Melanotic neuroectodermal tumor of infancy: A rare case report with differential diagnosis and review of the literature

Abstract

Melanotic neuroectodermal tumor of infancy is a very rare benign but locally aggressive neoplasm of neural crest origin with a high recurrence rate. It usually affects infants of less than 1 year of age. Most commonly, the lesion affects the maxilla followed by skull, mandible, brain and other extracranial sites. We report an 11-month-old female baby with a similar tumor involving the anterior region of maxilla along with a brief review of the literature and differential diagnosis. A wide surgical excision was performed by an intraoral approach. A 2-year follow-up of the patient showed no recurrence.

Melanotic Neuroectodermal Tumor of Infancy of the Upper Arm

“…MNTI was first described in 1918 by Krompecher as a congenital melanocarcinoma [1,2,5]. The lesion usually involves the head and neck region of children less than 1 year of age [5].…”

“…Although a benign lesion, it can cause major deformities due to its rapid growth. The reported rate of recurrence is approximately 15%, while the rate of malignant transformation is 6.8% [1,2,4]. We present a rare case of a 6-month-old infant with MNTI of the upper arm.…”

“…The lesion most commonly presents in children less than 1 year of age with no gender predilection [1]. Clinically, the tumor presents as a solitary, rapidly growing, expansile painless mass.…”

“…Clinically, the tumor presents as a solitary, rapidly growing, expansile painless mass. The majority (90%) of MNTI present in the head and neck region, most commonly maxilla, although other locations have been described including the skull, mandible, and brain, as well as extracranial sites such as long bones, mediastinum, soft tissues of extremities, and genital organs [1,2]. Almost 500 cases of MNTI have been reported [3].…”

“…Melanotic neuroectodermal tumor of infancy (MNTI) is a rare and benign tumor of neural crest origin that occurs in early infancy [1,2]. The lesion most commonly presents in children less than 1 year of age with no gender predilection [1].…”