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Combined presence of multiple gastrointestinal stromal tumors along with duodenal submucosal somatostatinoma in a patient with neurofibromatosis type 1.

Authors :
Kumar T
Gupta B
Das P
Jain D
Jain HA
Madhusudhan KS
Dash NR
Gupta SD
Source :
Indian journal of pathology & microbiology [Indian J Pathol Microbiol] 2016 Jul-Sep; Vol. 59 (3), pp. 359-61.
Publication Year :
2016

Abstract

Neurofibromatosis type-1 (NF-1) is an autosomal dominant disorder, with increased risk of developing benign and malignant tumors of the gastrointestinal tract (GIT). However, the synchronous presence of multiple GIT stromal tumors and duodenal submucosal somatostatinoma, like in this 50-year-old female NF-1 patient, is very rare. She presented with hematemesis, malena, along with multiple neurofibromas all over the body. Thorough radiological and peroperative work-up revealed multiple ulcerated submucosal and serosal nodules in the proximal small intestine. Histological work-up revealed diagnosis of a duodenal submucosal somatostatinoma with multifocal serosal gastrointestinal stromal tumors. This case is being reported to highlight the rare coincidence of multiple GIT tumors in an NF-1 patient.

Details

Language :
English
ISSN :
0974-5130
Volume :
59
Issue :
3
Database :
MEDLINE
Journal :
Indian journal of pathology & microbiology
Publication Type :
Report
Accession number :
27510677
Full Text :
https://doi.org/10.4103/0377-4929.188123