A case of retroperitoneal malignant peripheral nerve sheath tumor in a patient with neurofibromatosis
Miura, T.; Yamada, Y.; Muramaki, M.; Tanaka, K.; Hara, I.; Fujisawa, M.
Hinyokika Kiyo. Acta Urologica Japonica 52(3): 207-209
2006
ISSN/ISBN: 0018-1994 PMID: 16617875 Document Number: 596970
We report a case of retroperitoneal malignant peripheral nerve sheath tumor (MPNST) in a patient with neurofibromatosis 1. A 42-year-old woman was admitted because of a palpable left abdominal mass. Her mother, son, and daughter had neurofibromatosis 1. Computed tomography and magnetic resonance imaging revealed a 73 x 76 mm retroperitoneal mass. We performed complete resection of the tumor, confirming the margin status by frozen section examination intraoperatively. The histopathological examination revealed MPNST. Although no further therapy was performed, she is alive with no evidence of disease 11 months after surgery.