Zollinger ellison syndrome due to primary nodal gastrinoma located outside the limits of the conventional gastrinoma triangle: report of a case (original) (raw)

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.

Management of Primary Lymph Nodal Gastrinoma With Liver Metastases Resulting in Zollinger-Ellison Syndrome

Clinical Nuclear Medicine, 2018

Primary lymph node gastrinoma has been defined as gastrinproducing tumor present in lymph nodes and predominantly found in well-defined anatomical region known as gastrinoma triangle. They are usually localized preoperatively with imaging, and their surgical resection results in long-term relief. The authors report a case of unresectable primary lymph nodal gastrinoma with liver metastases in a 14-year-old adolescent boy with proven histopathology detected on 68 Ga-DOTANOC whole-body PET/CT scan followed by preoperative multiple 177 Lu-DOTATATE cycles for cytoreduction. Subsequent surgical resection of residual mass resulted in complete response with a follow-up of around 4 years in this unusual case of Zollinger-Ellison syndrome.

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.

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.

Prospective Study of Gastrinoma Localization and Resection in Patients with Zollinger-Ellison Syndrome

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

Duodenal gastrinomas, duodenotomy, and duodenal exploration in the surgical management of Zollinger-Ellison syndrome

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

An atypical type I gastric neuroendocrine tumor

Romanian Journal of Internal Medicine

Gastric neuroendocrine tumors(GI-NETs) are rare lesions, usually discovered incidentally during endoscopy. Based on their pathology, there are 4 types of GI-NETs. Type I are multiple small polypoid lesions with central ulceration located in the gastric body or the fundus, associated with atrophic gastritis usually noninvasive and very rarely metastatic. We report on a rare case of a gastric NET arising from the muscularis propria layer of the pyloric ring.We present the case of a 65-year old woman with a history of alcoholic cirrhosis, investigated for melena. Upper endoscopy revealed a 30 mm submucosal pedunculated polypoid lesion located on the pylorus protruding in the duodenum, with normal overlying mucosa, fundic gastric atrophy and multiple small polyps at this level, with no active bleeding. CT scan did not reveal any distant metastases. An ultrasound endoscopy was performed, and a round hypoechoic heterogeneous solitary mass, evolving from the pyloric muscle was described. C...

Regression of Type II Gastric Carcinoids in Multiple Endocrine Neoplasia Type 1 Patients with Zollinger-Ellison Syndrome after Surgical Excision of All Gastrinomas

World Journal of Surgery, 2004

Enterochromaffin-like (ECL) tumors are documented in patients with hypergastrinemia secondary to chronic atrophic gastritis or with Zollinger-Ellison syndrome and multiple endocrine neoplasia type 1 (ZES-MEN-1). In patients with ECL tumors and atrophic gastritis, normogastrinemia after antrectomy has resulted in resolution, regression, or stabilization of ECL tumors. The natural history of ECL tumors associated with ZES-MEN-1 following normalization of gastrin levels after gastrinoma resection has not been previously reported. The purpose of this study was to determine the course of ECL tumors in patients with ZES-MEN-1 following normalization of serum gastrin levels after gastrinoma resection. Two patients with ZES-MEN-1 had biopsy-proven ECL tumors on endoscopic evaluation. They then underwent surgical exploration that included distal pancreatectomy, enucleation of pancreatic head tumors, duodenotomy with excision of submucosal tumors, and peripancreatic lymphadenectomy. Gastric ECL tumors larger than 1.0 cm were locally excised. Patients underwent long-term follow-up with biochemical and endoscopic surveillance. Normogastrinemia was achieved and sustained following gastrinoma resection in two patients with ZES-MEN-1. Periodic endoscopic surveillance over a 6-year period showed complete resolution of the ECL tumors. The development of ECL tumors associated with ZES-MEN-1 is multifactorial. Studies identified a genetic influence on tumor growth with loss of heterozygosity at the MEN-1 gene locus in ECL tumors. The resolution of ECL tumors in ZES-MEN-1 patients who are normogastrinemic indicates that an elevated gastrin level is a primary initiator for development of these tumors. Therefore both genetic defects and hypergastrinemia are causative agents. Normalization of serum gastrin levels is critical for the prevention of aggressive forms of ECL tumors.

Primary Hepatic Gastrinoma Causing Zollinger-Ellison Syndrome: A Rare and Challenging Diagnosis

Cancers, 2012

The majority of gastrinomas causing Zollinger-Ellison syndrome (ZES) are located in the duodenum or the pancreas. Primary hepatic gastrinomas (PHG) are extremely rare and difficult to diagnose because the liver is the commonest site of metastatic disease and gastrinomas can be very small. Furthermore, gastrinomas are typically slow-growing thus a missed, occult primary tumour may not become evident for many years. The diagnosis of PHG is therefore dependent on a careful search for a primary and long-term biochemical follow-up following curative hepatic resection. We report a case of a 7 cm PHG in a 48 year old man with ZES. Preoperatively, both a basal and stimulated gastrin levels were elevated. Surgical exploration including intraoperative ultrasound and duodenotomy, failed to reveal a primary. Patient underwent a right hepatectomy. Yearly, gastrin and secretin stimulation tests remain normal 6 years following surgery. He remains symptom free off all medication. An additional 26 cases of PHG were found. Including this case, 21 had at least 1 year follow-up, however only eight had greater than 5 years (median 24 months). Post-op gastrin levels were reported in 25, however provocative testing was done in only 10. Persistence and recurrence occurred in one and four, respectively. PHG causing ZES is extremely rare. Although the current literature claims to include 26 additional cases of PHG, without a thorough search for the primary and long-term follow-up data including provocative testing, this diagnosis remains a challenge.

Outcome of Lymph Node Involvement in Patients with the Zollinger-Ellison Syndrome

Annals of Surgery, 1988

Prognosis of gastrinoma patients with metastases to lymph nodes only is uncertain, and the true nature of isolated nodal gastrinomas remains controversial. The purpose of this study was to determine the outcome of such patients and whether nodal gastrinomas may occur as primary lesions. Eleven patients with nodal involvement but without hepatic metastases are reported (mean follow-up of 129 months). Primary gastrinomas were located in the duodenum in seven (Group 1) and not identified in four (Group 2). In Group 1, five patients remained eugastrinemic after excision of all gross tumors and gastrectomy (n = 4) or pancreaticoduodenectomy (n = 1), one patient had residual disease and died of other causes (survival of 88 months), and one patient had MEA-I syndrome with multiple gastrinomas (follow-up of 126 months). In Group 2, three patients became eugastrinemic after nodal excision and total gastrectomy (mean follow-up of 212 months) and may represent primary nodal gastrinomas, and in one patient, liver metastases developed and the patient died. Four deaths occurred in a 27-year period, but only one was tumor-related. There was no significant difference in 20-year survival rates between the two groups (85% vs. 75%). It is concluded that 1) lymph node gastrinomas are usually metastatic from primary duodenal lesions, 2) although rare, nodal gastrinomas may occur as primary lesions, and 3) in the absence of hepatic metastases, lymph node gastrinomas, whether primary or metastatic, have a good prognosis and should not deter aggressive surgical treatment. T n HE MANAGEMENT OF patients with the Zollinger-Ellison syndrome has been significantly altered by the advent of the histamine H2 receptor antagonists.' The realization that these agents can successfully control gastric acid hypersecretion has made operative intervention less urgent, and in many