Introduction: Neurothekoma is a rare benign tumor which must be distinguished from certain malignant tumors such as fibrohistiocyte tumors or plexiform cell tumors, neurotropic melanomas and clear-cell sarcoma.
Case report: An 11-year-old girl consulted for a recurrent subcutaneous tumor of the chin which had been operated 4 months earlier. The resection was incomplete. A wider revision resection successfully stopped recurrence. The histology study established the diagnosis of neurothekoma due to the presence of mitosis atypia, cellular nodules, and extension to the hypodermis. Immunohistochemistry confirmed the diagnosis.
Discussion: Neurothekoma is a benign tumor observed in young women, mainly on the face. It occurs as a dermal cohesive mass without infiltration of the epidermis. The typical immunohistochemical pattern enables differential diagnosis with myxoid neurothekoma, melanocytic and nervous system tumors. Surgical resection is indicated.