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Year : 2019  |  Volume : 2  |  Issue : 2  |  Page : 29-32

“Primitive myxoid mesenchymal tumor of infancy” - A rare childhood soft-tissue tumor of the mandible

Department of Pathology, Amrita Institute of Medical Sciences, Kochi, Kerala, India

Correspondence Address:
Gittwa Vatsaraj Kottangal
Department of Pathology, Amrita Institute of Medical Science, Kochi, Keral
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JHNP.JHNP_13_20

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Primitive myxoid mesenchymal tumor of infancy (PMMTI) is an extremely rare soft-tissue tumor of childhood. We report a case of an 18-month-old male child diagnosed with PMMTI of the mandible. Till now <30 cases had been reported in the English literature. Histologically, the tumor consists of primitive spindle/polygonal cells dispersed in a myxoid background with delicate blood vessels. Immunohistochemically, tumor cells are positive for vimentin and negative for all other myoid, neural, epithelial, vascular, and neuroendocrine markers. This tumor was previously included under the diagnostic categories of congenital infantile fibrosarcoma or infantile fibromatosis. Increased awareness of this novel entity is a must as its biological and chemosensitive nature is vastly different from its differentials. Recent immunohistochemical stains and cytogenetic studies have helped to differentiate PMMTI from the other tumors of similar morphology.

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