Malignant melanotic nerve sheath tumor

Malignant melanotic nerve sheath tumor, previously melanotic schwannoma, is a rare peripheral nerve sheath tumor composed of melanin-producing Schwann cells, often clinically misdiagnosed as melanoma. It is an uncommon neoplasm with unpredictable biological behavior and varying treatments. Despite its histologic features indicative of benignity, including a low proliferative index, its clinical course is frequently malignant, with a high local recurrence rate and metastatic potential. The distinctive characteristics of malignant melanotic nerve sheath tumor include a relatively young patient age, signs of Carney complex, and psammoma bodies within the tumor. This study presents a case of a patient with malignant melanotic nerve sheath tumor located in the neck with a recurrent clinical course who underwent two surgical procedures. Microscopic and immunohistochemical evaluation of postoperative specimens showed positivity for melanoma markers, absence of psammoma bodies, and low mitotic activity. The clinical course of the disease was malignant, with recurrence developing in the postoperative scar. Diagnosing malignant melanotic nerve sheath tumor is radiologically and pathologically challenging. Multidisciplinary collaboration among the surgeon, radiologist, and pathologist; understanding the lesion’s anatomical context; and close attention to histopathologic findings are critical for establishing an accurate diagnosis.