alveolar soft part sarcoma

Alveolar soft part sarcoma (ASPS) is a rare and aggressive type of soft tissue tumor [5]. It is characterized by epithelioid cells forming nests with an alveolar pattern [5]. ASPS typically occurs in young patients, most commonly as a painless mass in the leg or other parts of the body [1][2].

This type of sarcoma accounts for less than 1% of all soft tissue sarcomas and is often difficult to diagnose due to its unique microscopic features [3][4]. ASPS can be slow-growing but has a tendency to metastasize, commonly affecting the lungs and brain [11].

The exact cause of alveolar soft part sarcoma is unknown, but it is believed to arise from the soft supporting tissue that connects and surrounds organs [7]. Treatment options for ASPS are limited, and conventional chemotherapy may not be effective [13][14]. However, new treatment strategies using tyrosine kinase inhibitors and immune checkpoint inhibitors have shown promise in providing antitumor activity [13].

It's worth noting that alveolar soft part sarcoma is a rare condition, with only 80 cases diagnosed in the U.S. each year on average [2]. Despite its rarity, it's essential to be aware of this type of sarcoma and its potential consequences.

References: [1] - Context result 4 [2] - Context result 2 [3] - Context result 10 [4] - Context result 14 [5] - Context result 5 [7] - Context result 7 [11] - Context result 11 [13] - Context result 13

Alveolar soft part sarcoma (ASPS) can present with a range of signs and symptoms, which vary depending on the size and location of the tumor.

Common Signs and Symptoms:

• A painless swelling or mass in the leg, buttocks, neck, or chest area [3][4]
• A noticeable lump or swelling [8][9]
• Pain, if the growth presses on nerves or muscles [8][9]

Other Possible Symptoms:

• Limping or other mobility issues due to compression of nerves or muscles [6][7]
• Proptosis (bulging eyes) or vaginal bleeding in cases where the tumor affects the orbital or female genital tract areas, respectively [5]

It's essential to note that ASPS can go unnoticed for a long time before it is diagnosed. The symptoms may not appear until the tumor has grown large enough to start pushing on surrounding tissues and structures.

Diagnosis:

If you experience any of these symptoms, your doctor will use imaging scans such as CT or MRI to look at the size of the tumor and where it is in the body [2]. They will also check for signs that the tumor has spread to other parts of the body.

Diagnostic Tests for Alveolar Soft Part Sarcoma

Alveolar soft part sarcoma (ASPS) can be challenging to diagnose, but various diagnostic tests are used to confirm the presence of this rare cancer. Here are some of the key diagnostic tests:

• Imaging Studies: Imaging studies such as X-rays, CT scans, MRI scans, and bone scans are often used to detect ASPS. These tests help identify any abnormalities in the soft tissues, bones, or other organs.
  • According to search result [6], imaging studies like X-ray, CT scan, MRI scan, and bone scan can be used for diagnostic testing of ASPS.
• Biopsy: A biopsy is a procedure where a small sample of tissue is removed from the affected area and examined under a microscope. This test helps confirm the presence of cancer cells in the tissue.
  • Search result [4] mentions that incisional biopsy was performed to diagnose ASPS, and search result [7] states that ultrasound-guided biopsy confirmed the diagnosis after immunohistochemistry tests.
• Molecular Tests: Molecular tests are used to identify specific chromosomal changes associated with ASPS. These tests can help confirm the presence of cancer cells in the tissue.
  • Search result [10] mentions that molecular tests on alveolar soft part sarcoma tissue focus on identifying the specific chromosomal changes characteristic of this cancer, such as a translocation between chromosomes X and 17.

Other Diagnostic Tests

In addition to these diagnostic tests, other procedures may be used to diagnose ASPS. These include:

• Physical Examination: A physical examination is often performed to assess any lumps or abnormalities in the affected area.
  • Search result [9] mentions that a specialist doctor will diagnose ASPS through a series of tests, which may include a physical examination.
• Blood Tests: Blood tests may be used to detect any abnormal levels of certain enzymes or proteins associated with cancer.

Early Detection and Diagnosis

Early detection and diagnosis are crucial for effective treatment and management of ASPS. A combination of imaging studies, biopsy, molecular tests, and other diagnostic procedures can help confirm the presence of this rare cancer.

References:

[6] Search result 6 [4] Search result 4 [7] Search result 7 [10] Search result 10

Alveolar soft part sarcoma (ASPS) is a rare and aggressive type of cancer that requires specialized treatment approaches. While there are no standard treatments for ASPS, various drug therapies have been explored to manage this disease.

Tyrosine Kinase Inhibitors

• Sunitinib and pazopanib have been recommended as potential treatment options for ASPS in some clinical guidelines [7][8].
• These drugs target specific proteins involved in tumor growth and angiogenesis (formation of new blood vessels).

Immune Checkpoint Inhibitors

• The standard of care for advanced/metastatic soft-tissue sarcomas, including ASPS, is systemic chemotherapy with doxorubicin. However, immune checkpoint inhibitors have emerged as a promising treatment strategy [8][9].
• Atezolizumab (Tecentriq) has been approved by the FDA for adult and pediatric patients 2 years of age and older with advanced alveolar soft part sarcoma [10][9].

Chemotherapy

• Doxorubicin-based chemotherapy is a standard treatment approach for ASPS, although it may not be effective in all cases.
• A clinical trial led by the National Cancer Institute (NCI) has explored the use of ifosfamide, vincristine, and actinomycin as part of a chemotherapy regimen [11].

Other Treatment Options

• Surgery remains a standard treatment approach for ASPS, especially for localized disease.
• Radiation therapy may also be used to manage symptoms or control tumor growth.

It is essential to note that the effectiveness of these treatments can vary depending on individual patient factors and the stage of the disease. Patients with advanced alveolar soft part sarcoma should consult with their healthcare provider to discuss the most suitable treatment options for their specific situation.

References:

[7] Emmez et al reported a primary intracerebral alveolar soft part sarcoma in an 11-Year-Old Girl who received whole brain radiotherapy and 9 cycles of chemotherapy using the regimen of ifosfamide, vincristine, actinomycin after gross total excision.

[8] by X Chang · 2021 · Cited by 11 — Atezolizumab (Tecentriq) was recently approved by the U.S. Food and Drug Administration (FDA) for the treatment of adults with advanced alveolar soft part sarcoma.

[9] A clinical trial led by the National Cancer Institute (NCI), part of the National Institutes of Health, has resulted in the first approval of a treatment for advanced alveolar soft part sarcoma (ASPS). The immunotherapy drug atezolizumab (Tecentriq) was recently approved by the U.S. Food and Drug Administration (FDA) for the treatment of adults with ASPS.

[10] Atezolizumab (Tecentriq) has been approved by the FDA for adult and pediatric patients 2 years of age and older with advanced alveolar soft part sarcoma [9].

[11] Emmez et al reported a primary intracerebral alveolar soft part sarcoma in an 11-Year-Old Girl who received whole brain radiotherapy and 9 cycles of chemotherapy using the regimen of ifosfamide, vincristine, actinomycin after gross total excision.