Abstract
In May 2009, a 59-year-old woman with neurofibromatosis type 1 was operated for masses of the head and left shoulder growing to 10 cm in diameter over 6 months, and the pathological diagnosis was malignant peripheral nerve sheath tumors (MPNST). In April 2010, she developed anemia. Computed tomography (CT) and positron emission tomography (PET) showed a mass of in both the intestine and the left lung. A 1 cm diameter gastric mass was also shown, but the biopsy was negative and there was no bleeding. Suspecting bleeding from the intestinal mass, a partial resection of the small intestine was performed and the mass was diagnosed as MPNST. After 2 weeks from discharge, she developed anemia again, and gastric mass showed to be growing and bleeding. Partial gastrectomy was performed and after 4 weeks, a left lingular segmentectomy was performed. Both were diagnosed as MPNST. After 4 weeks, she developed pulmonary embolism and multiple masses in the intestine, peritoneum, subcutaneous tissue. She developed small-intestinal intussusceptions, obstructive ileus and bleeding, so a partial resection of the small intestine was performed and diagnosed as MPNST.
The next day she was discharged, she developed disturbance of consciousness and left-side partial paralysis and was diagnosed with multiple brain metastases, and has subsequently died.
MPNSTs are rare tumor entities that originate from peripheral nerve sheaths. Gastrointestinal MPNSTs have been reported in only 15 cases, including ours, in Japan.
