eMedicine Specialties > Pediatrics: General Medicine > Oncology

Liposarcoma: Treatment & Medication

Author: Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP, Associate Professor of Pediatric Hematology-Oncology, Department of Pediatrics, Albany Medical Center; Faculty, Alden March Bioethics Institute
Coauthor(s): Anastasios K Konstantakos, MD, Clinical Associate Surgeon, Brigham and Women's Hospital, Harvard University; 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

Treatment

Medical Care

Chemotherapy has been shown to be active in these tumors, but its role needs to be defined in clinical trials.

Postoperative radiotherapy may be administered (see Further Inpatient Care).

Surgical Care

Surgical objectives include obtaining an accurate histologic diagnosis, minimizing the chance of local recurrence, achieving the best possible functional and anatomic result, and maximizing the probability of survival. Surgery should be performed at a children's cancer center with significant experience dealing with these rare tumors.

Open biopsy must be meticulously performed to avoid hematoma, tumor cell spillage, and postoperative infection. The incision must be oriented so that the biopsy site can be completely encompassed in the definitive resection. A longitudinal incision parallel with the fiber direction of the underlying muscle is used. Under ideal conditions, the surgeon performing the definitive resection also should perform the initial biopsy. Sometimes, performing the incisional biopsy and resection is possible during the same procedure, provided that the frozen section is definitive.

The 3 main techniques of surgical resection used in patients with liposarcoma include simple excision, wide en bloc resection, and amputation. The type of resection used is determined by the tumor's histology and by the anatomic findings at the time of surgery.

  • If the lesion appears to be grossly and histologically consistent with lipoma or well-differentiated liposarcoma, simple excision is acceptable.
  • If the mass contains areas suggestive of low-grade liposarcoma with clear margins, simple marginal excision can be curative. When evidence suggesting high-grade liposarcoma is present, either a wider resection of the tumor bed may be performed or adjuvant radiotherapy may be added.
  • If preoperative studies (CT scanning, MRI, biopsy specimen analysis) suggest a high-grade lesion, either wide en bloc resection or amputation can be planned. Avoid shelling out a high-grade tumor because microscopic disease is left behind. In patients in whom amputation is under serious consideration (either as an initial procedure or following a limb-sparing operation), preoperative education is imperative.
  • In upper extremity tumors, axillary dissection is not performed unless the nodes feel abnormal.

Consultations

  • Pediatric oncologist: For lesions in which malignancy is strongly suspected or in which a previous incisional biopsy has revealed liposarcoma, consultation with pediatric oncologist is recommended prior to the definitive surgical procedure.
  • Radiation oncologist: Adjuvant therapy may be indicated in patients in whom excision is incomplete or when close margins are noted along with concern about microscopic residual disease. Consultation with a radiation oncologist is recommended.

Activity

Consultation with a physical therapist and referral for rehabilitation may be appropriate, depending on the site of the primary and the degree of surgical resection performed.

Medication

The role of adjuvant chemotherapy in soft tissue sarcomas is not clearly defined. A definitive survival advantage for patients with incompletely resected tumors who are treated with combination chemotherapy has not been established, despite the fact that the tumors can respond to chemotherapy.15

Although no definitive chemotherapeutic protocol has been established, doxorubicin and ifosfamide appear to be the most effective cytotoxic agents for nonrhabdomyosarcoma soft tissue sarcomas. In this setting, chemotherapy is investigational and consultation with a pediatric oncologist who has experience with nonrhabdomyosarcoma soft tissue tumors is required.

More on Liposarcoma

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

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  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].

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  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].

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  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].

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  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].

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  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, MBBS, MBA, MRCP(UK), FAAP, Associate Professor of Pediatric Hematology-Oncology, Department of Pediatrics, Albany Medical Center; Faculty, Alden March Bioethics Institute
Vikramjit S Kanwar, MBBS, 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, Brigham and Women's Hospital, Harvard University
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.com, Inc
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

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, UNC, 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, Executive Director, Center for Cancer and Blood Disorders, Children's National Medical Center, Washington, DC; 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 Clinical Oncology, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

 
 
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