Surg Neurol 1992;37:123-9

123

Intracranial Melanotic Neuroectodermal Tumor of Infancy: Two Case Reports J o h n S. Y u , M . D . , M a t t h e w

R. M o o r e ,

M . D . , W i l l i a m J. K u p s k y , M . D . ,

a n d R. M i c h a e l S c o t t , M . D . Department of Neurosurgery, Brigham & Women's Hospital and The Children's Hospital; and Department of Pathology, The Children's Hospital, Harvard Medical School, Boston, Massachusetts

Yu JS, Moore MR, Kupsky WJ, Scott RM, Intracranial melanotic neuroectodermal tumor of infancy: two case reports. Surg Neurol 1992;37:123-9.

Two childhood cases of intracranial melanotic neuroectodermal tumor, one benign a n d o n e m a l i g n a n t , a r e presented. The clinical, radiologic, and pathologic features of these c a s e s a r e d e s c r i b e d a n d the relevant literature is reviewed. KEY WORDS: Melanotic neuroectodermal tumor of infancy; Melano6c progonoma

The melanotic neuroectodermal tumor of infancy (MNTI or melanotic progonoma) is a rare tumor that typically occurs in the maxilla of infants [5,8,9,14]. It has less frequently arisen in other sites, including the cranium, skin, epididymis, mediastinum, thigh, and the central nervous system (CNS). Most M N T I occur in the first year of life and follow a benign course [8,14,20]. The tumor has been referred to by a variety of names, including melanotic adamantinoma, melanotic epithelial odontoma, retinal anlage tumor, and odontogenic hamartoma, which indicates the controversial theories of its histogenesis. M N T I arising in the posterior fossa has features that overlap those of melanin-containing primitive neuroctodermal tumors of the cerebellum ("melanotic medulloblastoma"). We present two cases ofintracranial MNTI, one supratentorial and one infratentorial, and discuss their different outcomes and differential diagnoses. Case 1 An otherwise healthy 4-month-old boy was admitted for evaluation o f a midline frontal scalp mass. His mother Address for D r Yu: Department of Surgery (Neurosurgery Service), Massachusetts General Hospital, Boston, Massachusetts 02114. Address reprint requests to." R. Michael Scott, M.D., Department of Neurosurgery, The Children's Hospital, 300 Longwood Avenue, Boston, Massachusetts 02115. Received June 15, 1990; accepted August 6, 1991.

Intracranial melanotic neuroectodermal tumor of infancy: two case reports.

Two childhood cases of intracranial melanotic neuroectodermal tumor, one benign and one malignant, are presented. The clinical, radiologic, and pathol...
6MB Sizes 0 Downloads 0 Views