Pancreatic somatostatinoma with obscure inhibitory syndrome and mixed pathological pattern

Zhang, Bo; Xie, Qiuping; Gao, Shunliang; Fu, Yanbiao; Wu, Yulian

doi:10.1631/jzus.b0900166

Cited by 10 publications

(3 citation statements)

References 16 publications

(18 reference statements)

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“…Tumors in most of the reported cases were smaller than 5 cm. More than 85% were symptomatic and more than two-thirds were malignant 7-9. The most common locations of this tumor were the duodenum and pancreas.…”

Section: Discussionmentioning

confidence: 99%

Gastric Somatostatinoma: An Extremely Rare Cause of Upper Gastrointestinal Bleeding

Prachayakul

Aswakul²,

Deesomsak³

et al. 2013

Clin Endosc

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A 49-year-old woman presented with chronic abdominal discomfort, significant weight loss, and chronic intermittent diarrhea. She suddenly developed massive upper gastrointestinal bleeding and was referred for further treatment. Endoscopy indicated a large mass in the upper gastric body with antral and duodenal bulb involvement. Endosonography showed a large well-defined isoechoic gastric subepithelial mass with multiple intra-abdominal and peripancreatic lymphadenopathy, suspected to be malignant on the basis of fine needle aspiration cytology. The tumor was surgically removed, and histopathology showed typical characteristics of a neuroendocrine tumor. On the basis of immunohistochemical staining, somatostatinoma, a rare neuroendocrine tumor, was diagnosed. Gastrointestinal bleeding is a rare presentation and the stomach is an uncommon tumor location.

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Section: Discussionmentioning

confidence: 99%

Gastric Somatostatinoma: An Extremely Rare Cause of Upper Gastrointestinal Bleeding

Prachayakul

Aswakul²,

Deesomsak³

et al. 2013

Clin Endosc

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“…In most cases, the histologic pattern does not determine the functional status of the tumors. 31 The accurate diagnosis of somatostatinoma depends on the intense positive immunohistochemical reaction for somatostatin 32 .…”

Section: Discussionmentioning

confidence: 99%

Somatostatinoma syndrome: a challenging differential diagnosis among pancreatic tumors

Vianna¹,

Ferreira²,

Campos³

2013

ACR

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Among the neuroendocrine neoplasia, the pancreatic somatostatin-producing tumors are very rare. Usually functional, these tumors produce the somatostatinoma syndrome, which encompasses diabetes mellitus, diarrhea/steatorrhoea, and cholelithiasis. Other symptoms may include dyspepsia, weight loss, anemia, and hypochlorhydria. All theses symptoms are explained by the inhibitory actions of the somatostatin released by tumoral cells originated from pancreatic delta cells or endocrine cells of the digestive tract. The diagnosis is easy to overlook since these symptoms are commonly observed in other more common syndromes. Besides the clinical features, diagnosis is based on serum determination of somatostatin, and imaging exams, such as ultrasound, computer tomography and positron emission tomography. Pathologic examination is characterized by the positivity of immunohistochemical reaction for synaptophysin, chromogranin, and somatostatin. These tumors can be classified according to tumor size, mitotic index, neural or vascular invasion, and distant metastases. The authors describe the case of a 61-year-old female patient who sought medical care because of a 6-month history of watery diarrhea, weight loss, and depression. She was diagnosed with diabetes mellitus 3 years ago. Imaging examination revealed a tumoral mass of 4 cm in its longest axis in the topography of the head of the pancreas and calculous cholecistopathy. The patient’s clinical status was unfavorable for a surgical approach. She died after 20 days of hospitalization. The definitive diagnosis was achieved with the autopsy findings, which disclosed a pancreatic somatostatinoma.

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“…Somatostatin is a cyclic tetra-decapeptide that derives from D cells of the pancreas, neuroendocrine cells of the duodenum, central nervous system and thyroid gland [2]. Somatostatinomas are mainly located at the head of the pancreas (70% of the reported cases), but they are also found in the small intestine, the duodenal papilla and the ampulla of Vater [3,4]. Somatostatinomas are usually presented with manifestations of the "inhibitor syndrome", caused by the suppressive effects of somatostatin [5].…”

Section: Introductionmentioning

confidence: 99%

Somatostatinoma of the Ampulla of Vater: A Systematic Review

Giannakodimos

Ziogou

et al. 2022

JGLD

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Background and Aims: Somatostatinoma of the ampulla of Vater (SAV) is a rare neuroendocrine tumor that usually appears with atypical clinical manifestations and is associated with Von Recklinghausen’s disease. The aims of this study were to systematically review the literature regarding SAV and to highlight the clinicopathological characteristics and optimal therapeutic management of this rare entity. Methods: A systematic search of the literature in PubMed/Medline and Scopus databases was performed by two independent investigators, including all case reports and case series concerning SAVs from 1980 until September 2021. Results: In total, 37 articles were retrieved, including 43 patients, with a male to female ratio of 1.8:1 and a mean age of 46.8 ± 11.3 years (mean, SD). For 23 out of 43 patients (53.5%), Von Recklinghausen’s disease was proved. The main clinical manifestations were abdominal pain (41.9%), jaundice (27.9%), weight loss (20.9%) and bowel disorders (20.9%). Typical histological findings included psammoma bodies, nests or clusters of epithelial cells with eosinophilic cytoplasm, while somatostatin staining was positive in 35 patients (81.4%), chromogranin-A in 21 patients (48.8%) and synaptophysin in 18 patients (41.9%). Surgery was the initial therapeutic approach in 34 patients (79.1%), whereas Whipple’s procedure was the preferred surgical approach in 23 patients (53.4%). The longest survival among included patients was 13 years and only two postoperative deaths (4.7%) were reported. Conclusions: Somatostatinomas of the ampulla of Vater are rare malignancies that require increased physicians’ suspicion and accurate surgical approach in order to achieve optimal therapeutic results.

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Pancreatic somatostatinoma with obscure inhibitory syndrome and mixed pathological pattern

Cited by 10 publications

References 16 publications

Gastric Somatostatinoma: An Extremely Rare Cause of Upper Gastrointestinal Bleeding

Gastric Somatostatinoma: An Extremely Rare Cause of Upper Gastrointestinal Bleeding

Somatostatinoma syndrome: a challenging differential diagnosis among pancreatic tumors

Somatostatinoma of the Ampulla of Vater: A Systematic Review

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