Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Multicenter Study
. 2011 Dec 15;57(7):1142-6.
doi: 10.1002/pbc.23095. Epub 2011 Mar 10.

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

Winston W Huh  1 , 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
Affiliations
Multicenter Study

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

Winston W Huh et al. Pediatr Blood Cancer. .

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.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Kaplan-Meier estimate of overall survival (OS) for entire evaluated cohort.
Figure 2
Figure 2
Kaplan-Meier estimate of OS for patients with myxoid and well differentiated liposarcomas versus pleomorphic liposarcomas.
See this image and copyright information in PMC

References

    1. National Cancer Institute: Surveillance, Epidemiology, and End Results (SEER) Program Public-Use Data (1973–2006) National Cancer Institute, DCCPS, Surveillance Research Program, Cancer Statistics Branch; released April 2009, based on the November 2008 submission. www.seer.cancer.gov.
    1. Mack TM. Sarcomas and other malignancies of soft tissue, retroperitoneum, peritoneum, pleura, heart, mediastinum, and spleen. Cancer. 1995;75:211–244. - PubMed
    1. Sandberg AA. Updates on the cytogenetics and molecular genetics of bone and soft tissue tumors: liposarcoma. Cancer Genet Cytogenet. 2004;155:1–24. - PubMed
    1. Dalal KM, Kattan MW, Antonescu CR, et al. Subtype specific prognostic nomogram for patients with primary liposarcoma of the retroperitoneum, extremity, or trunk. Ann Surg. 2006;244:381–391. - PMC - PubMed
    1. Engstrom K, Bergh P, Gustafson P, et al. Liposarcoma. Outcome based on the Scandinavian Sarcoma Group Register. Cancer. 2008;113:1649–1656. - PubMed

Publication types

Substances