Malignant Gastrointestinal Stromal Tumor in a Patient with Neurofibromatosis type 1
The Korean Journal of Internal Medicine
;
: 21-23, 2007.
Article
in English
| WPRIM
| ID: wpr-199150
ABSTRACT
Neurofibromatosis type 1 (von Recklinghausen's disease, NF-1) is an autosomal-dominant neurocutaneous disorder characterized by abnormal skin pigmentation (cafe au lait spots and axillary freckling), cutaneous and plexiform neurofibromas, skeletal dysplasias, and Lisch nodules (pigmented iris hamartomas). Gastrointestinal stromal tumors (GISTs) are the most common tumors of mesenchymal origin in the gastrointestinal tract, mesentery, omentum, and retroperitoneum. Here, we report a case of GIST in the ileum of a 76-year-old woman previously diagnosed as NF-1. She was admitted due to sudden onset of abdominal pain. Contrast enhanced CT scan revealed a moderately defined, peripherally enhanced soft tissue mass of about 8.8 x 7.3 cm, originating from the small bowel in the left of the abdomen. Surgical excision was performed and the tumor was found to be composed of tumor cells that were positive for c-kit protein. The patient started imatinib treatment a month later, but stopped medication due to dyspepsia after a few months and eventually progressed after 18 months.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Abdominal Pain
/
Risk Factors
/
Neurofibromatosis 1
/
Gastrointestinal Stromal Tumors
Type of study:
Etiology study
/
Risk factors
Limits:
Aged
/
Female
/
Humans
Language:
English
Journal:
The Korean Journal of Internal Medicine
Year:
2007
Type:
Article
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