Multiple gastrointestinal stromal tumors in neurofibromatosis type 1: report of a case.
This report presents a case of multiple gastrointestinal stromal tumors (GIST) with neurofibromatosis type 1 (NF1). A 68-year-old woman was admitted to the hospital because of a tumor close to the head of the pancreas. Imaging studies revealed submucosal tumors of the duodenum. The retroperitoneal tumor was diagnosed before surgery. Besides the main tumor in the duodenum, multiple small submucosal tumors were found in the duodenum and upper part of the jejunum during the operation. All of these tumors were resected. The histological diagnosis of all these tumors was GISTs. These tumors were immunohistochemically positive for KIT, but they demonstrated no mutation in c-kit exons 9, 11, 13, and 17, and platelet-derived growth factor receptor alpha exons 12 and 18. No recurrence occurred for a year after surgery.
Hirashima
Kotaro
K
Department of Gastroenterological Surgery, Kumamoto University, Kumamoto 860-8556, Japan.
Takamori
Hiroshi
H
Hirota
Masahiko
M
Tanaka
Hiroshi
H
Ichihara
Atsushi
A
Sakamoto
Yasuo
Y
Ikuta
Yoshiaki
Y
Karashima
Ryu-ichi
R
Watanabe
Masayuki
M
Iyama
Ken-ichi
K
Baba
Hideo
H
D000368:Aged; D003937:Diagnosis, Differential; D013505:Digestive System Surgical Procedures / Q000379:methods*; D004386:Duodenum / Q000473:pathology*; D005260:Female; D046152:Gastrointestinal Stromal Tumors / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D006801:Humans; D007583:Jejunum / Q000473:pathology*; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000175:diagnosis