What is Sarcoma?
Sarcomas consist of abnormal cells that grow uncontrollably to form malignant (cancerous) tumors that begin in bone or the soft tissues that surround and support your body. Since bone, cartilage, and soft tissue including tendons, muscle, joints, nerves, blood vessels, lymph vessels, and fat cells run throughout the body, sarcomas can occur anywhere. Both adults and children can develop sarcomas. Though there are over 75 types of sarcoma, less than one percent of all patients with cancer have sarcoma.
Sarcomas grow and spread differently than other cancers. Sarcomas of the bone arise primarily from bone, as opposed to spreading to bone from other parts of the body. Sarcomas tend to spread to the lungs, and they can spread to other bones and to the liver, but sarcomas rarely spread to lymph nodes. Sarcomas grow into ball-like masses that can push against adjacent parts of the body, such as arteries, nerves, and veins to compress adjoining muscles. The local ball-like growth of a sarcoma enables surgical removal in most instances.
Mount Sinai Health System treats all forms of sarcomas: bone and soft tissue, adult and pediatric, according to the stage of the tumor. Staging is a way of describing if the cancer has spread, and if it is affecting other parts of the body. Generally, staging assesses tumor size, its grade, if it is superficial or deep, if it is confined, or if it has spread. High-grade bone or soft tissue sarcomas are aggressive and considered stage II tumors. Stage IV tumors refer to those tumors of any size that have spread to other parts of the body at the time the tumor is discovered. Stage I tumors are early stage tumors that have the best prognosis, making early diagnosis important.
Soft Tissue Sarcomas: Soft tissue sarcomas are malignant tumors that grow in and resemble fat, muscle, or nerve cells, blood vessels, and connective joint tissue.
The first noticeable sign of soft-tissue sarcomas (STS) in the legs, arms, or torso(where most soft-tissue sarcomas start) is usually a painless lump or swelling under the skin. Most lumps are harmless (benign or non-cancerous), but those that are growing require immediate medical attention. STS that begin in other parts of the body, like the abdomen, may not cause symptoms until they become quite large and start pressing on nearby organs. Symptoms of abdominal or chest sarcomas can include pain and difficulty breathing. These symptoms can be caused by other conditions, as well, and requires appropriate diagnosis.
Each type of soft tissue sarcoma has specific characteristics based on its location in the body. Mount Sinai treats all types of soft tissue sarcomas, including, but not limited to the following:
- Gastrointestinal stromal tumor (GIST) occurs in the gastrointestinal tract.
- Leiomyosarcoma occurs in smooth muscle.
- Liposarcoma occurs in fatty or adipose tissue.
- Undifferentiated pleomorphic sarcoma (UPS/MFH)/malignant fibrous histiocytoma is a rare childhood cancer.
- Synovial sarcoma may originate in more than one type of body tissue.
- Desmoid tumors/deep fibromatosis occurs in deep connective tissue.
- Angiosarcoma occurs in the lining of blood vessels.
- Rhabdomyosarcoma occurs in muscles attached to bones, and mostly affects children.
Bone Sarcomas: Bone sarcomas are malignant tumors that grow in bones and resemble bone tissue. Early symptoms of bone sarcoma include pain and swelling that comes and goes at first in the area of the tumor. Advanced bone cancer can result in a fracture (break) in the bone where the tumor is located. While a bone tumor can be any abnormal growth from the bone or in the bone, either benign or malignant, bone cancer refers to a malignant bone tumor. Bone cancer can be a primary malignant tumor, like an osteosarcoma, Ewing's sarcoma, or chondrosarcoma that originated in the bone. Bone cancer can also be a metastatic carcinoma, such as breast cancer, lung cancer, prostate cancer, kidney cancer, or thyroid cancer that began in one of those organs and spread to the bone. It is important to differentiate between the various types of cancers that affect the bone, because each has its own type of treatment and prognosis. Mount Sinai treats all types of bone tissue sarcomas, including, but not limited to the following:
- Osteosarcoma occurs most often in the knee and upper arm.
- Chondrosarcoma occurs most often in the pelvis located between the hip bones, upper leg, and shoulder and contains cancerous bone cells.
Ewing Sarcoma: Ewing sarcoma is unusual because while it most often occurs in the bone, it may also occur in soft tissue, typically appearing along the backbone, pelvis, and in the arms and legs.
Chest Wall Sarcomas: The chest wall is the bone-and-tissue framework—including the spine, sternum, and ribs—that forms a “cage” around vital organs such as the heart and lungs. Many types of tumors can grow within the chest wall. Some are primary tumors, which originate in the chest wall and can be either benign or malignant. Some are secondary tumors, which metastasize to the chest wall from other parts of the body, are often malignant.
Malignant chest wall tumors include many types of sarcoma. Chest wall sarcoma is a category of cancerous tumor that can form in bones, soft tissues, and cartilage anywhere in the body. Symptoms of chest wall sarcomas vary with the tumor’s classification and severity, and could include difficulty breathing as well as pain and swelling surrounding the tumor.
The following types of bone and soft tissue sarcomas can grow in the chest wall:
- Chondrosarcoma forms in cartilage, and is the most common type of primary chest wall bone cancer. It usually presents as a slowly enlarging painful mass.
- Osteosarcoma forms in bone, typically during a period of accelerated growth, such as adolescence. It typically presents as a rapidly enlarging painful mass.
- Ewing’s sarcoma forms in bone and most often affects children and young adults. It usually presents as a painful mass with systemic symptoms such as fever and a general sense of weakness or discomfort.
- Plasmacytoma arises from plasma cells and is an isolated form of multiple myeloma. Patients often feel pain but there appears to be no mass. This is one of the few chest wall tumors where surgery is not the primary treatment.
- Malignant fibrous histiocytoma is the most common soft tissue sarcomas and often occurs in adults. This type of soft tissue sarcoma usually appears as a slowly growing, painless mass.
- Rhabdomyosarcoma forms in striated muscle typically attached to bones and skin, enabling movement. It most often affects children and young adults and appears as a rapidly growing painless mass.
To diagnose a chest wall tumor, your doctor typically first conducts a physical examination. Then you will need an imaging test, such as an X-Ray, CT scan, or MRI scan for the doctors to determine the location and presence of a mass or tumor. Next, before deciding the appropriate treatment, your doctors will require more information: you will need a biopsy, a procedure that involves removing a small sample of tissue from the affected area for further study.
Treatment can vary based on factors such as the type of tumor and the stage of its progression. Surgical resection (removing the tumor) is the main type of treatment for most early stage chest wall tumors. Additional treatment can include radiotherapy (the use of radiation to kill cancer cells) and/or chemotherapy (the use of drugs to kill cancer cells).
At Mount Sinai, your thoracic surgeon will coordinate a team of specialists in managing your care. Our treatment philosophy is to remove the entire tumor, while preserving respiratory function and protecting the underlying chest structures (heart, lungs, major vessels that bring blood to and from the heart). Our goal is to cure the cancer with minimal or no effect on your quality of life as we care for you.
Mount Sinai offers comprehensive consultations by Board-Certified specialists, ensuring your speedy access to needed tests and the latest technologies. In addition to your medical history and physical examination, we use advanced, non-invasive imaging techniques, such as PET/CT (positron emission tomography and computed tomography) and biopsies to diagnose sarcomas and determine the best treatment strategy for you. Mount Sinai has pathologists and imaging specialists dedicated solely to sarcoma with the capability to conduct sound tissue and molecular tests to ensure your proper diagnosis and ideal treatment.
Removing the tumor surgically is the primary treatment for sarcomas. In addition, to achieve the best results, radiation therapy, and in some cases, chemotherapy may supplement surgical removal of the tumor. Our surgical oncologists work closely with other cancer specialists throughout the Mount Sinai Health System to develop a custom treatment plan for you.
Many Mount Sinai patients have the opportunity to participate in clinical trials and to receive promising new therapies not otherwise available. Participation in clinical trials is completely voluntary.
For a list of currently open clinical trials, search this list of opportunities, here.
For more information on our sarcoma research, email Mount Sinai at email@example.com.