title: Clear cell sarcoma of the jaw: a case report and review of the literature.
abstract: 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.
authors.affiliation: Department of Pediatrics, Division of Pediatric Hematology/Oncology, Michigan State University College of Human Medicine, East Lansing, MI, USA. sinoue1@hurleymc.com
authors.forename: Susumu
authors.initials: S
authors.lastname: Inoue
authors.forename: Douglas B
authors.initials: DB
authors.lastname: Chepeha
authors.forename: David R
authors.initials: DR
authors.lastname: Lucas
authors.forename: Shahzad
authors.initials: S
authors.lastname: Faisal
authors.forename: Ronald C
authors.initials: RC
authors.lastname: Stewart
authors.forename: Rao
authors.initials: R
authors.lastname: Mushtaq
authors.forename: Nkechi
authors.initials: N
authors.lastname: Onwuzurike
authors.forename: Lori
authors.initials: L
authors.lastname: McQuillan
mesh_terms: D000293:Adolescent; D005260:Female; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D007573:Jaw Neoplasms / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D020133:Reverse Transcriptase Polymerase Chain Reaction; D018227:Sarcoma, Clear Cell / Q000235:genetics / Q000473:pathology* / Q000601:surgery
chemical_list: C560860:EWSR1-ATF1 fusion protein, human; D015514:Oncogene Proteins, Fusion