Primary Hepatic Gastrinoma Causing Zollinger-Ellison Syndrome: A Rare and Challenging Diagnosis (original) (raw)
Related papers
Zollinger-Ellison syndrome: Revelation of the gastrinoma triangle
Zollinger-Ellison syndrome is a complex condition in which one or more tumors form in the patient’s pancreas or upper duodenum. These tumors, called gastrinomas, secrete excessive amounts of gastrin, and almost all develop ulcers. The vast majority of gastrinomas are present within the “gastrinoma triangle,” which is composed of the porta hepatis, duodenal sweep, and pancreatic head. As surgery remains the treatment of choice, localization of the primary lesion is often challenging but essential. We present a 50-year-old man with a tentative diagnosis of Zollinger-Ellison syndrome. His In-111 pentetreotide scan, fused onto a Tc-99m abdomen image, revealed an avid lesion adjacent to the duodenal loop. Operative resection was performed, and a primary pancreatic gastrinoma was diagnosed by immunohistochemical staining. The neuroendocrine tumors have somatostatin receptors upon them. Therefore, a penteteotide scan, using In-111 radiolabelled somatostatin analogues, is the current technique of choice. This dual-isotope display permits a visual perception of anatomic landmarks around the lesion.
Annals of Surgery, 1986
In 1982, a prospective study was initiated of 52 consecutive patients ts with proven Zollinger-Ellison syndrome (ZES), involving surgical exploration with the goal of removing the gastrinoma after an extensive protocol to localize the tumor. Each patient underwent ultrasound, computed tomography (CT) with oral/ intravenous (IV) contrast, and selective arteriography. Eighteen patients had metastatic disease identified by imaging studies and confirmed by percutaneous biopsies, and two patients had multiple endocrine neoplasia type I (MEN-I) with negative imaging studies; therefore, these 20 patients did not undergo laparotomy. Each of the remaining 32 patients (3 with MEN-I and positive imaging studies) underwent laparotomy, and gastrinomas were removed in 20 patients. Preoperative ultrasound localized tumors in 20% of patients, CT in 40%, arteriography in 60%, and any of the modalities in 70% of patients. Infusion CT and arteriography were 100% specific. In 18 patients with either negative imaging (17) or false-positive imaging (1 ultrasound), gastrinomas were found and removed in six patients (33%). Twenty-four gastrinomas were found in 20 patients at laparotomy: eight in lymph nodes around the pancreatic head, four in the pancreatic head, one in the pancreatic body, three in the pancreatic tail, three in the pyloric channel, one in the duodenal wall, two in the jejunum at the ligament of Treitz, one in the ovary, and multiple liver metastases in one patient. If one excludes patients with MEN-I or liver metastatic disease, 12/28 (43%) of patients were biochemically "cured" immediately after operation. This result decreased to 7/23 (30%) with greater than 6 months follow-up. No patients with gastrinomas resected have developed recurrent gastrinoma on follow-up imaging studies (longest follow-up: 4 years). This study indicates that 95% of metastatic gastrinoma can be diagnosed before operation and that, by a combination of careful imaging studies and thorough exploration at surgery, 30% of patients with gastrinomas may be curable. I N 1954, ZOLLINGER AND ELLISON first described a syndrome associated with severe peptic ulcer diathesis and pancreatic tumors.' The syndrome would later bear their name, and their initial treatment, total
Sporadic Duodenal Gastrinoma causing Zollinger Ellison Syndrome – A Rare Case Report
International Journal of Contemporary Medical Research [IJCMR], 2019
Introduction: Gastrinoma is a rare gastrin secreting neuroendocrine tumor. Clinical manifestations are nonspecific gastrointestinal (GI) symptoms or as Zollinger Ellison Syndrome(ZES) which often delays the diagnosis in majority of patients. Hence a high clinical index of suspicion is warranted in patients who present with nonspecific GI symptoms to rule out gastrinoma. Case report: We report a case of 55 year old female who presented with long standing dyspepsia and chronic diarrhoea and was diagnosed with Sporadic Duodenal Gastrinoma on detailed evaluation. She was treated with surgical resection and pathologically diagnosed as well differentiated neuroendocrine tumor, Gastrinoma-grade I after IHC analysis. Patient was relieved of her symptoms and became eugastrinemic after surgery. Conclusion: Any patient with refractory dyspepsia associated with long standing non specific abdominal symptoms should be evaluated for Gastrinoma and treated accordingly to avoid misdiagnosis and complications in the long run.
Incidence and Prognosis of Primary Gastrinomas in the Hepatobiliary Tract
JAMA surgery, 2018
Zollinger-Ellison syndrome (ZES) is a life-threatening disease caused by a malignant tumor that secretes gastrin (gastrinoma). Gastrinomas typically occur in the pancreas or the duodenum. To describe the incidence and prognosis of very unusual gastrinomas originating in the hepatobiliary tract. This study included 223 consecutive patients at the National Institutes of Health and Stanford University Hospital who were enrolled in a prospective protocol to treat ZES using proton pump inhibitors to control acid hypersecretion and surgical resection to ameliorate the tumoral process. Data were collected from June 1982 to August 2017. Incidence, location, surgical results, and cure rate and overall survival among patients with gastrinomas that originate in the liver or bile ducts. Cure was defined as serum gastrin levels within the reference range, negative results of a secretin test, and no tumor found on imaging. Of the 223 patients who underwent surgery to remove gastrinomas, 7 (3.1%) ...
Pathomorphologic, biochemical, and diagnostic aspects of gastrinomas (Zollinger-Ellison syndrome
Human Pathology, 1975
The clinical symptomatolog y of the Zollinger-Ellison syndrome and the pathologic anatomy of gastrinomas are reviewed. Experience with 17 patients with the Zollinger-Ellison syndrome is presented with special reference to stimulation tests (secretin, glucagon, calcium infnsion, test meal) and to localization and immunohistologic, tdtrastructural, and biochemical findings in gastrinomas. Muhiple hormone production by the tumors is frequent. The ultrastructure and the Sephadex G-50 gel filtration patterns of imnaunol'eactive gastrin in sera and tumors are not uniform and are not related to localization of the tumors in the pancreas or duodenum or to the gastrin concentration. Hyperplasia of the pancreatic islets is a frequent finding in gastrinorna patients, suggesting that hypergastrinemia may stimnlate islet growth.
International Surgery Journal, 2021
Neuroendocrine tumors of the gastrointestinal tract are rare. Existence of primary nodal gastrinoma is rare and mired with controversies as to whether it is a primary nodal disease or a metastasis to a lymphnode from primary in the pancreas or gastrointestinal tract. They usually occur in the nests of embryonic tissue and lymph nodes in the region of gastrinoma triangle. Rarely the nodes may be located outside the Passaro triangle. Here we report one such case of gastrinoma with only discernable nodal disease located outside the confines of the conventional gastrinoma triangle and discuss the relevant available literature.
The Zollinger-Ellison syndrome. A collective surgical experience
Annals of …, 1992
A retrospective study of 90 surgically treated patients with the Zollinger-Ellison syndrome seen from 1958 through 1990 was performed. Fifteen patients had Zollinger-Ellison syndrome as a manifestation of multiple endocrine neoplasia type I. Preoperative tumor localization was positive in 46% of 54 patients studied. Gastrinomas were identified in 66% of patients, 38% of the tumors being malignant. Postoperative eugastrinemia was achieved in 11% of patients after a variety of surgical procedures. Exploratory laparotomy provides the only chance for cure and identifies the significant prognostic factors associated with longterm patient survival: small tumor size, extrapancreatic primary, and absence of tumor metastases. A LTHOUGH DOCTORS ZOLLINGER and Ellison uncovered the mystery ofhypergastrinemia with their epoch publication in 1955, which recommended total gastrectomy for effective therapy,' multiple improvements in radiographic imaging, pharmacologic therapy, and surgical techniques in the ensuing three decades have made the current treatment of patients with Zollinger-Ellison syndrome (ZES) controversial. Historically, it was believed that tumor excision for cure was possible in only 2% to 5% ofpatients.24 Understandably, with the introduction of pharmacologic agents that essentially shut off gastric acid secretion (histamine-receptor [H2] blockade in 1976 and blockage of the H+-K+ adenosine triphosphatase pump with omeprazole in 1984), some authorities recommended medical therapy in place of surgical treatment for patients with ZES.3 Coupling the initial development (1968)5 and subsequent improved accuracy of gastrin radioimmunoassay with a heightened physician awareness of ZES, patients are currently seen earlier in the course of their disease.
Assessing the Cure of the Zollinger-Ellison Syndrome after Gastrinoma Resection
Annals of Internal Medicine, 1994
References 1. Wanner C, Rader D, Bartens W, Kramer J, Brewer HB, Schollmeyer P, et al. Elevated plasma lipoprotein(a) in patients with the nephrotic syndrome. Ann Intern Med. 1993;119:263-9. 2. Craig WY, Poulin SE, Ledue TB, Kamboh MI. Apolipoprotein(a): a comparison of isoforms identified by sodium dodecyl sulfate-polyacrylamide gel electrophoresis or by sodium dodecyl sulfate-agarose gel electrophoresis. Electrophoresis. 1993;14:1038-41. 3. Ehnholm C, Garoff H, Renkonen O, Simons K. Protein and carbohydrate composition of Lp(a) lipoprotein from human plasma. Biochemistry. 1972;11:3229-32. 4. Glassock RJ, Adler SG, Ward HJ, Cohen AH. Primary glomerular diseases.
World Journal of Surgery, 1993
Studies have shown that the duodenum is a more common site (30-40%) of primary gastrinomas than previously reported. These tumors are often occult to conventional exploration, and their detection requires duodenotomy and meticulous evaluation of the mucosa by eversion and direct palpation. Furthermore, tumors as smaU as 1 to 2 mm may be associated with iymph node metastases. Seven patients with duodenal gastrinomas treated during a 2-year period are reported. Four had sporadic ZoUinger-Ellison syndrome (ZES), and three had multiple endocrine neoplasia type I (MEN-I) syndrome. Ail seven patients had malignant duodenal gastrinomas as proved by metastatic lymph nodes and in one case a large liver metastasis as well. Local excision of the tumor, iymph node excisions, and in one case liver Iobectomy resulted in eugastrinemia in the 4 with sporadic ZES. Two of the three with MEN-I were also eugastrinemic after similar procedures, with the addition of distal pancreatectomy. Duodenotomy is considered an essential procedure in sporadic ZES patients when no pancreatic tumor is found and in ail MEN-I/ZES patients, regardless of the pancreatic findings. Regional lymph node excisions are essential when a duodenal tumor has been found. At one time it was assumed that most patients with Zollinger-Ellison syndrome (ZES) had malignant pancreatic tumors with metastases that precluded a curative surgical resection. Therefore nearly ail patients were treated by total gastrectomy with the exception of a few with primary extrapancreatic tumors [1, 2]. Once drugs became available that adequately controlled gastric acid hypersecretion, more emphasis was placed on identifying ZES patients who were potentially curable by tumor resection. Currently, it is estimated that up to 40% of patients with sporadic ZES have tumors that can be successfully resected [3]. These patients have primary tumors arising in the pancreas or duodenum with or without regional lymph node spread but no liver metastases. However, during the past decade, as many as 40% of patients proved biochemically to have ZES who have undergone exploration have had negative findings [4]. As a result, these patients were subsequently treated with drug therapy or had palliative surgical procedures such as selective vagotomy or even total gastrectomy. Furthermore, in most centers, multiple endocrine neoplasia type I (MEN-I)/ZES patients have been treated by drugs without
The Zollinger–Ellison Syndrome and Mismeasurement of Gastrin
Gastroenterology, 2011
Zollinger-Ellison syndrome (ZES) is characterized by hypersecretion of gastric acid, severe peptic ulcerations in the upper small intestine, and diarrhea. It is usually diagnosed by measuring increased levels of gastrin in plasma. METHODS: We examined the accuracy of commercial kits to measure gastrin (7 radioimmunoassays and 5 enzyme-linked immunosorbent assays), using plasma from 40 patients suspected or known to have ZES. Each sample was analyzed using the 12 kits and a reference assay that measures bioactive gastrin in plasma, irrespective of size and amino acid derivatization. Known concentrations of peptides with identical sequences to circulating gastrins were also assessed by all assays. Molecular patterns in plasma from patients with ZES were examined by chromatography and monitored by kits that measure false-low or falsehigh concentrations of gastrin. RESULTS: Failure to diagnose gastrinomas has serious consequences. Four kits found false-low concentrations of gastrin in 20% to 80% of the patients. Specificity assessment showed that the antibodies used in these kits bound only gastrin-17. Three kits found false-high concentrations of gastrin, because the reagents had increased reactions to sulfated gastrins or to unspecific factors in plasma. Thus, only 5 of 12 kits tested accurately measure plasma concentrations of gastrin. CONCLUSIONS: Seven of 12 tested commercial kits inaccurately measure plasma concentrations of gastrin; these assays used antibodies with inappropriate specificity that were insufficiently validated. Misdiagnosis of gastrinoma based on lack of specificity of assays for gastrin results in ineffective or inappropriate therapy for patients with ZES.