Symptoms & causes
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Overview
Alveolar soft part sarcoma is a very rare, slow growing, highly angiogenic (vessel-forming) tumor that can occur in any age group. It is most frequently found in young adults and teenagers and often begins in the lower extremities.
A recent analysis of the MD Anderson Cancer Center's institutional database, which may overrepresent incidence as a tertiary care center for rare tumors. estimated 90 total new cases of ASPS in the United States in 2004; it predicted that about half of the patients would fall between the ages of 15 and 29 years (Herzog 2005).
Alveolar soft part sarcoma is characterized by a translocation between the ASPL locus on chromosome 17 and the TFE3 locus on the X chromosome (der(17)t(X.17)(p1lq25))
Epidemiology
Most patients with alveolar soft part sarcoma probably have had the cancer for some time before they come to medical attention. The reason is that the tumor grows so slowly that it at first causes few symptoms and does not form a large mass.
By the time the tumor is big enough that the patient feels a lump from the primary lesion and seeks out a physician for help, the tumor has frequently spread, establishing small metastatic colonies throughout the body, frequently found in the lungs and even the brain.
It grows even more slowly than clear cell sarcoma, but is definitely a malignant tumor that tends to spread inexorably if not completely removed by surgery. Many patients can live with disease for years and even decades (Pappo, Parham et al. 1996). Although most patients with alveolar soft part sarcoma can never be rid of their cancer completely, many can undergo repeated surgery over the years to keep it somewhat at bay (Weis and Goldblum 2001).
Distinct Clinical Features
Alveolar soft part sarcoma gets the "alveolar" part of its name from the arrangement of cells seen under the microscope by the pathologist (Weis and Goldblum 2001).
Alveoli are a small air sacks deep within the lung where oxygen is absorbed into the body, and this cancer has an appearance similar to these air sacks. On gross pathological inspection, meaning on visual inspection without the aid of a microscope. of the tumor after it has been cut out, alveolar soft part sarcoma has numerous blood vessels. reflecting its angiogenic nature.
The increased blood flow can even cause an audible noise from blood rushing through the tumor-known in medical terms as a bruit (Pappo. Parham et al. 1996). They must be distinguished from vascular malformations (collections of blood vessels that have grown out of control but they are generally not malignant and will not spread like alveolar soft part sarcoma).
