Large Duodenal Somatostatinoma in the Third Portion Associated with Severe Glucose Intolerance

Kimura, Ryosuke; Hayashi, Yoshinori; Takeuchi, Toyo; Shimizu, Manabu; Hiratsuka, Maki; Yoshida, Michihiro; Hamajima, Eiji; Iwata, Masaru; Imoto, Masami; Hattori, Hideo

doi:10.2169/internalmedicine.43.704

Cited by 11 publications

(8 citation statements)

References 11 publications

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“…Although none of our AST cases showed unequivocal signs of somatostatin-related endocrine hyperfunction relieved by tumor excision, these findings (particularly diabetic ketoacidosis) have been reported by others [26,27,28]. Another point of potential clinical interest is AST's frequent lack of type 2A somatostatin receptors, coupled with the intense expression of type 5 receptors, which may have implications for the choice of appropriate somatostatin analogue therapy [29,30].…”

Section: Discussionsupporting

confidence: 61%

Four Neuroendocrine Tumor Types and Neuroendocrine Carcinoma of the Duodenum: Analysis of 203 Cases

Rosa²,

et al. 2016

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Background: Several types of neuroendocrine neoplasms (NENs) have been described in the duodenal tract, from low-grade tumors (NETs) to high-grade neuroendocrine carcinomas (NECs). A comprehensive analysis of histology, hormonal profile and prognostic parameters of a sufficiently large duodenal NEN series to cover all main kinds of neoplasms is however lacking. Methods: We collected a retrospective series of 203 duodenal wall and ampullary region NENs, from six specialized endocrine pathology centers. All were characterized histopathologically and histochemically, and 190 were followed for a median of 9 years. Results: Twenty-seven poorly differentiated NECs, mostly from the ampullary region, were identified and shown to lead to patient demise in a median of 10 months. Among 176 NETs, four subtypes were characterized, including 20 gastrinomas, 37 ampullary-type somatostatin-producing NETs (ASTs), 12 gangliocytic paragangliomas (GPs) and 106 nonfunctioning NETs (nfNETs). ASTs and GPs were mostly localized in the ampullary/periampullary region, while gastrinomas and nfNETs were mainly from the proximal duodenum. ASTs and gastrinomas showed high rates of local infiltration (especially lymphoinvasion and deep duodenal wall/pancreatic tissue invasion) and lymph node metastasis, while nfNETs had significantly lower and more size-dependent local invasive potential. Disease-specific survival differed significantly between NETs and NECs, though not among NET subtypes. NET cases with distant metastases (n = 23) were significantly associated with larger size, higher proliferative grade, lymphovascular invasion, deep invasion and local lymph node metastasis. Conclusion: Our careful analysis of a large series of duodenal NENs identified five histologically and prognostically different histotypes of potential clinical relevance.

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Section: Discussionsupporting

confidence: 61%

Four Neuroendocrine Tumor Types and Neuroendocrine Carcinoma of the Duodenum: Analysis of 203 Cases

Rosa²,

et al. 2016

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“…The estimated annual incidence is 1 in 40 million [14] with a median age at onset of 54 years (range 24-84 years). No racial predilection Deschamps et al [27] Colovic et al [28] Yu et al [29] He et al [30] Cao et al [31] Chetty et al [32] South et al [33] Takai et al [34] Nesi et al [35] Sakorafas et al [36] Biffoni et al [37] Sreenarasimhaiah et al [38] Peskova et al [39] Martinez-Penalver Gutierrez et al [40] Bettini et al [41] Choi et al [42] Guercioni et al [43] Morisawa et al [44] Marakis et al [45] Kim et al [46] Hendi et al [47] Bastian et al [48] Fendrich et al [49] Kimura et al [50] Suzuki et al [51] Cappelli et al [52] Ohwada et al [53] Tomono et al [54] Patel et al [55] Takashi et al [56] Wakeman et al [57] Usui et al [6] House et al [58] Gregersen et al [59] Green et al [60] Guo et al [61] Rios et al [19] Borobia et al [62] Karasawa et al [4] Cohen et al [63] Tanaka et al [64] Table 3 Reports of Somatostatinoma published during the last decade mal ...…”

Section: Discussionmentioning

confidence: 99%

Duodenal somatostatinoma

et al. 2010

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Aim -Background: Somatostatinomas is a very rare neuroendocrine tumour of the gastrointestinal tract, first described in the pancreas in 1977 and in the duodenum in 1979. We present the case a 67-year-old woman with duodenal somatostatinoma that was resected with a pancreaduodenectomy procedure and also provide a brief review of the literature. Method: Databases from Pubmed and Medline were searched using a list of keywords such as somatostatinoma, duodenum, pancreas, neuroendocrine tumour and references from review articles.The study of such a case and those in the literature provides useful knowledge into the clinical management and diagnosis of these patients. Results: The search of the literature yielded 348 articles, 92 reviews, 175 case reports, 328 journal articles, 6 clinical trials, 1 editorial, 21 comparative studies and 3 multicenter studies. Forty-five articles related to type-1 neurofibromatosis were found, 5 associated with von Hippel-Lindau disease and 33 associated with multiple endocrine neoplasia type-1. Around 201 cases of somatostatinoma were reported Conclusion: Two different symptomatic presentations can be found that include the inhibitory syndrome in pancreatic somatostatinomas, whereas tumours arising from the duodenal wall are usually associated with mechanical enteric obstructive symptoms. The clinical symptoms are often variable and non-specific; many somatostatinomas are found incidentally during cholecystectomy or during gastrointestinal imaging studies. Surgery is the standard treatment and it is also preferred in metastatic disease providing diagnosis is made early. Patients with metastatic somatostatinoma display 5-year postoperative survival rates of 30-60% as opposed to those without metastasis whose 5-year survival rate approaches 100%.

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“…Diabetes mellitus, non‐responsive to antiglycemic therapy, was present in both the pancreatic and duodenal cases, as a consequence of the inhibitory effect of somatostatin on insulin secretion 23 . On the other hand, hypoglycemia in the patient with the jejunal tumor was secondary to inhibition of growth hormone and impairment of carbohydrate absorption 17 …”

Section: Discussionmentioning

confidence: 99%

Somatostatinoma: Clinico‐pathological features of three cases and literature reviewed

Nesi¹,

Marcucci²,

Rubio

et al. 2008

J of Gastro and Hepatol

103

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Somatostatinoma is a rare endocrine tumor that comprises around 1% of all gastroenteropancreatic endocrine neoplasms. This paper gives an updated review on somatostatinoma and describes three sporadic cases of somatostatinoma located in the pancreas, duodenum, and jejunum. Approximately 200 case histories of somatostatinoma have been published, with the duodenum being the most frequent site, followed by the pancreas. Somatostatinomas may be sporadic or associated with neurofibromatosis type 1, Multiple Endocrine Neoplasia type 1, and Von Hippel-Lindau syndromes. Functional somatostatinomas release excessive amounts of somatostatin suppressing gallbladder motility and inhibiting the secretory activity of various endocrine and exocrine cell types. A triad of mild diabetes mellitus, cholelithiasis, and diarrhea/steatorrhoea characterizes the somatostatinoma or 'inhibitory' syndrome. Non-functional somatostatinomas tend either to be asymptomatic or to present with obstructive symptoms. These tumors are often malignant and by the time they are detected, nearly two-thirds have already metastasized to the regional lymph nodes or the liver. A comparison between our three cases and those in the literature provides useful insights into the clinical management of these patients. Interestingly, the jejunal somatostatinoma described here is the second case ever reported.

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Large Duodenal Somatostatinoma in the Third Portion Associated with Severe Glucose Intolerance

Cited by 11 publications

References 11 publications

Four Neuroendocrine Tumor Types and Neuroendocrine Carcinoma of the Duodenum: Analysis of 203 Cases

Four Neuroendocrine Tumor Types and Neuroendocrine Carcinoma of the Duodenum: Analysis of 203 Cases

Duodenal somatostatinoma

Somatostatinoma: Clinico‐pathological features of three cases and literature reviewed

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