title: Epithelioid sarcoma in children.
abstract: Literature regarding the natural history and prognosis of epithelioid sarcoma in the pediatric population is sparse. During the past 11 years, eight patients with epithelioid sarcoma were treated at St Jude Children's Research Hospital. Of the eight children, six of whom were male, seven presented with localized disease and one with metastatic disease. The primary tumor was located in the upper extremity in four patients; the other sites were the scalp, external ear, back, and mediastinum. The mean duration of symptoms before diagnosis was 8.3 months (range, 1 to 12 months). Surgical treatment comprising amputation or wide local excision was performed in six patients, three of whom had additional lymph node dissection. Three patients received multiagent chemotherapy, and four patients were treated with supplementary radiation. Two patients died of progressive local and distant disease within 9 months of diagnosis. The other six patients are alive and have no evidence of disease 12 months to 10 years 6 months after the completion of treatment.
authors.affiliation: Department of Surgery, Hematology-Oncology, Diagnostic Imaging, St Jude Children's Research Hospital, Memphis, TN 38105, USA.
authors.forename: E
authors.initials: E
authors.lastname: Gross
authors.forename: B N
authors.initials: BN
authors.lastname: Rao
authors.forename: A
authors.initials: A
authors.lastname: Pappo
authors.forename: L
authors.initials: L
authors.lastname: Bowman
authors.forename: P
authors.initials: P
authors.lastname: Shearer
authors.forename: S
authors.initials: S
authors.lastname: Kaste
authors.forename: C
authors.initials: C
authors.lastname: Greenwald
authors.forename: E
authors.initials: E
authors.lastname: Michalkiewicz
authors.forename: C
authors.initials: C
authors.lastname: Pratt
mesh_terms: D000293:Adolescent; D000367:Age Factors; D000671:Amputation, Surgical; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008197:Lymph Node Excision; D008297:Male; D011379:Prognosis; D011878:Radiotherapy; D012509:Sarcoma / Q000401:mortality / Q000628:therapy*; D012983:Soft Tissue Neoplasms / Q000401:mortality / Q000628:therapy*; D015996:Survival Rate
chemical_list: D000970:Antineoplastic Agents