Melanotic neuroectodermal tumor of infancy: A rare entity

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm with a predilection for the head-and-neck region. Approximately 472 cases reported in literature till date. We report an unusual case of MNTI in a 1-month old child. A 10 cm × 5 cm × 5 cm nontender, firm, deep-seated swelling was seen involving the right zygomatic and maxillary region. Magnetic resonance imaging was suggestive of rhabdomyosarcoma and immunomorphological assessment of the excision specimen revealed the final diagnosis of MNTI. On follow-up, the patient developed recurrence 4 months after surgery and was reoperated. The present case emphasizes that albeit rare, pediatric surgeons and pathologists must always keep in mind the possibility of MNTI while dealing with maxillofacial tumors in infants. Owing to rather nonspecific clinicoradiological features, histopathology supplemented with immunohistochemistry remains the gold standard for diagnosis. Although considered to be a benign tumor, close clinicoradiological follow-up is strongly recommended given the significant risk of recurrence as highlighted by our case.