Pediatric Liposarcoma Clinical Presentation

Updated: Jul 09, 2013
  • Author: Alexander Gozman, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
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Presentation

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.

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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.

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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. [15] No specific causative environmental factors have been identified because of the rarity of these tumors.

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