Are Soft Tissue Sarcomas Vascular?

A sarcoma (malignant tumor) is a type of cancer that originates in the bones or muscles.

Two types of sarcomas include:

1. Bone sarcoma: Such as osteosarcomas, Ewing family of tumors, and rhabdomyosarcoma
2. Soft tissue sarcoma: Develops in connective tissue such as the fats, muscles, tendons, ligaments, nerves, fibrous tissues, blood vessels, or deep skin tissues

Soft tissue sarcomas can be found in any part of the body. Although most of them start in the arms or legs, they can be found in the trunk, head, and neck area; internal organs, and area in the back of the abdominal cavity.

There are more than 50 types of soft tissue sarcomas, a few are very common and some of them are quite rare.

Sarcomas are classified based on the location of their origin:

1. Leiomyosarcoma: Originates in the smooth involuntary muscles of the womb, digestive system, and blood vessels
2. Gastrointestinal stromal tumor: Originates in the gastrointestinal tract
3. Liposarcoma: Soft tissue sarcoma in fatty tissues or adipose tissues
4. Malignant schwannoma or malignant peripheral nerve sheath tumors: Originate in the peripheral nervous system
5. Angiosarcoma, hemangioendothelioma, hemangiopericytoma, and solitary fibrous tumor: Originates in the blood vessels
6. Fibrosarcoma, dermatofibrosarcoma, low-grade fibromyxoid sarcoma, and fibromatosis: Sarcomas of the connective tissues
7. Alveolar soft part sarcoma: Rare type that commonly starts in the legs
8. Clear cell sarcoma: Originates in the tendons of the arms or legs
9. Desmoplastic small round cell tumor: Rare sarcoma of the abdomen
10. Epithelioid sarcoma: Originates in the tissues under the skin of the hands, forearms, feet, or lower legs
11. Kaposi sarcoma: Originates in the cells lining the lymph or blood vessels
12. Synovial sarcoma: Malignant tumor of the tissue around joints
13. Undifferentiated pleomorphic sarcoma: Most often found in the arms or legs

Six risk factors for soft tissue sarcoma include:

1. Genetic mutations
2. Exposure to chemicals such as herbicides, arsenic, dioxin or thorium dioxide, and vinyl chloride
3. Radiation exposure (history of radiation treatment for other types of cancer)
4. Lymphedema in the arms or legs for a long time
5. Human immunodeficiency virus and human herpesvirus 8 infections
6. Certain inherited syndromes such as hereditary retinoblastoma, Li-Fraumeni syndrome, familial adenomatous polyposis, von Recklinghausen disease (neurofibromatosis), tuberous sclerosis, Gardner syndrome, Nevoid basal cell carcinoma syndrome, and Werner syndrome

In the early stages, a soft tissue sarcoma may not cause any signs and symptoms.

As the tumor grows, it may present as:

• A noticeable painless lump or swelling under the skin, often on an arm or a leg
• Pressure on nearby organs, nerves, muscles, or blood vessels causing:
  • Pain
  • Bleeding
  • Difficulty breathing

Knowing the type of tumor helps predict the tumor response to specific treatments and allows to personalize the treatment.

To confirm the diagnosis, the doctor may recommend the following:

• Imaging tests: The area of concern can be evaluated by
  • X-rays
  • Computed tomography scans
  • Magnetic resonance imaging
  • Positron emission tomography
• Biopsy: A sample of tissue is taken from the body to examine it closely.
  • Core needle biopsy: Samples from several sections of the tumor are obtained to be analyzed in the laboratory.
  • Surgical or incisional biopsy: Surgical removal of a larger sample of tissue or removal of a small tumor entirely.

Depending on the size, type, location, and aggressiveness of the tumor, the treatment may comprise:

• Surgical removal: The most common treatment that involves removing cancer and some surrounding healthy tissue
• Radiation therapy: Involves treating cancer with high-powered beams of energy
• Chemotherapy: A drug treatment that uses chemicals to kill cancer cells
• Targeted drug therapy: For some cases of soft tissue sarcoma that have specific characteristics in their cells can be attacked by targeted drug treatments