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Keywords:

  • pleomorphic;
  • sarcoma;
  • fibrous;
  • histiocytoma;
  • parotid

Abstract

Background.

Undifferentiated pleomorphic sarcoma, or malignant fibrous histiocytoma (MFH) of the head and neck is an uncommon malignancy that is exceedingly rare in the pediatric population. Although MFH was once considered the most common soft tissue sarcoma in adults, recently authors have questioned its existence as a distinct pathologic entity.

Methods.

In light of this debate, we present a case of MFH with giant cells involving the parotid gland, and we review its pathology.

Results.

We describe a 6-year-old male with parotid gland MFH. Diagnosis was fraught with difficulty, typical of this disease. He was treated with a combination of chemotherapy and radiation therapy with a good initial response.

Conclusion.

The classification of MFH has been recently debated. Nevertheless, our case is the second report of pediatric MFH involving the parotid gland. Surgical resection is the preferred treatment, but combined chemoradiation may be necessary in the head and neck region. © 2007 Wiley Periodicals, Inc. Head Neck, 2008