Alveolar soft part sarcoma

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Alveolar soft part sarcoma
ICD-O: 9581/3

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Overview

Alveolar soft part sarcoma (ASPS) is a very rare type of Soft tissue sarcoma - a slow growing tumor of an unknown origin that arises mainly in children and young adults. ASPS is highly angiogenic: it involves intensive growth of new blood vessels, that connect the tumor to the blood system and enable dissemination of tumor cells into the bloodstream. Tumor cells can then easily migrate into other parts of the body - typically the lungs, and the brain.

ASPS is a sarcoma, and that indicates that this cancer initially arises in soft tissues that connect, support, or surround other structures and organs of the body, rather than bones. Typically, ASPS arises in muscles and deep soft tissue of the thigh or the leg (lower extremities), but can also appear in the upper extremities: the hands, the neck, and the head. While ASPS is a soft tissue sarcoma, it can spread and grow inside the bones.

The term alveolar comes from the microscopic pattern, visible during the analysis of slides of ASPS under the microscope in histopathology. The tumor cells seem to be arranged in the same pattern as the cells of the small air sacks (alveoli) in the lungs. However, this is just a structural similarity. ASPS was first described and characterized in 1952[1].

Very Rare

ASPS is an extremely rare cancer. While sarcomas comprise about 1% of all newly diagnosed cancers, and 15% of all childhood cancers, ASPS comprises less than 1% of sarcomas. According to the American Cancer Society, about 9530 new cases of soft tissue sarcoma will be diagnosed in the USA in 2006. This predicts under 100 new cases of ASPS. Such low numbers of occurrence seriously impede the search for a cure by making it hard to gather any meaningful statistics about the disease. As a result, finding the best treatment option often involves making a lot of educated guesses.

Primary Diagnosis

ASPS may exist in the patient’s body for a long time before being diagnosed. It can grow large and push aside surrounding tissues for a long time before causing any discomfort. Therefore, ASPS symptoms may either be a painless swelling, or a soreness caused by compressed nerves or muscles, affecting the range of motion in the area.

What causes ASPS

Chromosomal analysis of ASPS shows the breaking and joining of two chromosomes in the tumor cells. A piece of chromosome X breaks and is joined to chromosome 17. This translocation creates a fusion between two genes named ASPL and TFE3, which results in the formation of an aberrant protein (termed fusion protein) that is not found in normal cells. Two sorts of fusions between chromosome X and chromosome 17 are found in different ASPS tumors: Type one, and type two. Dr. Ladanyi at Memorial Sloan-Kettering Cancer Center, in New York, has pioneered this work.

Prognosis

Although ASPS displays a relativly indolent course, the ultimate prognosis is poor and is often characterized by late metastases.

Notes

  1. Christopherson WM, Foote FW, Stewart FW: Alveolar soft part sarcomas: structurally characteristic tumors of uncertain histogenesis. Cancer 1952, 5:100-111

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