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Abstract

Melanotic neuroectodermal tumor of infancy: clinical, radiologic, and pathologic findings in five cases.

D R Mirich, S I Blaser, D C Harwood-Nash, D C Armstrong, L E Becker and J C Posnick
American Journal of Neuroradiology July 1991, 12 (4) 689-697;
D R Mirich
Department of Diagnostic Radiology, Hospital for Sick Children, University of Toronto, Ontario, Canada.
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S I Blaser
Department of Diagnostic Radiology, Hospital for Sick Children, University of Toronto, Ontario, Canada.
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D C Harwood-Nash
Department of Diagnostic Radiology, Hospital for Sick Children, University of Toronto, Ontario, Canada.
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D C Armstrong
Department of Diagnostic Radiology, Hospital for Sick Children, University of Toronto, Ontario, Canada.
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L E Becker
Department of Diagnostic Radiology, Hospital for Sick Children, University of Toronto, Ontario, Canada.
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J C Posnick
Department of Diagnostic Radiology, Hospital for Sick Children, University of Toronto, Ontario, Canada.
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Abstract

Five pathologically proved melanotic neuroectodermal tumors of infancy are reported. These rare neoplasms of infancy exhibit a distinct predilection for the maxillary bone. Three tumors originated in the maxilla, one in the calvaria, and one in the cerebellar vermis. Those occurring in bone did not metastasize but were locally invasive, as reflected in their radiologic appearance. Bone erosion, expansion, hyperostosis, and osteogenesis can occur in the same neoplasm and were appreciated best on CT. MR imaging showed the soft-tissue component and extent of the neoplasm better than CT did. The pathologic findings from all five cases (and one possibly related melanotic tumor of the face) revealed abundant melanin. MR imaging of two melanotic tumors showed isointense T1-weighted and slightly hyperintense T2-weighted signals. This appearance is contrary to that of most melanin-containing tumors, which exhibit enhanced T1 and T2 relaxation, and indicates that variables other than the absolute amount of melanin may determine the MR signal. Clinically, rapid neoplastic growth and excessive melanin production by the tumor cells caused facial disfigurement and visible blue black discoloration. All five melanotic neuroectodermal tumors were resected and the vermian tumor was also irradiated. Four of five children were well and free from disease 1 month to 7 years after resection. The calvarial tumor was incompletely resected and involved the underlying brain, eventually causing death. The clinical, radiologic, and pathologic features of melanotic neuroectodermal tumors of infancy are reviewed. Melanotic neuroectodermal tumors of infancy that involve bone can be diagnosed from the clinical and radiologic findings. Prompt diagnosis and surgical resection are essential for cure.

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American Journal of Neuroradiology
Vol. 12, Issue 4
1 Jul 1991
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Melanotic neuroectodermal tumor of infancy: clinical, radiologic, and pathologic findings in five cases.
D R Mirich, S I Blaser, D C Harwood-Nash, D C Armstrong, L E Becker, J C Posnick
American Journal of Neuroradiology Jul 1991, 12 (4) 689-697;

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Melanotic neuroectodermal tumor of infancy: clinical, radiologic, and pathologic findings in five cases.
D R Mirich, S I Blaser, D C Harwood-Nash, D C Armstrong, L E Becker, J C Posnick
American Journal of Neuroradiology Jul 1991, 12 (4) 689-697;
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