Neuroblastoma‐like schwannoma of the skull base: an enigmatic peripheral nerve sheath tumor variant

Neuroblastoma‐like schwannoma is an extremely rare histological variant of schwannoma, which histologically mimics a malignant small round cell tumor. Only 19 cases have been reported in the literature to date. We report a case of this tumor located at the skull base in a 44‐year‐old woman who presented with symptoms of right‐sided earache and hearing loss. MRI revealed a large, lobulated, extra‐axial mass measuring 8.8 cm × 3.6 cm × 4.2 cm in the floor of the middle and posterior cranial fossa. Microscopic examination revealed a perplexing histopathology with peculiar collagenous rosettes. Differential diagnoses included a broad range of benign and malignant tumors. Typical schwannoma seldom poses a difficulty in diagnosis; however, this unusual variant is a diagnostic challenge which requires an extensive clinico‐radiological correlation and immunohistochemical work‐up. Hence, knowledge of this entity is a must to avoid erroneous diagnosis and inappropriate treatment.