glucagonoma pathology

CONTEXT: Pancreatic α-cell hyperplasia (ACH) was once an esoteric pathological entity, but it has become an important differential diagnosis of hyperglucagonemia after inactivating glucagon receptor (GCGR) genomic mutations were found in patients with ACH. Recently, the controversy over the pancreatic effects of incretins has stimulated much discussion of ACH that often includes inaccurate statements not supported by the literature. DATA ACQUISITION: Literature related to ACH was reviewed...

CONTEXT: Glucagonomas of the pancreas are neuroendocrine tumours (NETs) that arise from well-differentiated neuroendocrine cells within the pancreatic islets. They are considered to be aggressive NETs and often have metastases at initial presentation. In contrast localised glucagonoma without metastatic spread may have prolonged disease free survival with radical resectional surgery. CASE REPORT: The authors present a case of a glucagonoma that initially presented with classical necrolytic migratory erythema and a large solitary mass in the body and tail of the pancreas that was surgically resected...

glucagonoma is a rare islet alpha-cell tumor. We report a case of glucagonoma in a 55-year-old male patient with such clinical findings of necrolytic migratory erythema, diabetes mellitus, body weight loss, and anemia. CT examination found a space-occupying lesion in the pancreas, and an elevated serum glucagon level indicate the diagnosis of glucagonoma, which was confirmed postoperatively by pathological examination of the tumor tissue. A definite diagnosis of glucagonoma relies on pathological report, and so far no standard treatment strategy has been available for this tumor...

BACKGROUND: The pancreatic neuroendocrine tumor (pNET) is relatively rare and generally felt to follow an indolent course. EUS has an important role in detection of pNET. This is a review of clinical and radiological presentation and pathologic reports of 22 patients with pNET. PATIENTS AND METHODS: In this study we analyzed clinical and radiological presentations and pathologic reports of all relevant cases who were referred to Taleghani hospital for 3 years since 2008...

AIMS: We investigated the prevalence and characterization of asymptomatic pancreatic tumors in response to our experience of asymptomatic insulinoma. METHODS: A patient with a moderately low glucose level and pancreatic incidentaloma detected by CT was examined. Pancreas specimens from 423 autopsy cases were also pathologically examined systematically by hematoxylin-eosin staining. RESULTS: The examined patient showed no profile characteristic of insulinoma by fasting or loading tests, however, ASVS led to diagnosis of insulin-producing tumor...

The differential diagnosis of hypervascular pancreatic lesions is complex, and includes endocrine and exocrine tumors of the pancreas, metastases to the pancreas, and masses, or mass-like lesions, arising from the neurovascular networks traversing the pancreas. In this manuscript, we will discuss salient imaging findings of these masses, pertinent differential diagnoses, as well as review clinical symptomatology that may aid in the diagnosis of some of these lesions.

Inhibition of glucagon signaling causes hyperglucagonemia and pancreatic α cell hyperplasia in mice. We have recently demonstrated that a patient with an inactivating glucagon receptor mutation (P86S) also exhibits hyperglucagonemia and pancreatic α cell hyperplasia but further develops pancreatic neuroendocrine tumors (PNETs). To test the hypothesis that defective glucagon signaling causes PNETs, we studied the pancreata of mice deficient in glucagon receptor (Gcgr(-/-)) from 2 to 12 months, using WT and heterozygous mice as controls...

BACKGROUND: Surgery for pancreatic endocrine tumors (PETs) with blood vessel involvement is controversial. HYPOTHESIS: Resection of PETs with major blood vessel involvement can be beneficial. DESIGN: The combined databases of the National Institutes of Health and Stanford University hospitals were queried. MAIN OUTCOME MEASURES: Operation, pathologic condition, complications, and disease-free and overall survival. RESULTS: Of 273 patients with PETs, 46 (17%) had preoperative computed tomography evidence of major vascular involvement...

Pathologic hyperplasia of various pancreatic endocrine cells is rare but has been long known. β cell hyperplasia contributes to persistent hyperinsulinemic hypoglycemia of infancy, which is commonly caused by mutations in the islet ATP-sensitive potassium channel, and to non-insulinoma pancreatogenous hypoglycemia in adults, which may or may not be associated with bariatric surgery. α cell hyperplasia may cause glucagonoma syndrome or induce pancreatic neuroendocrine tumors. An inactivating mutation of the glucagon receptor causes α cell hyperplasia and asymptomatic hyperglucagonemia...

Pancreatic endocrine tumors (PETs) are primarily well-differentiated tumors composed of cells that resemble normal islet cells but that arise from pancreatic ductal cells. They are classified as functioning or nonfunctioning according to their associated clinical symptoms; insulinoma, gastrinoma, and glucagonoma are the most common functioning PETs. They also are classified according to their biologic behavior, although all PETs have malignant potential. Most are sporadic, but some are associated with familial syndromes such as multiple endocrine neoplasia type 1, von Hippel-Lindau syndrome, and neurofibromatosis type 1...

INTRODUCTION: This case report demonstrates that the differential immunohistochemical reactivities of Siemens' Double Antibody Glucagon compared to DakoCytomation's Polyclonal Rabbit Anti-Human Glucagon allow for pathologic distinction of enteral versus pancreatic glucagonoma. CASE PRESENTATION: A 64-year-old Caucasian man was diagnosed with a duodenal enteroglucagonoma following presentation with obstructive jaundice. He had a low serum glucagon level using Siemens' Double Antibody Glucagon, a clinical syndrome consistent with glucagon hypersecretion...

A 38-year-old man was admitted because of exacerbation of erythema. Full-body computed tomography (CT) scanning revealed a tumor mass in the tail of the pancreas; CT and magnetic resonance imaging (MRI) scans confirmed the presence of a spherical mass. In contrast CT scans, although the contrast was gradually increased, no strong contrast differences were observed between the tumor and the surrounding tissue. Endoscopic ultrasonography (EUS) revealed a spherical low-density mass. Blood test results revealed that the patient had a high glucagon level...

INTRODUCTION: Somatostatin receptors are expressed in a large number of human tumours. The somatostatin receptors types 1-5 expression in a series including 100 gastro-entero-pancreatic endocrine tumours were analysed. METHODS: From a prospectively built database of patients with gastro-entero-pancreatic endocrine tumours referred from three institutions, 100 cases with clinical and pathological data were selected. Somatostatin receptors expression by immunohistochemistry with somatostatin receptor types 1-5 antibodies in tissue paraffin sections were studied and correlated with the histological diagnosis according to the WHO classification, location and functional status...

OBJECTIVE: To study the diagnosis and treatment of glucagonoma. METHODS: A retrospective review of glucagonoma cases was committed between June 1993 and July 2008 in Peking Union Medical College Hospital. It was measured by sex, age, misdiagnosis, clinical symptoms, laboratory data, imaging studies, diagnosis, treatment procedures and so on. RESULTS: The tumors of eleven cases were found in the tail, and one case was in the head of the pancreas at the same time...

BACKGROUND: Cystic pancreatic endocrine neoplasms (CPENs) are considered rare, and their behavior is thought to be similar to that of solid pancreatic endocrine neoplasms (PENs). This study aims to describe the characteristics of CPENs in a large patient cohort. STUDY DESIGN: We performed a retrospective review of 170 patients who underwent resections for PENs at Massachusetts General Hospital from 1977 to 2006. Twenty-nine patients (51% men, mean age 53) with CPENs were compared with 141 patients with solid PENs...

OBJECTIVE: To review experience in preoperative detection of islet cell tumors using multislice computed tomography (MSCT) and summarize various imaging features of functioning islet cell tumors on enhanced MSCT. METHODS: Seventy patients with clinical or pathological diagnosis of functioning pancreatic islet cell tumor between October 2003 and February 2007 were included in this retrospective study. Seventy-four enhanced MSCT scans in these patients were identified...

We report a rare case of nesidioblastosis and hyperplasia of alpha cells, microglucagonoma, and nonfunctioning islet cell tumor of the pancreas. The patient's clinical presentation, diagnosis, treatment, pancreas pathology, and follow-up are reviewed. A 60-year-old patient was incidentally found to harbor a pancreatic mass with markedly elevated glucagon levels but without glucagonoma syndrome. She was initially diagnosed with glucagonoma, and the tumor was resected. Pathological examination demonstrated that the tumor was a nonfunctioning islet cell tumor and revealed nesidioblastosis and hyperplasia of alpha cells and microglucagonoma in the apparently normal surgical margin...

Since the first reports with laparoscopic resection of islet cell tumors in 1996, the experience worldwide is still limited, with only short-term outcomes available. Some have suggested that a malignant tumor is a contraindication to laparoscopic resection. Aim The aim of this study was to evaluate the feasibility, safety, and long-term outcome of the laparoscopic approach in patients with functioning, nonfunctioning, or overt malignant pancreatic neuroendocrine tumor (PNT). To our knowledge this is the largest single-institution series on this subject to date...

Various metabolic events may lead to dermatologic pathology. Three illustrative examples are glucagonoma syndrome, uremic pruritus, and zinc deficiency. The glucagonoma syndrome, resulting from a glucagon secreting-tumor, is characterized by a distinctive dermatitis, necrolytic migratory erythema. This skin rash is a generalized, pruritic eruption which first appears as erythematous patches, then progresses to form superficial vesicles and bullae. Uremic pruritus is a clinical phenomenon seen in patients with chronic renal failure; it provokes vigorous scratching and may lead to numerous cutaneous lesions including extensive excoriations, lichen simplex chronicus, prurigo nodularis, keratotic papules, or secondary impetigo...

BACKGROUND/AIMS: Pancreatic neuroendocrine tumors (PNET) are rare and manifest as functioning tumor (FT) or non-functioning tumor (NFT). Although malignant changes are observed in some cases, its prognosis is better than pancreatic cancer. We evaluated clinicoradiologic features and prognosis of FT and NFT. In addition, we tried to find the predictive factors for the recurrence of NFT after resection. METHODS: Between October 1994 and June 2004, we retrospectively evaluated the clinicopathologic features and prognosis of 12 cases of FT and 31 cases of NFT diagnosed by surgical pathology at single medical center in Korea...