Clear cell sarcoma (CSS) is a unique malignant soft tissue tumor that mainly occurs from the aponeurotic tissue and tendons of extremities. It is rare in the pediatric population. The tumor does not respond well to chemotherapy or irradiation. Complete surgical resection offers the best chance for a cure. Most studies have demonstrated poor prognosis of this tumor, if it is >5 cm. The literature suggests that local recurrence and distant metastasis are not uncommon even with wide resection and that late recurrence and metastasis commonly occur. This case report discusses CSS in the jaw of a pediatric patient. To our knowledge, this is the only case of CSS of the jaw.
Departments of *Pediatrics, Division of Pediatric Hematology/Oncology
¶Combined Medicine/Pediatrics Residency Program, Hurley Medical Center, Flint
†Department of Pediatrics and Human Development, Michigan State University College of Human Medicine, East Lansing
§Department of Pathology, University of Michigan School of Medicine
‡Department of Head and Neck Oncology, Division of Microvascular-Reconstructive Surgery
∥Surgical Pathology Fellowship Program, University of Michigan, Ann Arbor, MI
The authors declare no conflict of interest.
Reprints: Susumu Inoue, MD, Hurley Medical Center, One Hurley Plaza, Flint, MI 48503 (e-mail: firstname.lastname@example.org).
Received December 6, 2011
Accepted March 29, 2012