Melanotic neuroectodermal tumour of infancy

Abstract

Melanotic neuroectodermal tumour of infancy (MNTI) is a rare benign tumour of neural crest origin that was first described by Krompecher in 1918.1 It is predominantly found in infancy, with about 92% of cases below the age of 12 months and 82% below the age of 6 months. The predominant site of origin is in the premaxilla though it is reported at other sites also including the skull, the mandible, the epididymis and the brain.2 The lesions often have areas of bluish discolouration on the surface and are characterised by displacement of the involved tooth bud and local aggressiveness. The present report deals with two cases of MNTI, a 5-month-old baby girl and a 6-month-old baby boy who reported to the Department of Oral and Maxillofacial Pathology, Dr R Ahmed Dental College and Hospital, Kolkata, India. The clinical, radiological, histological and immunohistochemical findings, confirmed the diagnosis of MNTI. Flow cytometry was performed to analyse aneuploidy. The tumours were treated surgically with no history of recurrence to date.

An Alternate Method of Fabrication of an Obturator in a Patient Diagnosed with Melanotic Neuroectodermal Tumor of Infancy

“…[4] Many investigators have proposed numerous theories regarding tumor histogenesis which has resulted in its varied nomenclature. [6][7][8][9][10] Current nomenclature of MNTI which reflects its neural crest cell origin is widely accepted and recognized by WHO. [11] Radiologically, the tumor is osteolytic in appearance with adjoining deciduous teeth appearing to be "floating" within the lesion.…”

Epididymal melanotic neuroectodermal tumor of infancy: A rare cause of scrotal mass in an infant

“…MNTI primarily affects the pediatric population with a slight male predilection . Clinically, MNTI is a firm, non‐tender, rapidly‐growing mass most commonly occurring in the maxilla (63% of all reported cases) followed by the skull and mandible . MNTI, irrespective of location, usually occurs in the first year of life with a median age at presentation of 4.5 months and only a minority of cases presents in older children and adults .…”

“…Of 12 reported cases in the epididymis, two showed malignant transformation and metastases and the rate of malignancy has been shown to be higher in epididymal MNTI relative to MNTI in extrascrotal locations . A few cases have been reported where death occurred due to disseminated MNTI …”

“…US can also help stage the extent of these lesions in addition to ongoing surveillance for recurrence. Given the extremely low incidence of this tumor type, pathologic diagnosis is essential to hasten treatment, avert misdiagnosis as a malignant lesion, and subsequent over‐treatment …”

“…Melanotic neuroectodermal tumor of infancy (MNTI) was first described by Krompecher in 1918. It is a rare, benign neoplasm of neural crest origin that predominantly involves the craniofacial region . MNTI involving the epididymis is quite unusual, with fewer than 25 cases reported .…”