Liposarcoma in children and young adults: a multi-institutional experience.

Abstract

BACKGROUND:

There are limited data regarding the differences in clinical presentation and outcome of liposarcomas between adult and pediatric patients. The role of adjuvant radiotherapy in the treatment of childhood liposarcoma is unclear.

PROCEDURE:

A multi-institutional retrospective analysis of medical records was performed for patients ≤ 21 years of age presenting with a verified histologic diagnosis of liposarcoma.

RESULTS:

Thirty-three patients were evaluable for this study, 23 of whom were male. Median age was 17.2 years. Twenty-four cases were myxoid subtype and 7 were pleomorphic subtype. In myxoid cases, 17 (71%) presented with extremity tumors; none had metastases. Eleven of these patients with myxoid subtype were treated with surgery only, seven with surgery + radiation, three with surgery + radiation + chemotherapy. Median radiation therapy dose for patients with myxoid tumors was 60 Gy. At median follow-up of 4.2 years (range 0.1-32.2 years), two patients relapsed with one death from progressive disease. In seven pleomorphic cases, four patients had primary tumors at central axial sites. Six patients (86%) received multimodal therapy, but six patients experienced relapse of disease. Four patients died from progressive disease.

CONCLUSIONS:

Pediatric liposarcoma has a different spectrum of presentation compared to adult cases. Myxoid liposarcoma is the more common subtype, usually occurs in extremities, and has an excellent prognosis. Pleomorphic liposarcoma occurs in axial sites, and despite multimodal therapy, outcome is poor. Further study is needed to identify the optimal therapy for pediatric liposarcoma.

• Authors: Winston W Huh, Carrie Yuen, Mark Munsell, Andrea Hayes-Jordan, Alexander J Lazar, Shreyaskumar Patel, Wei-Lien Wang, Nadia Barahmani, M Fatih Okcu, John Hicks, Larisa Debelenko, Sheri L Spunt
• Journal: Pediatric blood & cancer (Pediatr Blood Cancer) Vol. 57 Issue 7 Pg. 1142-6 (Dec 15 2011) ISSN: 1545-5017 [Electronic] United States
• PMID: 21394894 (Publication Type: Journal Article, Multicenter Study, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2011 Wiley Periodicals, Inc.

Chemical References

• Antineoplastic Agents

Topics

• Adolescent
• Antineoplastic Agents (therapeutic use)
• Child
• Combined Modality Therapy
• Female
• Humans
• Kaplan-Meier Estimate
• Liposarcoma (mortality, pathology, therapy)
• Male
• Radiotherapy
• Retrospective Studies
• Soft Tissue Neoplasms (mortality, pathology, therapy)
• Young Adult