The Coexistence of Somatostatinoma and Gastrointestinal Stromal Tumor in the Duodenum of a Patient with Von Recklinghausen's Disease

Yamamoto, Ryōichi; Kato, Shingo; Maru, Toshinori; Ninomiya, Riki; Ozawa, Fumiaki; Beck, Yoshifumi; Abe, Keiko; Tamaru, Jun‐ichi; Nagoshi, Sumiko; Yakabi, Koji

doi:10.2169/internalmedicine.55.5761

Cited by 13 publications

(13 citation statements)

References 16 publications

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“…Malignant carcinoid of ampulla Vateri and bilateral feochromocitoma have been also described with low responses to imatinib (105). Few papers reported extremely rare combination with pancreatic neuroendocrine neoplasia (64) and somatostatinoma in NF 1 (35) and Brunner's gland cysts (78).…”

Section: Risk Factorsmentioning

confidence: 99%

Surgical treatment of gastrointestinal stromal tumors of the duodenum: a literature review

Popivanov¹,

Tabakov²,

Mantese³

et al. 2018

Transl. Gastroenterol. Hepatol

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Background: Gastrointestinal stromal tumors (GIST) are the most frequent mesenchymal tumours in the digestive tract. The duodenal GIST (dGIST) is the rarest subtype, representing only 4-5% of all GIST, but up to 21% of the resected ones. The diagnostic and therapeutic management of dGIST may be difficult due to the rarity of this tumor, its anatomical location, and the clinical behavior that often mimic a variety of conditions; moreover, there is lack of consent for their treatment. This study has evaluated the scientific literature to provide consensus on the diagnosis of dGIST and to outline possible options for surgical treatment.Methods: An extensive research has been carried out on the electronic databases MEDLINE, Scopus, EMBASE and Cochrane to identify all clinical trials that report an event or case series of dGIST.Results: Eighty-six studies that met the inclusion criteria were identified with five hundred forty-nine patients with dGIST: twenty-seven patients were treated with pancreatoduodenectomy and ninety-six with only local resection (segmental/wedge resections); in four hundred twenty-six patients it is not possible identify the type of treatment performed (pancreatoduodenectomy or segmental/wedge resections).Conclusions: dGISTs are a very rare subset of GISTs. They may be asymptomatic or may involve symptoms of upper GI bleeding and abdominal pain at presentation. Because of the misleading clinical presentation the differential diagnosis may be difficult. Tumours smaller than 2 cm have a low biological aggressiveness and can be followed annually by endoscopic ultrasound. The biggest ones should undergo radical surgical resection (R0). In dGIST there is no uniformly adopted surgical strategy because of the low incidence, lack of experience, and the complex anatomy of the duodenum. Therefore, individually tailored surgical approach is recommended. R0 resection with 1-2 cm clear margin is required. Lymph node dissection is not recommended due to the low incidence of lymphatic metastases. Tumor rupture should be avoided.

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Section: Risk Factorsmentioning

confidence: 99%

Surgical treatment of gastrointestinal stromal tumors of the duodenum: a literature review

Popivanov¹,

Tabakov²,

Mantese³

et al. 2018

Transl. Gastroenterol. Hepatol

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“…The first case of duodenal somatostatinoma was reported in 1979. Duodenal somatostatin-producing endocrine tumors, which belong to the group of gastrointestinal, endocrine tumors, are rare neoplasms that have a prevalence of 1 in 40 million [6,7]. Duodenal tumors tend to be smaller averaging between 13 and 75 mm and 18 mm as a medium size, compared to pancreatic tumors with a median size of 42.5 mm.…”

Section: Discussionmentioning

confidence: 99%

A Case Report of Duodenal Psammomatous Somatostatinoma

Foad¹,

Mirghani²

2018

SJMS

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Somatostatinoma is a neuroendocrine tumor; its incidence in the duodenum around the ampulla of Vater is rare, and it is often not associated with secretory manifestation. We report a case of a female with an ampullary (Vater) tumor displaying neuroendocrine nuclear features, psammoma bodies, and the positivity for immunohistochemical panel of neuroendocrine tumors. Our patient presented with some features compatible with somatostatin secretion associated syndrome; albeit with normal serum levels of the hormone. Initial attempt of complete resection failed, and the involved margins were revised with a subsequent surgery, and the patient showed an uneventful course on follow-up for 2 years.

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“…Most of extrapancreatic tumors originate in the upper gastrointestinal tract and primarily occur in the duodenum or the peri-ampullary region [8]. These tumors are often detected incidentally, with an average overall post-operative 5-year survival rate of 75% [15]. Patients with metastases on diagnosis have a 5-year survival rate ranging from 40% to 60% [15].…”

Section: Discussionmentioning

confidence: 99%

“…These tumors are often detected incidentally, with an average overall post-operative 5-year survival rate of 75% [15]. Patients with metastases on diagnosis have a 5-year survival rate ranging from 40% to 60% [15]. Patients without metastases have an approximately 100% 5-year survival rate when their tumors are resected [15].…”

Section: Discussionmentioning

confidence: 99%

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Synchronic Duodenal Gastrointestinal Stromal Tumor and Neuroendocrine Tumor in Association with Neurofibromatosis Type 1: A Case Report

Doria¹,

Quadros²,

Lyra³

et al. 2018

JCT

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Introduction: The coexistence of synchronic duodenal gastrointestinal stromal tumor (GIST) and neuroendocrine tumor in a patient with neurofibromatosis type 1 (NF1) is extremely rare, and only eight cases were described in the literature. Clinical Case: This is a rare case of a 38-year-old female patient with NF1 who developed synchronic GIST and neuroendocrine tumor, which were both in the second portion of the duodenum. The first symptoms were abrupt digestive bleeding and anemia. Upper digestive endoscopy revealed two tumors, sizes 2.5 and 3.0 cm, in the second portion of duodenum, with biopsies identifying a GIST and a neuroendocrine tumor. Therapeutic decision was to proceed to surgical resection, and Whipple's procedure was indicated. Surgical procedure was performed with good outcome. Currently the patient has excellent quality of life and maintains follow up for thirty months without recurrence. Discussion: Long-term disease-free survival and excellent quality of life are reported when these tumors are fully resected in this context. However, it is not always easy to access the gastrointestinal tract, especially the small intestine, and proceed to the histopathologic diagnosis of these tumors. Conclusion: It is important to be aware of the possibility of the coexistence of various tumors in the NF1 scenario for adequate screening, staging, and surgical treatment of these patients, as good prognosis can be achieved when such tumors are detected and treated properly.

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The Coexistence of Somatostatinoma and Gastrointestinal Stromal Tumor in the Duodenum of a Patient with Von Recklinghausen's Disease

Cited by 13 publications

References 16 publications

Surgical treatment of gastrointestinal stromal tumors of the duodenum: a literature review

Surgical treatment of gastrointestinal stromal tumors of the duodenum: a literature review

A Case Report of Duodenal Psammomatous Somatostatinoma

Synchronic Duodenal Gastrointestinal Stromal Tumor and Neuroendocrine Tumor in Association with Neurofibromatosis Type 1: A Case Report

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