Melanotic neuroectodermal tumor of infancy: discussion of a case and a review of the imaging findings

Abstract

Melanotic neuroectodermal tumor of infancy (MNTI) is an uncommon melanin-containing mesenchymal tumor of neural crest origin. What make this tumor unique and interesting is its characteristic predilection for anterior maxilla (premaxilla) and the presence of pigment melanin which gives the tumor distinct clinicopathological, immunohistochemical, ultrastructural and imaging features. Although first described almost a century ago, to the authors’ knowledge, only a few hundred cases of MNTI have been reported worldwide in the English medical literature. The pool of documented radiological findings is even more sparse as not more than a dozen cases could be abstracted from an Internet search of the radiology literature. We document a case of MNTI and describe the imaging findings with intent to contribute to its small but accruing radiological data.

Metastatic Melanotic Neuroectodermal Tumor of Infancy: Report of a Case and Review of the Literature

“…12 The histologic features (Figs. 5 -7) showed a small round cell neoplasm which was compartmentalized by varying sized fibrous septa.…”

Melanotic neuroectodermal tumour of infancy: CT and MR findings

“…It has a predilection for the head and neck region, particularly for the maxilla. Presence of melanin in this tumour is said to give it distinct clinicopathological, immunohistochemical, ultrastructural and imaging features [1]. We describe five further cases of MNTI, with an emphasis on computed tomography (CT) and magnetic resonance (MR) imaging findings, which have yet to be clearly described in the available radiological literature for this tumour.…”

Melanotic Neuroectodermal Tumor of Infancy Arising in the Temporal Bone

“…Most tumors were iso/hypointense with focal areas of hyperintensity on T1-and T2-weighted magnetic resonance images, and showed intense enhancement after intravenous gadolinium administration. 1,3,4,9 Although uncommon, the typical pattern of T1-shortening because of melanin deposition was helpful in diagnosis of this lesion. 10 Clinical and radiologic findings can suggest a diagnosis of melanotic neuroectodermal tumor of infancy, but histopathologic and immunohistochemical examination are required for a definite diagnosis.…”