Soft Tissue Sarcomas
A 35-year-old woman with known neurofibromatosis type 1 (NF1) presents with rapid enlargement of a previously stable thigh mass over 3 months. She reports new onset of severe pain and weakness in the affected leg. Examination reveals a large, firm mass with hard consistency and neurological deficit in the sciatic nerve distribution. MRI shows a heterogeneous mass with areas of hemorrhage and necrosis arising from the sciatic nerve. PET scan shows high FDG uptake. Regarding malignant peripheral nerve sheath tumor (MPNST):
Mark each as TRUE or FALSE
MPNST is a rare, aggressive soft tissue sarcoma arising from peripheral nerves or from pre-existing ...
Clinical warning signs of malignant transformation in NF1 patients include: rapid increase in tumor ...
MPNST is a benign condition; it never occurs in NF1 patients; sporadic cases are more common than NF...
Histologically, MPNST shows spindle cells with fascicular growth pattern, nuclear atypia, high mitot...
Treatment is wide surgical resection with negative margins; sacrifice of the involved nerve is typic...
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Click T (True) or F (False) for each option