Malignant peripheral nerve sheath tumor (
MPNST) is a neurogenic
tumor arising from peripheral nerves or nerve sheaths. MPNSTs are highly aggressive
sarcomas mainly associated with
neurofibromatosis type-1 (NF-1) with high rates of local recurrence and distant
metastasis carrying a dismal prognosis. Lung is the most common metastatic site. Bone
metastasis although documented in the literature is still very rare, while dissemination to brain without the involvement of lungs and that too in a non-NF-1 case is extremely unusual. A 48-year-old female was diagnosed with a case of non-NF-1
MPNST left thigh with bone
metastases including sphenoid. Despite showing complete resolution of skeletal and primary lesions postpalliative
chemoradiotherapy, she developed
brain metastases and succumbed to
her disease. This case is discussed to highlight an unusual scenario we encountered, the
clinical course of the disease with its management, and overall poor prognosis. To the best of our knowledge, this may be the earliest case of
MPNST with sphenoid
metastases detected by 18-fluorodeoxyglucose positron-emission computed tomography scan and a sporadic case of
brain metastases reported in the world literature.