Alveolar Soft Part Sarcoma

Alveolar Soft Part Sarcoma is a rare, slow growing, cancerous tumor that most commonly occurs in the deep soft-tissues of the thigh. It is treated with surgical excision, or removal, and systemic treatments.

66B) Coronal T1 MRI Of Lower Leg Alveolar Soft Parts Sarcoma
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What is Alveolar Soft Part Sarcoma?

Alveolar Soft Part Sarcoma is a rare, malignant (cancerous) soft-tissue tumor that can affect fats, muscles, nerves, tendons, blood vessels, and lymph vessels. It most commonly affects adolescents and young adults. Early treatment is necessary because it can spread, or metastasize, to other parts of the body including the lungs, liver, brain, or bone. Treatment commonly consists of systemic and local treatment, such as surgical excision of the tumor.

Who is usually affected?
Who is usually affected?
  • • Affects adolescents and young adults aged 15-35 years old.
  • • Slightly more common in females than males.
  • • Rare- less than 1% of soft tissue sarcomas.
Causes
Causes
  • • This condition is characterized by a genetic translocation t(x;17)(p11;q25) and becomes chimeric ASPSCR1-transcription factor E3 (TFE3). It is not passed down to children (non hereditary).
Signs and Symptoms
Signs and Symptoms
  • • Signs and symptoms include a growing, painless, and palpable mass.
Biological Behavior
Biological Behavior
  • • This condition has been linked to a genetic component, called a chromosomal translocation.
Common Sites Involved
Common Sites Involved
  • Soft-tissues of the:
  • thigh (most common site)
  • • buttock
  • • head
  • • neck
  • • Can metastasize to lungs, liver, brain, and bone.
Diagnosis
Diagnosis
  • • The work-up often consists of a physical examination, MRI, and CT scans. Also, a bone scan, CT PET scan, and CT chest/abdomen/pelvis may be ordered to check if the tumor has spread, or metastasized, to the chest/abdomen/ pelvis or other parts of the body. The diagnosis is often confirmed with a biopsy, which samples the tumor for further analysis.

Risk to your limbs

Alveolar Soft Part Sarcoma is a rare, cancerous soft-tissue sarcoma. Soft-tissue sarcomas, such as this condition, grow in the soft-tissues of the extremities and compromise or destroy the affected soft-tissue and muscles. These tumors can compress or stretch important vessels and nerves, and occasionally wrap around these structures making it difficult to remove the mass without an amputation. They also have the potential to invade adjacent bones. Without treatment, this condition can metastasize or spread throughout the body, with the lungs, liver, and bones being the most common sites.

Radiographic imaging is used to help form a diagnosis. These include MRI, CT and Bone Scans

An example of an MRI is shown.

Treatment of Alveolar Soft Part Sarcoma

The treatment for Alveolar Soft Part Sarcoma includes limb-sparing surgery to remove the mass with a wide or radical resection. Systemic treatments, such as chemotherapy and radiation, may also be used to treat this condition, especially if the cancer has metastasized, or spread to other parts of the body.

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Hey! I am Dr. Wittig.

Myself and my amazing team are dedicated to saving your life and your limb. Losing a limb because of a tumor can be a terrifying experience. But, it does not have to be the only option. I’ve spent 20+ years as a Board-Certified Orthopedic Surgeon and Orthopedic Oncologist.

I’ve devoted my career to helping children and adults afflicted with bone and soft tissue masses by performing complex limb saving surgeries. Most patients can have their limb saved, which may require innovative techniques.

Patients afflicted with musculoskeletal tumors have complex conditions that are best taken care of at large hospitals. I am the Chairman of Orthopedics and Chief of Orthopedic Oncology at Morristown Medical Center. My philosophy is a multidisciplinary team approach, working together to tailor treatment to individual patients. Education and research are essential to my practice, providing the best setting for extraordinary patient care. Because of this, we have some of the top results in the country.