eMedicine Specialties > Pediatrics: General Medicine > Oncology

Liposarcoma

Author: Vikramjit S Kanwar, MD, MBA, MRCP(UK), FAAP, Associate Professor of Pediatric Hematology and Oncology, Department of Pediatrics, Albany Medical Center; Faculty, Alden March Bioethics Institute
Coauthor(s): Anastasios K Konstantakos, MD, Clinical Associate Surgeon, Department of Cardiovascular Surgery, Billings Clinic, Billings, Montana; David L Dudgeon, MD, Rainbow Babies and Children's Hospital, Professor, Department of Surgery, University Hospitals of Cleveland and Case Western Reserve University
Contributor Information and Disclosures

Updated: Jun 4, 2008

Introduction

Background

Liposarcoma is one of the least frequent nonrhabdomyosarcoma soft tissue sarcomas that occur in childhood; it comprises less than 5% of all soft tissue sarcomas in childhood.1,2  Surgical excision is the primary treatment, and prognosis depends on the histologic subtype and degree of resection. For patients with residual disease, radiotherapy has been used.1

Pathophysiology

Liposarcoma is a lipogenic tumor of large deep-seated connective tissue spaces. The 3 major locations in which liposarcomas are found are the lower extremities, the retroperitoneal region, and the shoulder area. The favored sites of occurrence in the lower extremities include the popliteal fossa and medial thigh. The most common retroperitoneal location is the perineal region. Occasionally, tumors may originate in the subcutis of shoulder, neck, and facial areas. Children tend to have a higher incidence of lower extremity tumors.3  

Well-differentiated liposarcomas have ring or marker chromosomes commonly derived from 1q and 12q13–15.4

The consistent cytogenetic abnormality in myxoid liposarcoma is translocation t(12;16)(q13;p11.2). This involves fusion of the transcription factor gene CHOP, which is essential for adipocytic differentiation, to the translocated in liposarcoma (TLS) gene on chromosome 16.5 In about 2% of cases, CHOP may fuse with the EWS gene on chromosome 22 in translocation t(12;22)(q13;q12).

These cytogenetic abnormalities have also been reported in the more aggressive round-cell liposarcoma; myxoid and round cell liposarcoma form a clear spectrum with regard to disease aggressiveness.6

Pleomorphic liposarcoma usually lacks this distinct translocation and appears to be biologically closer to other aggressive pleomorphic sarcomas.

Frequency

United States

In children, liposarcomas are rare and comprise fewer than 5% of soft tissue sarcomas.

Overall, around 100 cases of childhood liposarcoma have been reported in the literature, usually in the second decade of life. At a large New York Cancer Hospital, 18 cases of liposarcoma were reported in patients aged 22 years or younger over a period of 4 decades.7

Mortality/Morbidity

Due to its rarity, survival data for liposarcoma patients are often extrapolated from small series or from adult data. As with other childhood nonrhabdomyosarcoma soft tissue sarcomas, outcome is linked to various prognostic factors, including stage and grade. Complete surgical resection is crucial. The estimated 5-year survival rates for nonmetastatic, completely resected extremity tumors are impacted by histologic subtype and are as follows:8

  • Pleomorphic tumors - 50%
  • Round-cell tumors - 50%
  • Myxoid tumors - 80%
  • Well-differentiated tumors - 100% 
Local recurrence following resection is common and may be avoided by wide excision or adjuvant radiation.

Metastatic spread varies but commonly occurs to the lungs in high-grade pleomorphic tumors. Lymphatic spread is not seen. Myxoid liposarcoma is often considered intermediate grade but may still metastasize in 10-35% patients, sometimes to extrapulmonary soft tissue sites, such as the retroperitoneum or chest wall9 or even brain and spine.10

Race

No racial predilection is apparent.

Sex

In the several small series reported, gender predominance varies; assessing an accurate male-to-female ratio is not currently possible.

Age

Overall, the average age at presentation is 50 years. Liposarcomas are rarely seen in the teenage years and are almost never found in patients younger than 10 years. Earlier reports of liposarcoma in infancy were mostly thought to be lipoblastomatosis upon review.3

Clinical

History

  • Presentation varies, but the tumor usually presents as a painless slow-growing lesion. Only 10-15% patients have a painful rapidly growing mass or functional limitations.
  • Depending on the location and involvement of adjacent structures in the extremity, weakness or limitation of motion may be observed.
  • Rarely, nonspecific symptoms, such as weight loss, fatigue, and lassitude, may also be observed.

Physical

  • Fascial compartmentalization may cause soft tissue sarcomas to adopt awkward discoid and fusiform shapes rather than smooth round forms.
  • A fairly well-circumscribed palpable mass slowly increasing in size over many months appears to be the first manifestation of disease in many patients.
  • Pain is not often a prominent manifestation.
  • Diffuse abdominal enlargement may be observed in patients with retroperitoneal disease.
  • Characteristics of the primary tumor, such as size, texture, and mobility, are important to note.
  • The neurovascular status of the involved extremities distal to the tumor should be evaluated.
  • Palpation of draining lymph nodes usually does not reveal disease.

Causes

  • Although the precise etiology of liposarcomas is not yet defined, the presumed origin likely involves terminal dedifferentiation of mesenchymal cellular components.
  • For myxoid/round-cell liposarcomas, the TLS-CHOP oncoprotein plays a key role in tumor formation.11
  • No specific causative environmental factors have been identified because of the rarity of these tumors.

More on Liposarcoma

Overview: Liposarcoma
Differential Diagnoses & Workup: Liposarcoma
Treatment & Medication: Liposarcoma
Follow-up: Liposarcoma
References
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References

  1. Marcus KC, Grier HE, Shamberger RC, Gebhardt MC, Perez-Atayde A, Silver B. Childhood soft tissue sarcoma: a 20-year experience. J Pediatr. Oct 1997;131(4):603-7. [Medline].

  2. Spunt SL, Poquette CA, Hurt YS, et al. Prognostic factors for children and adolescents with surgically resected nonrhabdomyosarcoma soft tissue sarcoma: an analysis of 121 patients treated at St Jude Children's Research Hospital. J Clin Oncol. Dec 1999;17(12):3697-705. [Medline].

  3. Shmookler BM, Enzinger FM. Liposarcoma occurring in children. An analysis of 17 cases and review of the literature. Cancer. Aug 1 1983;52(3):567-74. [Medline].

  4. Italiano A, Cardot N, Dupre F, et al. Gains and complex rearrangements of the 12q13-15 chromosomal region in ordinary lipomas: the "missing link" between lipomas and liposarcomas?. Int J Cancer. Jul 15 2007;121(2):308-15. [Medline].

  5. Crozat A, Aman P, Mandahl N, Ron D. Fusion of CHOP to a novel RNA-binding protein in human myxoid liposarcoma. Nature. Jun 17 1993;363(6430):640-4. [Medline].

  6. Fiore M, Grosso F, Lo Vullo S, et al. Myxoid/round cell and pleomorphic liposarcomas: prognostic factors and survival in a series of patients treated at a single institution. Cancer. Jun 15 2007;109(12):2522-31. [Medline].

  7. La Quaglia MP, Spiro SA, Ghavimi F, Hajdu SI, Meyers P, Exelby PR. Liposarcoma in patients younger than or equal to 22 years of age. Cancer. Nov 15 1993;72(10):3114-9. [Medline].

  8. Chang HR, Hajdu SI, Collin C, Brennan MF. The prognostic value of histologic subtypes in primary extremity liposarcoma. Cancer. Oct 1 1989;64(7):1514-20. [Medline].

  9. Cheng EY, Springfield DS, Mankin HJ. Frequent incidence of extrapulmonary sites of initial metastasis in patients with liposarcoma. Cancer. Mar 1 1995;75(5):1120-7. [Medline].

  10. Schwab JH, Boland PJ, Antonescu C, Bilsky MH, Healey JH. Spinal metastases from myxoid liposarcoma warrant screening with magnetic resonance imaging. Cancer. Oct 15 2007;110(8):1815-22. [Medline].

  11. Antonescu CR, Elahi A, Humphrey M, et al. Specificity of TLS-CHOP rearrangement for classic myxoid/round cell liposarcoma: absence in predominantly myxoid well-differentiated liposarcomas. J Mol Diagn. Aug 2000;2(3):132-8. [Medline]. [Full Text].

  12. Brandal P, Bjerkehagen B, Heim S. Rearrangement of chromosomal region 8q11-13 in lipomatous tumours: correlation with lipoblastoma morphology. J Pathol. Feb 2006;208(3):388-94. [Medline].

  13. Song T, Shen J, Liang BL, Mai WW, Li Y, Guo HC. Retroperitoneal liposarcoma: MR characteristics and pathological correlative analysis. Abdom Imaging. Sep-Oct 2007;32(5):668-74. [Medline].

  14. Brenner W, Eary JF, Hwang W, Vernon C, Conrad EU. Risk assessment in liposarcoma patients based on FDG PET imaging. Eur J Nucl Med Mol Imaging. Nov 2006;33(11):1290-5. [Medline].

  15. Cecchetto G, Alaggio R, Dall'Igna P, et al. Localized unresectable non-rhabdo soft tissue sarcomas of the extremities in pediatric age: results from the Italian studies. Cancer. Nov 1 2005;104(9):2006-12. [Medline].

  16. Castleberry RP, Kelly DR, Wilson ER, et al. Childhood liposarcoma. Report of a case and review of the literature. Cancer. Aug 1 1984;54(3):579-84. [Medline].

  17. Enzinger FM, Weiss SW. Liposarcoma. In: Enzinger F, Weiss S, eds. Soft Tissue Tumors. St Louis, MO: Mosby; 1995:431-66.

  18. Ferrari A, Casanova M, Spreafico F, et al. Childhood liposarcoma: a single-institutional twenty-year experience. Pediatr Hematol Oncol. Sep-Oct 1999;16(5):415-21. [Medline].

  19. Gronchi A, Casali PG, Fiore M, et al. Retroperitoneal soft tissue sarcomas: patterns of recurrence in 167 patients treated at a single institution. Cancer. Jun 1 2004;100(11):2448-55. [Medline].

  20. Raaf JH, Ragsdale BD. Surgical management of liposarcoma. In: Bogumil GP, Fleegler EJ, eds. Tumors of the Hand and Upper Limb. 1993.

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Further Reading

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Keywords

liposarcoma, nonrhabdomyosarcoma soft tissue sarcoma, lipogenic tumor, connective tissue tumor, differentiated liposarcoma, myxoid liposarcoma, dedifferentiated liposarcoma, round-cell liposarcoma, pleomorphic liposarcoma, pediatric neoplasm, pediatric tumor, lower extremity tumor, retroperitoneal tumor, shoulder tumor, well-differentiated liposarcoma, pediatric cancer

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Contributor Information and Disclosures

Author

Vikramjit S Kanwar, MD, MBA, MRCP(UK), FAAP, Associate Professor of Pediatric Hematology and Oncology, Department of Pediatrics, Albany Medical Center; Faculty, Alden March Bioethics Institute
Vikramjit S Kanwar, MD, MBA, MRCP(UK), FAAP is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, and Royal College of Physicians of the United Kingdom
Disclosure: Nothing to disclose.

Coauthor(s)

Anastasios K Konstantakos, MD, Clinical Associate Surgeon, Department of Cardiovascular Surgery, Billings Clinic, Billings, Montana
Disclosure: Nothing to disclose.

David L Dudgeon, MD, Rainbow Babies and Children's Hospital, Professor, Department of Surgery, University Hospitals of Cleveland and Case Western Reserve University
Disclosure: Nothing to disclose.

Medical Editor

Stephan A Grupp, MD, PhD, Director, Stem Cell Biology Program, Department of Pediatrics, Division of Oncology, Children's Hospital of Philadelphia; Associate Professor of Pediatrics, University of Pennsylvania
Stephan A Grupp, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Steven K Bergstrom, MD, Assistant to the Chairman, Department of Pediatrics, Division of Hematology-Oncology, Kaiser Permanente Medical Center of Oakland
Steven K Bergstrom, MD is a member of the following medical societies: Alpha Omega Alpha, American Society of Clinical Oncology, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, and International Society for Experimental Hematology
Disclosure: Nothing to disclose.

CME Editor

Samuel Gross, MD, Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University
Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA, Senior Vice President, Children's National Medical Center (Center for Cancer and Blood Disorders); Director, Center for Cancer and Immunology Research, Children's Research Institute, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University
Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American Association for Cancer Research, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

 
 
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