Malignant epitheloid schwannoma: A rare tumor causing a diagnostic dilemma

Malignant peripheral nerve sheath tumor is a rare but well recognized tumor of peripheral nerves and neurofibroma accounting for 10% of soft tissue sarcomas. Epitheloid MPNST (EMPNST) accounts for 5% or less of MPNST. These tumors are locally aggressive and have high risk of distant metastasis. Early and correct diagnosis is necessary for timely appropriate management of these tumors. Histological features of EMPNST closely mimic other neuroendocrine tumors as well as clear cell carcinoma, posing extreme diagnostic difficulty. A careful histomorphological evaluation and use of immunohistochemical stains is necessary for correct diagnosis.