). […] Acid suppression medications: indicated for all patients with gastrinoma. First-line: PPIs (e.g., omeprazole). Second-line: H2RBs (e.g., famotidine). […] Somatostatin analog: (e.g., octreotide): consider in advanced disease. […] Exploration laparotomy: all patients with sporadic gastrinomas to identify tumors not seen on imaging. Resection considered for localized, sporadic gastrinomas. Resection considered for gastrinoma with MEN 1 if tumor size is 2 cm. […] Liver transplant: considered for patients with metastases confined to the liver. […] Consider the following therapies in patients with advanced or refractory disease: Chemotherapy (e.g., streptozocin, 5-fluorouracil, doxorubicin), Radiation (e.g., peptide receptor-targeted radiotherapy), Liver-directed therapy (e.g., chemoembolization) in patients with metastases primarily in the liver. […] Consider the following for patients with active nonmetastatic disease: Biochemical studies: every 3-12 months (i.e., levels of gastrin, calcium, PTH, vitamin B12), Cross sectional imaging: annually, Somatostatin receptor scintigraphy: at least every 3 years.

• #37 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

  Gastrinoma and Zollinger Ellison syndrome: A roadmap for the management between new and old therapies. The proper management of Zollinger-Ellison syndrome due to a gastrinoma includes both the medical treatment for symptoms relief and surgery whenever feasible with curative intent; the multidisciplinary approach, with close cooperation between gastroenterologists and surgeons, and the referral to tertiary centers with great expertise in the neuroendocrine field are mandatory. 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 excision is generally recommended either for sporadic gastrinoma or for MEN-1 associated gastrinoma if complete tumor removal is possible. 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. Only when PPIs are unable to control gastric acid secretion, SSAs can be considered, as they reduce gastrin secretion, even if they do not represent a first-line treatment at least for symptom control. The multitargeted tyrosine kinase inhibitor, sunitinib, has demonstrated an improved progression-free survival from 5.5 mo to 11.4 mo in metastatic pNENs. Peptide receptor radionuclide therapy (PRRT) may be the most promising systemic therapy, and it has been repeatedly reported as particularly useful for symptom relief in functioning forms, even if this aspect might be less important in the setting of gastrinomas due to concomitant PPI treatment which is considered to be the first-line approach for symptoms control.

• #38 Treatment of Zollinger-Ellison Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4320348/

  The role of surgery in the treatment of ZES has evolved considerably from 1955 to now. […] Since adequate medical therapy is able to eliminate the symptoms in all patients and, although it is hard to treat the neoplastic disease with chemotherapeutic drugs, some authors claim that the risks and complications of exeresis of the gastrinoma exceed the actual benefits of this procedure. […] At present, surgical exploration plays a key role in identifying and removing the primary tumor, and in preventing the onset of liver metastases. […] In conclusion, chemotherapy is not the treatment of first choice in patients with gastrin secreting tumors, but seems to be indicated in rapidly evolving tumors in which the mass of the primary tumor increases more than 25% in a period of follow-up of 12 mo or in which the tumoral symptoms cannot be treated by other means. […] Finally, we would like to emphasize that the choice of treatment must involve a multidisciplinary approach.

• #39 Gastrinomas | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/types/pancreatic-nets/gastrinoma

  Surgery is the main treatment for gastrinomas. But surgery is not always possible. Some gastrinomas might have already started to spread. Or you may not be well enough to have an operation. […] You might have other treatments if surgery isn’t an option. […] A team of doctors and other professionals discuss the best treatment and care for you. They are called a multidisciplinary team (MDT). […] The treatment you have depends on: where the NET is and its size, how many tumours you have, whether it has spread to other parts of the body, your general health and fitness, the symptoms you have. […] Your doctor will discuss your treatment. And tell you about its benefits and the possible side effects. […] Surgery to remove the gastrinoma is usually the main treatment. The type of surgery you have depends on whether the gastrinoma started in your duodenum or the pancreas.

• #40 Gastrinomas | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/types/pancreatic-nets/gastrinoma

  Surgery is the main treatment for gastrinomas. But surgery is not always possible. Some gastrinomas might have already started to spread. Or you may not be well enough to have an operation. […] You might have other treatments if surgery isn’t an option. […] A team of doctors and other professionals discuss the best treatment and care for you. They are called a multidisciplinary team (MDT). […] The treatment you have depends on: where the NET is and its size, how many tumours you have, whether it has spread to other parts of the body, your general health and fitness, the symptoms you have. […] Your doctor will discuss your treatment. And tell you about its benefits and the possible side effects. […] Surgery to remove the gastrinoma is usually the main treatment. The type of surgery you have depends on whether the gastrinoma started in your duodenum or the pancreas.

• #41 Zollinger-Ellison Syndrome Treatment & Management: Medical Care, Surgical Care

  https://emedicine.medscape.com/article/183555-treatment

  All patients with sporadic Zollinger-Ellison syndrome (ZES) without hepatic metastases or medical contraindications to surgery are advised to undergo surgical resection of the tumor because this decreases the risk of developing liver metastases, which can decrease the survival of these patients. […] An attempt at surgical resection has been recommended if the tumor is larger than 2.5 cm. […] After surgical resection of a gastrinoma, patients should be assessed for evidence of recurrence with serum fasting gastrin levels, a secretin test, and SRS. […] Proton pump inhibitors can be continued with the goal of maintaining the basal acid output (BAO) below 10 mEq/h before the next dose of the proton pump inhibitors.

• #42 Zollinger-Ellison Syndrome Treatment & Management: Medical Care, Surgical Care

  https://emedicine.medscape.com/article/183555-treatment

  All patients with sporadic Zollinger-Ellison syndrome (ZES) without hepatic metastases or medical contraindications to surgery are advised to undergo surgical resection of the tumor because this decreases the risk of developing liver metastases, which can decrease the survival of these patients. […] An attempt at surgical resection has been recommended if the tumor is larger than 2.5 cm. […] After surgical resection of a gastrinoma, patients should be assessed for evidence of recurrence with serum fasting gastrin levels, a secretin test, and SRS. […] Proton pump inhibitors can be continued with the goal of maintaining the basal acid output (BAO) below 10 mEq/h before the next dose of the proton pump inhibitors.

• #43 Zollinger-Ellison Syndrome: Challenges and Management Strategies

  https://www.longdom.org/open-access/zollingerellison-syndrome-challenges-and-management-strategies-102334.html

  Zollinger-Ellison Syndrome (ZES) is a rare and chronic disorder characterized by the development of gastrin-secreting tumors called gastrinomas, typically found in the pancreas or duodenum. […] Managing ZES is a lifelong challenge for those affected, as it demands a comprehensive approach that encompasses medical, dietary, and sometimes surgical interventions. […] The primary goal of medical management in ZES is to suppress gastric acid production. Proton pump inhibitors (PPIs) are the cornerstone of therapy, as they effectively reduce stomach acid secretion. […] In cases where medical therapy is insufficient or when complications like bleeding or obstruction occur, surgical intervention may be necessary. […] Regular monitoring and surveillance are essential to assess disease progression, tumor growth, and treatment effectiveness. […] Nutritional management plays a crucial role in ZES. […] Certain lifestyle changes can be beneficial for individuals with ZES. […] Coping with the challenges of ZES can be overwhelming, and psychological support is vital.

• #44 Zollinger-Ellison Syndrome – Endocrine Surgery | UCLA Health

  https://www.uclahealth.org/medical-services/surgery/endocrine-surgery/patient-resources/patient-education/endocrine-surgery-encyclopedia/zollinger-ellison-syndrome

  A class of acid-suppressing medications called proton pump inhibitors (for example, omeprazole, lansoprazole) is now the first choice of treatment of Zollinger-Ellison syndrome. These drugs dramatically reduce acid production by the stomach, and promote healing of ulcers in the stomach and small bowel. They also provide relief of abdominal pain and diarrhea. […] Surgical removal of a single gastrinoma may be attempted if there is no evidence that it has spread to other organs (such as lymph nodes or the liver). Surgery on the stomach (gastrectomy) to control acid production is rarely necessary today. […] Early diagnosis and surgical removal of the tumor is associated with a cure rate of only 20% to 25%. However, gastrinomas grow slowly, and patients may live for many years after the tumor is discovered. Acid-suppressing medications are very effective at controlling the symptoms of acid overproduction.

• #45 Zollinger-Ellison Syndrome – Endocrine Surgery | UCLA Health

  https://www.uclahealth.org/medical-services/surgery/endocrine-surgery/patient-resources/patient-education/endocrine-surgery-encyclopedia/zollinger-ellison-syndrome

  A class of acid-suppressing medications called proton pump inhibitors (for example, omeprazole, lansoprazole) is now the first choice of treatment of Zollinger-Ellison syndrome. These drugs dramatically reduce acid production by the stomach, and promote healing of ulcers in the stomach and small bowel. They also provide relief of abdominal pain and diarrhea. […] Surgical removal of a single gastrinoma may be attempted if there is no evidence that it has spread to other organs (such as lymph nodes or the liver). Surgery on the stomach (gastrectomy) to control acid production is rarely necessary today. […] Early diagnosis and surgical removal of the tumor is associated with a cure rate of only 20% to 25%. However, gastrinomas grow slowly, and patients may live for many years after the tumor is discovered. Acid-suppressing medications are very effective at controlling the symptoms of acid overproduction.

• #46 Zollinger-Ellison Syndrome: Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17960-zollinger-ellison-syndrome

  Your provider may recommend chemotherapy. This treatment may ease symptoms and slow down how quickly the gastrinomas spread. […] 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.

• #47 Successful Lifetime/Long-Term Medical Treatment of Acid Hypersecretion in Zollinger-Ellison Syndrome (ZES): Myth or Fact? Insights from an Analysis of Results of NIH Long-Term Prospective Studies of ZES

  https://www.mdpi.com/2072-6694/15/5/1377

  Long-term/lifelong acid antisecretory treatment with H2Rs/PPIs can be successfully carried out in all patients with both uncomplicated and complicated ZES (i.e., with MEN1/ZES, previous Billroth 2, severe GERD). This is only possible if drug doses are individually set by assessing acid secretory control to establish proven criteria, with regular reassessments and readjustments. […] The current study aimed to address these issues. The NIH prospective study on all aspects of ZES has been in effect since 1974, and part of the study involves the long-term medical management of the acid hypersecretion of all patients. In the current study, we analyzed data from this prospective database related to all issues of the long-term efficacy of both H2Rs and PPIs in these patients. […] The most important conclusion of the present study is that long-term/lifelong medical treatment of acid hypersecretion in ZES patients is possible and can be successful in controlling peptic acid symptoms and preventing the development of acid-related complications in all patients. While this statement includes long-term/lifelong treatment with either a PPI or H2R, for all practical purposes, the findings with PPIs will be of primary interest to most clinicians. Presently, because of the greater potency and longer duration of action of PPIs, which allows once or twice a day dosing, PPIs have become the drugs of choice for acid treatment in ZES.

• #48 Gastrinoma Treatment & Management: Medical Care, Surgical Care, Long-Term Monitoring

  https://emedicine.medscape.com/article/184332-treatment

  Granberg et al described a patient with almost complete response to treatment with Sandostatin LAR, a long-acting somatostatin analog. […] Interferon or targeted radiotherapy may also be considered in patients who are not candidates for chemotherapy. Treatment of metastasized gastrinoma with targeted somatostatin-based radiotherapy with repeated cycles of 90yttrium-labeled tetraazacyclododecane-tetraacetic acid modified Tyr-octreotide ([90Y-DOTA]-TOC) or with cycles alternating between [90Y-DOTA]-TOC and 177lutetium-labeled DOTA-TOC ([177Lu-DOTA]-TOC) appears to improve the overall survival. […] Surgical care is indicated for localized disease; resection is the treatment of choice for primary pancreatic neuroendocrine tumors (PNETs), because it is associated with increased survival. […] Surgical resection of localized disease leads to a complete cure without any recurrence in 20-25% of patients with gastrinomas.

• #49 Treatment of Zollinger-Ellison Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4320348/

  In this article, we have reviewed the main therapeutic measures for the treatment of Zollinger-Ellison syndrome (ZES). […] In ZES patients, the best therapeutic procedure is surgery which, if radical, can be curative. Medical treatment can be the best palliative therapy and should be used, when possible, in association with surgery, in a multimodal therapeutic approach. […] The goal of ZES therapy is to obtain improvement of the symptoms and healing of the ulcers by controlling the gastric hypersecretion. […] Omeprazole, the first proton pump inhibitor available, belongs to a new class of drugs which has proven to be safe and effective in controlling gastric hypersecretion in patients with ZES and has completely substituted the use of H2 antagonists. […] Somatostatin analogs can be useful in reducing gastric acid hypersecretion, serum gastrin and gastric ECL-cells, thus contributing to curing the disease more effectively.

• #50 Treatment of Zollinger-Ellison Syndrome

  https://www.wjgnet.com/1007-9327/full/v11/i35/5423.htm

  The main objectives of surgery are to stage the tumor, improve survival by removing the malignant tumor and control the acid hypersecretion syndrome. […] In conclusion, chemotherapy is not the treatment of first choice in patients with gastrin secreting tumors, but seems to be indicated in rapidly evolving tumors in which the mass of the primary tumor increases more than 25% in a period of follow-up of 12 mo or in which the tumoral symptoms cannot be treated by other means. […] Finally, we would like to emphasize that the choice of treatment must involve a multidisciplinary approach.

• #51 Zollinger-Ellison Syndrome – NIDDK

  https://www.niddk.nih.gov/health-information/digestive-diseases/zollinger-ellison-syndrome

  Doctors treat Zollinger-Ellison syndrome with medicines and surgery. […] Doctors treat Zollinger-Ellison syndrome with PPIs. PPIs lower the amount of acid your stomach makes, which can relieve symptoms and promote healing. Doctors may prescribe a higher dose of PPIs when starting treatment and may lower the dose over time. Many people with Zollinger-Ellison syndrome need lifelong treatment with PPIs. […] Doctors may recommend surgery to remove the tumors that cause Zollinger-Ellison syndrome in people who don’t have MEN1. In some cases, removing the gastrinomas will cure Zollinger-Ellison syndrome and prevent the tumors from spreading to other parts of the body. After surgery, people may still need to take PPIs to control stomach acid. […] Treating Zollinger-Ellison syndrome reduces the amount of acid in the stomach, which can help prevent or treat complications related to peptic ulcers or acid reflux. […] If gastrinomas are cancerous and spread to the liver, bone, or other parts of the body, doctors may recommend chemotherapy, surgery, or other cancer treatments.

• #52 ZOLLINGER-ELLISON SYNDROME (GASTRINOMA) | Harrison’s Manual of Medicine

  https://harrisons.unboundmedicine.com/harrisons/view/Harrisons-Manual-of-Medicine/623366/2.1/ZOLLINGER_ELLISON_SYNDROME__GASTRINOMA_

  Omeprazole (or lansoprazole), beginning at 60 mg PO q A.M. and increasing until maximal gastric acid output is 10 mmol/h before next dose, is drug of choice during evaluation and in pts who are not surgical candidates; dose can often be reduced over time. […] Radiolabeled octreotide scanning has emerged as the most sensitive test for detecting primary tumors and metastases; may be supplemented by endoscopic ultrasonography. […] Exploratory laparotomy with resection of primary tumor and solitary metastases is done when possible. […] In pts with MEN 1, tumor is often multifocal and unresectable; treat hyperparathyroidism first (hypergastrinemia may improve). […] For unresectable tumors, parietal cell vagotomy may enhance control of ulcer disease by drugs. […] Chemotherapy or biologic therapy is used for metastatic tumor to control symptoms (e.g., streptozocin, 5-fluorouracil, doxorubicin, or interferon); 40% partial response rate. […] Long-acting and radioactive somatostatin analogues and a combination of temozolomide plus capecitabine may produce regression or disease stabilization. […] VEGF antagonists and mTOR inhibitors are being tested.

• #53 Zollinger Ellison Syndrome Mnemonic

  https://www.picmonic.com/pathways/medicine/courses/standard/pathology-196/neuroendocrine-disorders-39305/zollinger-ellison-syndrome-diagnosis-and-treatment_1343

  Surgery is the only modality of treatment which may lead to a cure. This, however, is hinged on the tumor being in a resectable area and non-metastatic. All sporadic cases of Zollinger-Ellison syndrome (ZES) should be surgically explored (including duodenotomy) even with negative imaging results, because of the high likelihood of finding and removing a tumor for potential cure. Surgery for multiple endocrine neoplasia type 1 (MEN 1) related cases should be focused on prevention of metastatic disease, with surgery being recommended when pancreatic tumors are greater than 2 cm.

• #54 Gastrinomas | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/pancreatic-nets/gastrinoma/treatment

  The main treatment to stop you from making too much acid are drugs called proton pump inhibitors (PPIs). You usually have 1 of the following drugs: omeprazole, lansoprazole, pantoprazole, esomeprazole. […] You might have other treatments if: you can’t have surgery to remove the NET, the NET has spread to another part of the body, the NET came back after the initial treatment. […] These treatments can control your symptoms and help you feel better but won’t get rid of the NET. There are a number of different treatment options: somatostatin analogues – drugs include octreotide and lanreotide, peptide receptor radionuclide therapy (PRRT) – this uses a radioactive medicine to treat the NET, chemotherapy drugs include temozolomide, fluorouracil, capecitabine, streptozotocin, targeted cancer drugs drugs include everolimus and sunitinib. […] For cancer that has spread to your liver you might have: radiofrequency ablation and microwave ablation, surgery to remove cancer from your liver, a liver transplant this is a rare treatment for NETs.

• #55 Gastrinoma – Knowledge @ AMBOSS

  https://www.amboss.com/us/knowledge/gastrinoma/

  Gastrinomas are primarily managed by specialists (e.g., gastroenterology, oncology, surgery). […] Acid suppression medications: indicated for all patients with gastrinoma. First-line: PPIs (e.g., omeprazole). Second-line: H2RBs (e.g., famotidine). […] Somatostatin analog: (e.g., octreotide): consider in advanced disease. […] Exploration laparotomy: all patients with sporadic gastrinomas to identify tumors not seen on imaging. Resection considered for localized, sporadic gastrinomas. Resection considered for gastrinoma with MEN 1 if tumor size is 2 cm. […] Liver transplant: considered for patients with metastases confined to the liver. […] Consider the following therapies in patients with advanced or refractory disease: Chemotherapy (e.g., streptozocin, 5-fluorouracil, doxorubicin), Radiation (e.g., peptide receptor-targeted radiotherapy), Liver-directed therapy (e.g., chemoembolization) in patients with metastases primarily in the liver. […] Consider the following for patients with active nonmetastatic disease: Biochemical studies: every 3-12 months (i.e., levels of gastrin, calcium, PTH, vitamin B12), Cross sectional imaging: annually, Somatostatin receptor scintigraphy: at least every 3 years.

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