Pediatric Liposarcoma Treatment & Management

  • Author: Vikramjit S Kanwar, MD, MBA, MRCP(UK), FAAP; Chief Editor: Max J Coppes, MD, PhD, MBA   more...
 
Updated: Feb 15, 2011
 

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).

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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.[20]

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.

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Consultations

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.

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.

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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.

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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.

Specialty Editor Board

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.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

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.

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, Center for Cancer and Blood Disorders, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University School of Medicine; Clinical Professor of Pediatrics, George Washington University School of Medicine and Health Sciences

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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