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The Korean Journal of Pathology 1992;26(5): 490-495.
Malanotic Neuroectodermal Tumor of Infancy: A case report.
Young Chae Chu, Joon Mee Kim, Tae Sook Hwang, Il Kyu Kim
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.
Key Words: Melanotic neuroectodermal tumor of infancy; Retinal anlage tumor