eMedicine Specialties > Pediatrics: General Medicine > Oncology

Liposarcoma: Follow-up

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

Follow-up

Further Inpatient Care

  • Consideration for adjuvant therapy for liposarcoma should be based on the degree of residual disease left behind.
  • If microscopic disease remains following surgery, postoperative radiation therapy should be administered. External beam radiation doses can range from 4000-6500 centigray, with dosing to be determined in consultation with a radiation oncologist. In children, long-term effects of radiotherapy, such as skeletal and soft tissue deformation, effects on growth, and risk of neoplastic transformation, should be weighed against the child's current physiologic status and potential for remission from liposarcoma.
  • If macroscopic disease remains following surgery, radiotherapy, chemotherapy, and second-look surgery should all be considered.

Prognosis

  • Prognosis depends on the stage, histologic subtype or grade, anatomic location of the tumor, tumor size, and the overall treatment regimen used.
  • Five-year survival rates range widely vary, depending on histologic subtype.
  • Based on histologic characteristics alone, myxoid lesions, which tend to occur commonly in children, have 5-year survival rates approaching 80%.
  • In contrast, rare, highly-aggressive pleomorphic lesions have 5-year survival rates around 20%
  • With any histologic subtype, local recurrence is common and is related to the completeness of surgical excision. If metastases occur, they typically involve the lungs, but unusual extrapulmonary soft tissue sites such as retroperitoneum or chest wall may be involved.

Miscellaneous

Medicolegal Pitfalls

  • Although liposarcoma is an extremely rare lesion in the pediatric population, considering liposarcoma in the differential diagnosis of lipoma is important. Careful pathologic analysis of all excised specimens that clinically resemble lipoma is essential to avoid misdiagnosing a potentially lethal condition.
  • Lipoblastomatosis and liposarcoma should be differentiated in younger children to avoid unnecessarily mutilating surgery.
  • The surgeon needs to place and orient the biopsy incision in a location that allows subsequent curative resection of the liposarcoma.
 


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, 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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