Epithelioid sarcoma in children and adolescents: A report of four cases

J. de Viries; H. J. Hoekstra; J. W. Oosterhuis; A. Postma; H. Schraffordt Koops

doi:10.1016/S0022-3468(89)80246-5

Epithelioid sarcoma in children and adolescents: A report of four cases

J. de Viries, H. J. Hoekstra, J. W. Oosterhuis, A. Postma, H. Schraffordt Koops

Group Looijenga

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Abstract

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.

Original language	English
Pages (from-to)	186-188
Number of pages	3
Journal	Journal of Pediatric Surgery
Volume	24
Issue number	2
DOIs	https://doi.org/10.1016/S0022-3468(89)80246-5
Publication status	Published - Feb 1989

Keywords

Epithelioid sarcoma

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title = "Epithelioid sarcoma in children and adolescents: A report of four cases",

abstract = "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.",

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AU - de Viries, J.

AU - Hoekstra, H. J.

AU - Oosterhuis, J. W.

AU - Postma, A.

AU - Schraffordt Koops, H.

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N2 - 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.

AB - 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.

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Epithelioid sarcoma in children and adolescents: A report of four cases

Abstract

Keywords

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