Liposarcoma overview
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Liposarcoma overview On the Web |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Alejandro Lemor, M.D. [2]; Ammu Susheela, M.D. [3]
Overview
Liposarcoma is a uncommon cancer of connective tissues. Liposarcoma is a malignant tumor that arises in fat cells in deep soft tissue, such as that inside the thigh or in the retroperitoneum.
They are typically large bulky tumors which tend to have multiple smaller satellites extending beyond the main confines of the tumor.
Historical Perspective
Liposarcoma was first described by Dr. Rudolph Virchow, a German pathologist, in 1857. Virchow reported a "myxoma lipomatoides malignum", highlighting the malignant nature of the tumor. Between 1954 and 1979, several authors reported cases of liposarcoma and suggested that liposarcoma should be classified according to histopathological analysis into well-differentiated, myxoid, and dedifferentiated subtypes.
Classification
Liposarcoma may be classified into well-differentiated, de-differentiated, myxoid, round cell, or pleomorphic liposarcoma.
Pathophysiology
The pathogenesis of liposarcoma depends on the histological subtype. The role of proto-oncogenes has been implicated in the development of well-differentiated liposarcoma
Causes
There are no established causes for liposarcoma.
Differential Diagnosis
Liposarcoma must be differentiated from other diseases that cause a painless, growing mass such as spindle cell lipoma, neurofibroma, dermatofibrosarcoma protuberans, and malignant peripheral nerve sheath tumor.
Epidemiology and Demographics
Liposarcoma is the second most common type of all soft tissue sarcomas in adults. The annual incidence is 0.25 cases per 100,000 individuals. Men are slightly more affected with liposarcoma than women. It is most frequent in middle-aged and older adults.
Risk Factors
Common risk factors in the development of liposarcoma include chemical carcinogens, radiation, immunodeficiency, genetic disorders, and viral infections.
Natural History, Complications and Prognosis
Common complication of liposarcoma include metastasis. Mass may compress adjacent structures causing different clinical manifestations. Metastasis is the most important aspect to assess in the prognosis of liposarcoma. The prognosis will depend on the histopathological subtype, being the well differentiated liposarcoma the subtype with the best prognosis, and the pleomorphic liposarcoma with the worst prognosis.
Diagnosis
Staging
The staging of liposarcoma is based on the TNM classification, which includes primary tumor spread, lymph node involvement, and presence of metastasis.
History and Symptoms
Liposarcoma usually presents as a painless mass. The most common location of a liposarcoma is the lower extremities. Patients with retroperitoneal liposarcoma remain asymptomatic until the mass invades adjacent structures, which may cause pain or obstructive symptoms.
Physical Examination
Physical examination findings of liposarcoma depend on the location of the tumor. Since most liposarcomas are located in the lower extremities, liposarcoma is commonly associated with the findings of palpable firm nontender mass in one lower extremity. Physical examination of retroperitoneal liposarcomas is usually unremarkable, but other pertinent findings on physical examination of retroperitoneal liposarcomas include palpation of an abdominal mass, abdominal distension, and tenderness.