Korean J Pathol.
1992 Oct;26(5):490-495.
Malanotic Neuroectodermal Tumor of Infancy: A case report
- Affiliations
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- 1Department of Anatomical Pathology, Inha Hospital, Inha University, Sungnam, Korea.
- 2Department of Pathology, Inha Hospital, Inha University, Sungnam, Korea.
- 3Department of Dentistry, Inha Hospital, Inha University, Sungnam, Korea.
Abstract
- The melanotic neuroectodermal tumor of infancy(MNTl) is a rare, usually benign, pigmented lesion which most frequently involves the maxilla. Skull, brain, and mandible are other craniofacial sites; this anatomic region accounts for 87% of all cases. The vast majority of MNTl, nearly 95%, present in infants of both sexes within the first year of life. Most cases of MNTl follow a benign course, with a 15% propensity for local recurrence. MNTl is regarded as a primitive neuroectodermal tumor with polyphenotypic differentiation. We report a classical MNTl which occurred in left lower maxilla in 6-month old female infant. She developed an ill defined, firm swelling on the left alveolar ridge and gum during 3 weeks. Mass excision reveals ill defined, grayish-tan hard mass, measuring 2.0x1.6 cm. Microscopically, the tumor reveals irregular alveolar pattern of melanin-containing cuboidal cells and nests of small round cells. The immunohistochemical staining for melanoma-associated antigen, neuron-specific enolase and cytokeratin show positive reactions. Ultrastructural examination showed intracytoplasmic melanosome, tonofilament and desmosome.
