Melanotic Schwannomas Are Rarely Seen Pigmented Tumors with Unpredictable Prognosis and Challenging Diagnosis

Abstract EN

Melanotic Schwannoma (MS) is rarely seen and potentially malignant neoplasm that is categorized as a variant of Schwannoma.

MS most frequently involves intracranial structures followed by posterior nerve roots in the spinal canal.

Approximately 50% of the cases with MS have psammomatous calcifications and this type of MS is related to Carney complex with autosomal dominant inheritance.

Most cases of MS are benign, though 10% of them are malignant with metastatic potential.

MS mimics melanoma and the differential diagnosis should be made excluding other melanin producing neoplasms especially melanoma.

Case 1.

A 42-year-old hypertensive male presented for checkup.

He had a well-defined extraspinal oval lesion measuring 3.5 × 2.5 cm near right adrenal.

Case 2.

A 22-year-old female presented with neurofibromatosis-2, bilateral acoustic schwannomas and café au lait lesions on sacrococcygeal region.

She had an intradural extramedullary lesion measuring 6.1 × 2.0 cm at L1-2 level.

MS is a rare neoplasm composed of Schwann cells and melanin pigment.

These tumors are usually benign but they may become aggressive.

The biologic behavior of MS is difficult to predict; the patients have to be followed up for a longer period due to its malignant potential.