Unusual presentation of peripheral primitive neuroectodermal tumor of the maxilla.

The peripheral primitive neuroectodermal tumor (pPNET) is a rare and highly malignant soft tissue neoplasm in children and young adults. Fewer than 10 reported cases of pPNET of maxilla are available in the English literature. A 28-year-old woman was presented with the pPNET of the maxilla and metastasis. Two years after diagnosis, she experienced diplopia, and then magnetic resonance imaging was done, which showed a mass in the optic chiasma and parasellar region. The typical appearance resembled large noncalcified soft tissue masses in the magnetic resonance image and computed tomographic scan of the maxilla. Diagnosis was established by immunohistochemical features. She was treated with surgery, chemotherapy, radiation therapy, and gamma knife. She was under close observation since then (approximately 8 mo), and there has been no recurrence of tumor up to now.