Use of 2-tracer PET to diagnose gastrointestinal stromal tumour and pheochromocytoma in patients with Carney triad and neurofibromatosis type 1

2006 ◽  
Vol 41 (5) ◽  
pp. 626-630 ◽  
Author(s):  
Per Bümming ◽  
Bengt Nilsson ◽  
Jens Sörensen ◽  
Ola Nilsson ◽  
Håkan Ahlman
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Gastrointestinal Stromal Tumour ◽  
Neurofibromatosis Type ◽  
Stromal Tumour ◽  
Carney Triad

Neurofibromatosis type-1 with retroperitoneal stromal tumour: one case report

2013 ◽  
Vol 58 (1) ◽  
pp. e37-e40
Author(s):  
G-q Zong ◽  
Y Fei ◽  
F Wang ◽  
R-m Liu
Keyword(s):  
Case Report ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Stromal Tumour

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.

Pheochromocytoma and gastrointestinal stromal tumours in an adult neurofibromatosis type 1 patient: a rare co-occurrence

2020 ◽  
Vol 13 (6) ◽  
pp. e235129
Author(s):  
Nuttawut Vongsumran ◽  
Sarawut Kongkarnka ◽  
Pittaporn Watanawittawas ◽  
Worapaka Manosroi
Keyword(s):  
Small Bowel ◽  
Neurofibromatosis Type 1 ◽  
Bowel Resection ◽  
Gastrointestinal Symptoms ◽  
Gastrointestinal Stromal Tumour ◽  
Neurofibromatosis Type ◽  
Small Bowel Resection ◽  
Gastrointestinal Stromal Tumours ◽  
Rare Association

The risk of tumours including pheochromocytoma and gastrointestinal stromal tumour (GIST) has been reported to be higher in neurofibromatosis type 1 (NF1) patients. The concomitant occurrence of these two tumours was rare in NF1 patient and most were symptomatic. In this case report, we describe the case of a 47-year-old man with NF1 who presented with microscopic haematuria. Neither hypertension nor any gastrointestinal symptoms were reported by the patient. While investigating for haematuria, left adrenal mass and arterial enhancing lesions in the small bowel were incidentally documented during computerised urography. The patient subsequently underwent a left adrenalectomy and small bowel resection. The pheochromocytoma and multiple GIST tumours were diagnosed based on pathology. Here, we discuss the rare association of pheochromocytoma and GIST and the asymptomatic presentation of those tumours in an NF1 patient. We further suggest that in NF1 patients a heightened level of vigilance can help identify this infrequent combination.

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
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