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.^{[16, 17]}No specific causative environmental factors have been identified because of the rarity of these tumors.

Differential Diagnoses

Marcus KC, Grier HE, Shamberger RC, Gebhardt MC, Perez-Atayde A, Silver B. Childhood soft tissue sarcoma: a 20-year experience. J Pediatr. 1997 Oct. 131(4):603-7. [QxMD MEDLINE Link].
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. 1999 Dec. 17(12):3697-705. [QxMD MEDLINE Link].
Shmookler BM, Enzinger FM. Liposarcoma occurring in children. An analysis of 17 cases and review of the literature. Cancer. 1983 Aug 1. 52(3):567-74. [QxMD MEDLINE Link].
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. 2007 Jul 15. 121(2):308-15. [QxMD MEDLINE Link].
Somaiah N, Beird HC, Barbo A, Song J, Mills Shaw KR, Wang WL, et al. Targeted next generation sequencing of well-differentiated/dedifferentiated liposarcoma reveals novel gene amplifications and mutations. Oncotarget. 2018 Apr 13. 9 (28):19891-19899. [QxMD MEDLINE Link]. [Full Text].
Crozat A, Aman P, Mandahl N, Ron D. Fusion of CHOP to a novel RNA-binding protein in human myxoid liposarcoma. Nature. 1993 Jun 17. 363(6430):640-4. [QxMD MEDLINE Link].
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. 2007 Jun 15. 109(12):2522-31. [QxMD MEDLINE Link].
de Vreeze RS, de Jong D, Nederlof PM, et al. Added Value of Molecular Biological Analysis in Diagnosis and Clinical Management of Liposarcoma: A 30-Year Single-Institution Experience. Ann Surg Oncol. 2010 Mar. 17(3):686-93. [QxMD MEDLINE Link].
Castleberry RP, Kelly DR, Wilson ER, et al. Childhood liposarcoma. Report of a case and review of the literature. Cancer. 1984 Aug 1. 54(3):579-84. [QxMD MEDLINE Link].
Ferrari A, Casanova M, Spreafico F, et al. Childhood liposarcoma: a single-institutional twenty-year experience. Pediatr Hematol Oncol. 1999 Sep-Oct. 16(5):415-21. [QxMD MEDLINE Link].
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. 1993 Nov 15. 72(10):3114-9. [QxMD MEDLINE Link].
Alaggio R, Coffin CM, Weiss SW, et al. Liposarcomas in young patients: a study of 82 cases occurring in patients younger than 22 years of age. Am J Surg Pathol. 2009 May. 33(5):645-58. [QxMD MEDLINE Link].
Gronchi A, Casali PG, Fiore M, et al. Retroperitoneal soft tissue sarcomas: patterns of recurrence in 167 patients treated at a single institution. Cancer. 2004 Jun 1. 100(11):2448-55. [QxMD MEDLINE Link].
Cheng EY, Springfield DS, Mankin HJ. Frequent incidence of extrapulmonary sites of initial metastasis in patients with liposarcoma. Cancer. 1995 Mar 1. 75(5):1120-7. [QxMD MEDLINE Link].
Schwab JH, Boland PJ, Antonescu C, Bilsky MH, Healey JH. Spinal metastases from myxoid liposarcoma warrant screening with magnetic resonance imaging. Cancer. 2007 Oct 15. 110(8):1815-22. [QxMD MEDLINE Link].
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. 2000 Aug. 2(3):132-8. [QxMD MEDLINE Link]. [Full Text].
Tornin J, Hermida-Prado F, Padda RS, Gonzalez MV, Alvarez-Fernandez C, Rey V, et al. FUS-CHOP Promotes Invasion in Myxoid Liposarcoma through a SRC/FAK/RHO/ROCK-Dependent Pathway. Neoplasia. 2018 Jan. 20 (1):44-56. [QxMD MEDLINE Link]. [Full Text].
Gimber LH, Montgomery EA, Morris CD, Krupinski EA, Fayad LM. MRI characteristics associated with high-grade myxoid liposarcoma. Clin Radiol. 2017 Jul. 72 (7):613.e1-613.e6. [QxMD MEDLINE Link].
Song T, Shen J, Liang BL, Mai WW, Li Y, Guo HC. Retroperitoneal liposarcoma: MR characteristics and pathological correlative analysis. Abdom Imaging. 2007 Sep-Oct. 32(5):668-74. [QxMD MEDLINE Link].
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. 2006 Nov. 33(11):1290-5. [QxMD MEDLINE Link].
Yang J, Codreanu I, Servaes S, Zhuang H. Earlier detection of bone metastases from pleomorphic liposarcoma in a pediatric patient by FDG PET/CT than planar 99mTc MDP bone scan. Clin Nucl Med. 2012 May. 37(5):e104-7. [QxMD MEDLINE Link].
Enzinger FM, Weiss SW. Liposarcoma. Enzinger F, Weiss S, eds. Soft Tissue Tumors. St Louis, MO: Mosby; 1995. 431-66.
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. 2005 Nov 1. 104(9):2006-12. [QxMD MEDLINE Link].
Demetri GD, von Mehren M, Jones RL, et al. Efficacy and Safety of Trabectedin or Dacarbazine for Metastatic Liposarcoma or Leiomyosarcoma After Failure of Conventional Chemotherapy: Results of a Phase III Randomized Multicenter Clinical Trial. J Clin Oncol. 2016 Mar 10. Vol 34:786-93. [QxMD MEDLINE Link]. [Full Text].
Baruchel S, Pappo A, Krailo M, et al. A phase 2 trial of trabectedin in children with recurrent rhabdomyosarcoma, Ewing sarcoma and non-rhabdomyosarcoma soft tissue sarcomas: a report from the Children's Oncology Group. Eur J Cancer. 2012 Mar. Vol 48:579-85. [QxMD MEDLINE Link]. [Full Text].
Bill KL, Casadei L, Prudner BC, et al. Liposarcoma: molecular targets and therapeutic implications. Cell Mol Life Sci. 2016 Oct. Vol 73:3711-8. [QxMD MEDLINE Link]. [Full Text].
Raaf JH, Ragsdale BD. Surgical management of liposarcoma. Bogumil GP, Fleegler EJ, eds. Tumors of the Hand and Upper Limb. 1993.
Jones RL, Fisher C, Al-Muderis O, Judson IR. Differential sensitivity of liposarcoma subtypes to chemotherapy. Eur J Cancer. 2005 Dec. 41(18):2853-60. [QxMD MEDLINE Link].
Demetri GD, Chawla SP, von Mehren M, et al. Efficacy and safety of trabectedin in patients with advanced or metastatic liposarcoma or leiomyosarcoma after failure of prior anthracyclines and ifosfamide: results of a randomized phase II study of two different schedules. J Clin Oncol. 2009 Sep 1. 27(25):4188-96. [QxMD MEDLINE Link].
Zijoo R, von Mehren M. Efficacy of trabectedin for the treatment of liposarcoma. Expert Opin Pharmacother. 2016 Oct. 17 (14):1953-62. [QxMD MEDLINE Link].
Mahmood ST, Agresta S, Vigil C, et al. Phase II study of sunitinib malate, a multi-targeted tyrosine kinase inhibitor in patients with relapsed or refractory soft tissue sarcomas. Focus on 3 prevalent histologies: Leiomyosarcoma, liposarcoma, and malignant fibrous histiocytoma. Int J Cancer. 2010 Dec 10. [QxMD MEDLINE Link].
Brandal P, Bjerkehagen B, Heim S. Rearrangement of chromosomal region 8q11-13 in lipomatous tumours: correlation with lipoblastoma morphology. J Pathol. 2006 Feb. 208(3):388-94. [QxMD MEDLINE Link].

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Author

Alexander Gozman, MD Assistant Professor, Department of Pediatrics, Division of Hematology/Oncology, Albany Medical Center

Alexander Gozman, MD is a member of the following medical societies: American Medical Association, American Society of Clinical Oncology, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group

Disclosure: Nothing to disclose.

Coauthor(s)

Anastasios K Konstantakos, MD Clinical Associate Surgeon, Department of Cardiovascular Surgery, Billings Clinic

Disclosure: Nothing to disclose.

Vikramjit S Kanwar, MBBS, MBA, MRCP(UK) Professor Emeritus of Pediatrics, Albany Medical College; Chief of Pediatric Oncology, Homi Bhabha Cancer Hospital, Varanasi, India

Vikramjit S Kanwar, MBBS, MBA, MRCP(UK) is a member of the following medical societies: Children's Oncology Group, Indian Academy of Pediatrics, International Society of Pediatric Oncology

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Steven K Bergstrom, MD 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, Children's Oncology Group, American Society of Clinical Oncology, International Society for Experimental Hematology, American Society of Hematology, American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA Executive Vice President, Chief Medical and Academic Officer, Renown Heath

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American College of Healthcare Executives, American Society of Pediatric Hematology/Oncology, Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

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 School of Medicine

Stephan A Grupp, MD, PhD is a member of the following medical societies: American Association for Cancer Research, Society for Pediatric Research, American Society for Blood and Marrow Transplantation, American Society of Hematology, American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Sections Pediatric Liposarcoma
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Pediatric Liposarcoma Clinical Presentation

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