Mixed mucinous adenocarcinoma and somatostatinoma of the ampulla of Vater associated with neurofibromatosis type 1

Pathology ◽  
2017 ◽  
Vol 49 (5) ◽  
pp. 553-555
Author(s):  
Róisín Reynolds ◽  
Veli Marjoniemi
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Mucinous Adenocarcinoma ◽  
Neurofibromatosis Type ◽  
Ampulla Of Vater

Mediastinal Atypical Carcinoid and Neurofibromatosis Type 1

2000 ◽  
Vol 124 (2) ◽  
pp. 319-321
Author(s):  
Paul Mathew ◽  
Jeffrey A. Roberts ◽  
Joseph Zwischenberger ◽  
Abida K. Haque
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Mediastinal Mass ◽  
Neurofibromatosis Type ◽  
Ampulla Of Vater ◽  
Carcinoid Tumors ◽  
Shortness Of Breath ◽  
Atypical Carcinoid ◽  
Debulking Surgery ◽  
Thoracoscopic Biopsy

Abstract We report a case of mediastinal atypical carcinoid in a 63-year-old woman with neurofibromatosis type 1 (NF-1), who presented with shortness of breath and a bulky mediastinal mass. Initial consideration was given to a neurogenic tumor. However, on thoracoscopic biopsy, the histologic appearance was consistent with an atypical carcinoid. Carcinoid tumors have been reported in association with NF-1 previously, but commonly in such unusual sites as the ampulla of Vater and duodenum and not in the thorax. The bulky, extensive, and highly vascular nature of the lesion precluded resection or debulking surgery. To our knowledge, there are no previous reports of atypical carcinoid of the lung or mediastinum in a patient with neurofibromatosis. This case report, therefore extends the spectrum of solid neoplasia in general and carcinoid tumors in specific, as they occur in association with NF-1.

scholarly journals Duodenal somatostatinoma presenting as obstructive jaundice with the coexistence of a gastrointestinal stromal tumour in neurofibromatosis type 1: a case with review of the literature

2019 ◽  
Vol 12 (1) ◽  
pp. bcr-2018-226702 ◽  
Author(s):  
Subhanudh Thavaraputta ◽  
Suzanne Graham ◽  
Ana M Rivas Mejia ◽  
Joaquin Lado-Abeal
Keyword(s):  
Obstructive Jaundice ◽  
Neurofibromatosis Type 1 ◽  
Gastrointestinal Stromal Tumour ◽  
Neurofibromatosis Type ◽  
Ampulla Of Vater ◽  
Duodenal Wall ◽  
Von Hippel Lindau ◽  
Tract Dilation ◽  
Von Hippel Lindau Syndrome

Somatostatinomas are rare neuroendocrine tumours, mostly located in the pancreas or duodenum, with an estimated incidence of 1 in 40 million. Duodenal somatostatinomas (DSs) are usually found in association with neurofibromatosis type 1 (NF1), tuberous sclerosis and Von Hippel-Lindau syndrome. Gastrointestinal stromal tumours (GIST) have also been described in NF1, but the association with somatostatinoma is very uncommon. We report the case of a patient with NF1 who presented with obstructive jaundice due to multiple firm nodules around the ampulla of Vater. A pancreaticoduodenectomy was performed and revealed a 1 cm duodenal/ampullary mass which stained positive for somatostatin, together with a GIST also found on the duodenal wall. Despite its rarity, ampullary somatostatinomas should be considered in the differential diagnosis of biliary tract dilation in patients with NF1.

Social information processing of children with Neurofibromatosis Type 1

2008 ◽  
Author(s):  
Jonathan M. Kurss ◽  
Anna E. Craig ◽  
Jennifer Reiter-Purtill ◽  
Kathryn Vannatta ◽  
Cynthia Gerhardt
Keyword(s):  
Information Processing ◽  
Neurofibromatosis Type 1 ◽  
Social Information Processing ◽  
Social Information ◽  
Neurofibromatosis Type

Lovastatin improves impaired LTP-like plasticity in patients with Neurofibromatosis Type 1

2011 ◽  
Vol 42 (01) ◽  
Author(s):  
F. Mainberger ◽  
N. Jung ◽  
M. Zenker ◽  
I. Delvendahl ◽  
U. Wahlländer ◽  
...  
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type

Intima-Type Vasculopathy in Neurofibromatosis Type 1

2014 ◽  
Vol 45 (S 01) ◽  
Author(s):  
J. Keppler ◽  
A. Fiedler
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type
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