Myxoid malignant fibrous histiocytoma presenting as a midline nasal mass

March 31, 2012 by Jaimie DeRosa, MD and Jeffrey R. Smit, MD
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Abstract

Myxoid malignant fibrous histiocytoma is a rare type of pediatric non-rhabdomyosarcoma soft-tissue sarcoma. The case of a 5-year-old girl is presented, highlighting the potential for multiple pitfalls and aberrant differential diagnoses that need to be identified for successful treatment of pediatric myxofibrosarcomas. An awareness of these tumors and a call for standardized postsurgical treatment protocols is necessary in order to successfully treat children with this disease.

IntroductionOtolaryngologists must have a high degree of clinical suspicion for midline nasal masses in children. Benign tumors-including dermoid cysts, gliomas, and vascular malformations, as well as neoplasms of the head and neck-need to be considered. This report describes the histopathology of a rare pediatric non-rhabdomyosarcoma soft-tissue sarcoma (NRSTS), a myxoid malignant fibrous histiocytoma (MMFH), presenting in a young child as a subcutaneous nasal mass.

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