2000
DOI: 10.1053/ejso.1999.0911
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Ampullary carcinoid and jejunal stromal tumour associated with von Recklinghausen's disease presenting as gastrointestinal bleeding and jaundice

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Cited by 46 publications
(29 citation statements)
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“…29 Rarely, patients with NF-1 have had concurrent GEP-NETs. Specifically, cases reports have been published of NF-1 patients who had ampullary carcinoids, 30,31 duodenal somatostatinomas (for review, see Cappelli, et al 32 ), or somatostatinoma of the pancreas. 33 To date, no molecular analyses for potential NF1 gene involvement in GEP-NETs have been conducted.…”
Section: Nf-1/von Recklinghausen Diseasementioning
confidence: 99%
“…29 Rarely, patients with NF-1 have had concurrent GEP-NETs. Specifically, cases reports have been published of NF-1 patients who had ampullary carcinoids, 30,31 duodenal somatostatinomas (for review, see Cappelli, et al 32 ), or somatostatinoma of the pancreas. 33 To date, no molecular analyses for potential NF1 gene involvement in GEP-NETs have been conducted.…”
Section: Nf-1/von Recklinghausen Diseasementioning
confidence: 99%
“…The patient developed at the same time three tumours: one jejunal GIST and two endocrine neoplasms (one papilla major neoplasm and one papilla minor neoplasm), with strong immunohystochemical expression of somatostatin ("somatostatinoma"). This is the forth case described in the world literature of a VRD patient associated with this rare somatostatinoma [6][7][8] .…”
Section: Discussionmentioning
confidence: 99%
“…In 56% of patients the greatest diameter was 2 cm or less. The resection of choice was a pancreaticoduodenectomy (60%) while in the remaining cases a local resection was performed [6][7][8][9][10] . This latter procedure is considered as an appropriate treatment for small duodenal tumours (< 2 cm) far from the ampulla [6,7] .…”
Section: Discussionmentioning
confidence: 99%
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“…Only 0.2 to 0.3% of all gastrointestin REVIEWS n n 250 n 2009 n Volume 3, Number 2 nal carcinoids are localised to this region, but a quarter of these is associated with von Recklinghausen's disease [26,33,34]. Concomitant occurrence of NF1-GIST -neuroendocrine tumour has been reported in 11 cases, almost exclusively in the periampullary region [11,16,18,24,[35][36][37][38][39]. Characteristically these tumours show functional inactivity, and they have a relatively good prognosis [35].…”
Section: Associated Tumoursmentioning
confidence: 99%