What is Alveolar Soft Part Sarcoma (ASPS)?
Alveolar Soft Part Sarcoma (ASPS) is a rare type of cancer that develops in the soft tissues of the body such as muscles, tendons, and or fat tissues. It is a slow-growing tumour but tends to spread to other parts of the body as soft tissue sarcomas have a rich blood supply which helps the cancer cells grow rapidly.
It can occur anywhere in the body, but commonly develops in the thigh, buttocks and lower legs. In rare cases, it can also develop in the head, neck, chest, abdomen and eyes. In children, it is often developed in the head and neck region.
It is a rare tumour and accounts for 0.5-1% of all soft tissue sarcomas. It typically affects young adults aged 15-35 years and is more common in females.
Disease Causes
There is no well-known cause, however, scientists believe that genetic mutations (false alterations in the genes) may play a role in the development of alveolar soft part sarcoma but the specific risk factors are still unclear. Alveolar soft part sarcoma is not inherited and occurs sporadically.
Signs and Symptoms
The tumour grows slowly, so symptoms may not appear until it is quite large.
Common symptoms include:
- A painless lump or swelling.
- Pain or discomfort if the tumour presses on nearby structures.
- Symptoms from metastases, such as shortness of breath (if it spreads to the lungs).
Diagnosis
A multistep diagnosis is needed to diagnose alveolar soft part sarcoma.
- Imaging Tests: Imaging techniques such as CT-Scan or MRI are used to detect the site, size, and blood supply.
- Ultrasound: It helps to detect the site, size, and extent of the tumour along with the large blood vessels supplying the tumour mass.
- Biopsy: A small tissue sample is taken from the tumour for analysis under a microscope.
- Immunohistochemistry: It is a special test to rule out other types of tumours.
Treatment
Surgery: Surgical removal of the tumour is the main treatment option. Removal at earlier stages reduces the risks of recurrence.
Radiation Therapy: It is used in combination with surgery to kill any remaining cancer cells and reduce the chance of recurrence.
Chemotherapy: chemotherapy is used in cases where the tumour has spread to the other parts of the body, but its effectiveness in the case of alveolar soft part sarcoma is limited.
Prognosis (Disease Outcome)
- Early detection and complete surgical removal of the tumour increases the chances of long-term survival.
- In adult patients, the alveolar soft part sarcoma shows poor prognosis (disease outcome) due to the higher chances of spreading the tumour to the lungs, brain, and bones.
- Regular follow-ups are necessary to monitor for recurrence or metastases.
- If you feel you have a lump or mass, see your doctor right away. Results can be improved with early therapy.
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