Sarcomas

A sarcoma is a rare type of cancer that originates in the cells of connective tissue, which supports, protects, and structures other tissues and organs in the body. Connective tissue plays a crucial role in regulating cellular functions. Therefore, sarcomas can develop anywhere in the body, though they are most commonly found in the limbs, abdomen, and chest.

Based on their location, sarcomas are classified into two main types:

• Bone sarcomas: These originate in bone and cartilage tissues.
• Osteosarcoma: Arises from osteoblasts, the cells that form bones, in immature bone tissue. It grows rapidly and tends to spread to other parts of the body.
• Chondrosarcoma: Develops from cartilage-producing cells, the tissue that covers the ends of bones and joints. It typically grows slowly and is most commonly found in the pelvis, thigh, and shoulder.
• Ewing’s sarcoma: Forms in bones or nearby soft tissues. It most often appears in long bones and the pelvis. This is a very aggressive type of sarcoma, particularly affecting children.
• Chordoma: A rare tumor that arises in the bones of the spine, mainly in the sacrum and at the base of the skull.
• Soft tissue sarcomas: These originate in the tissues of muscles, tendons, fat, lymphatic and blood vessels, or nerves. The most common soft tissue sarcomas include:
• Angiosarcoma: Develops from the inner lining of a blood or lymphatic vessel.
• Fibrosarcoma: Originates in fibroblasts, the cells responsible for forming fibrous muscle tissues.
• Leiomyosarcoma: Forms in smooth muscle tissue.
• Rhabdomyosarcoma: Develops in skeletal or striated muscle tissue. It is more common in children.
• Liposarcoma: Arises in fat tissues.
• Malignant peripheral nerve sheath tumors: Originate in the cells surrounding peripheral nerves.
• Synovial sarcoma: Grows in the tissue surrounding joints.
• Gastrointestinal stromal tumor: Forms from the cells of the digestive tract.
• Kaposi’s sarcoma: Develops in blood and lymphatic vessels and forms tumor lesions on the skin, mucous membranes, lymph nodes, and other organs.
• Epithelioid sarcoma: Typically forms in tissues beneath the skin of the hands, arms, feet, or legs.
• Retroperitoneal and pelvic sarcoma: Develops in the back of the abdominal cavity and the pelvis.

Symptoms

Many sarcomas remain asymptomatic in their early stages and only present signs when they reach a significant size or invade nearby structures. Symptoms vary depending on the sarcoma’s location:

• A noticeable lump or swelling under the skin, which may be painful.
• Unexplained weight loss.
• Fatigue.
• Swelling and inflammation, especially in the limbs.
• Cough or breathing difficulties in cases of pulmonary sarcomas.
• Abdominal pain, vomiting, constipation, and intestinal bleeding in gastrointestinal tumors.
• Bone pain and unexplained fractures in bone sarcomas.

Causes

In general, tumors form due to genetic mutations in a cell’s DNA, disrupting its normal growth process and causing uncontrolled cell multiplication. The exact cause of connective tissue cell alterations is unknown, but research suggests a combination of environmental and hereditary factors, known as risk factors. However, most sarcomas occur without an apparent cause.

Risk Factors

Several factors may increase the likelihood of developing a sarcoma:

• Previous cancer treatments with radiation, chemotherapy, or stem cell transplants. These can lead to sarcoma formation in the treated area.
• Hereditary disorders, such as retinoblastoma, neurofibromatosis, Werner syndrome, Gorlin syndrome, tuberous sclerosis, or Paget’s disease.
• Chronic lymphatic system inflammation, which has been linked to angiosarcoma.
• Viral infections: Human herpesvirus 8 increases the risk of Kaposi’s sarcoma in immunocompromised individuals.
• Exposure to toxic chemicals, such as dioxins, herbicides, or vinyl chloride, which are risk factors for liver sarcomas.

Complications

The prognosis for sarcomas largely depends on size, location, and early detection. In localized sarcomas, survival rates are high. However, if the cancer is highly aggressive or detected late, metastasis can occur: cancer cells detach from the original tumor and spread through tissues, the bloodstream, or the lymphatic system, settling in other parts of the body. This significantly reduces the chances of a cure and may cause additional symptoms and damage in the affected areas.

Prevention

There are no specific preventive measures to avoid sarcoma development, aside from addressing risk factors:

• Avoid exposure to harmful chemicals.
• Protect yourself against infections if you have a weakened immune system by using masks, protective clothing, and maintaining strict hygiene.

Which Doctor Treats Sarcomas?

Sarcomas are diagnosed and treated by a multidisciplinary team, including medical oncologists, pathologists, radiation oncologists, and surgical oncologists.