Melanotic neuroectodermal tumor of infancy: A rare case repo
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Melanotic neuroectodermal tumor of infancy (MNTI) is an extremely rare benign tumor that usually affects premaxilla in newborns of <1 year of age. Clinically, this tumor is expressed as nonulcerative, rapidly progressing, painless pigmented swelling on the anterior maxilla. MNTI is known by many alternative names such as melanotic progonoma, pigmented epulis of infancy, pigmented ameloblastoma, and melanotic epithelial odontoma. Besides the involvement of maxillary region (68%–80%), it also involves skull (10.8%), a mandible (5.8%), and brain (4.3%). The mean age of patients as reported in the literature is 4.3 months and having a near-equal gender predilection ratio of 6:7. Hereby present a case of MNTI treated with wide surgical excision along with a review on recent histopathologic knowledge and treatment considerations.

A 2-year-old female baby came with complaint of swelling over the left maxillary region. Parents noted swelling 6 months before which was slowly growing initially and started progressing rapidly for 1½ months. There was no relevant medical or family history and the development of the child was normal for her age. On clinical examination, a single nonpedunculated, pigmented swelling of 4.5 cm × 3 cm seen without any ulceration or discharge seen over the left maxillary region. The swelling has obliterated labial vestibule uplifting upper lip and causing flaring of the nose on the right side. Color of the overlying skin in the center and next to ala of nose on affected side changed to pinkish red. On palpation swelling was nontender, soft to firm in consistency, compressible, and non-fluctuant. Radiographic presentation of computed tomography images showed a single expansile solid lesion in the right anterior maxilla with epicenter lying in the maxillary anterior alveolar ridge. The tumor mass surgically enucleated and sent for histopathological examination. Histological features were consistent with a small round cell tumor favoring MNTI, displaying the biphasic population of cells. Immunohistochemical studies were done for confirmation. Thus, a final diagnosis of MNTI was made by correlating clinical, surgical findings, histopathological, and immunohistochemistry (IHC) findings.