An Unusual Variant of Anlage Tumor of Pineal Region in an Infant

A 9-month-old male child was brought with complaints of increasing head size for 2 months, increasing lethargy and vomiting for the last 2 days. Radiology revealed a heterogeneously enhancing, globular lesion in the pineal region with hydrocephalus. Near total excision of the tumor was carried out. The histopathological examination of the lesion showed heterogenous elements in the form of mature neuroepithelial and ectomesenchymal tissue. The pathology and radiology of this unusual lesion is discussed with relevant review of literature.

Pineal Anlage Tumor: A case report

The term "pineal anlage tumor" has been recently proposed and few cases have been reported. We report the first Korean case of pineal anlage tumor in a 6-year-old girl who complained of headache and vomiting for 2 months. Brain MRI revealed a well defined, lobulated, calcifying mass in the pineal region. Tumor was totally removed. Pathological examination revealed a primitive pineal parenchymal tumor with melanotic epithelial component that was similar to histologic findings of melanotic neuroectodermal tumor of infancy, so-called retinal anlage tumor and of the developing pineal gland. The tumor was composed mostly of small, undifferentiated cells, Flexner-Wintersteiner rosettes, and ganglionic differentiation. The tumor also contained the cartilage and skeletal muscle cells.

Malanotic Neuroectodermal Tumor of Infancy: A case report

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.