Epithelioid sarcoma in children and adolescents: a report of four cases.
Epithelioid sarcoma is an uncommon soft tissue tumor, most prevalent in the extremities of young adults. Youth has been described as a favorable prognostic factor. We describe four patients (two children aged 10 years and two adolescents aged 18 years) with extremity epithelioid sarcoma. Both 10-year-old children had rapid disease evolution and died 3 and 6 months after the diagnosis was made. Both tumors were strict diploid at DNA flow cytometry, but one had an abnormal karyotype (trisomy 2). Both adolescents are disease-free 18 months and 18 years after combined treatment consisting of local resection, regional lymph node dissection, and isolated regional perfusion with cytostatic agents.
de Vries
J
J
Department of Surgery, University Hospital Groningen, The Netherlands.
Hoekstra
H J
HJ
Oosterhuis
J W
JW
Postma
A
A
Schraffordt Koops
H
H
D000293:Adolescent; D002648:Child; D005121:Extremities; D005260:Female; D006801:Humans; D008207:Lymphatic Metastasis; D008297:Male; D009336:Necrosis; D012509:Sarcoma / Q000473:pathology*; D012983:Soft Tissue Neoplasms / Q000473:pathology*