The month of July is observed as “Sarcoma Awareness Month” in the United States. The main objective of this observance is to raise awareness about what is considered to be the “forgotten cancer.” Sarcoma is a rare type of malignant (cancerous) tumor that develops in a variety of tissue structures in our body such as the muscle, nerves, cartilage, tendons, blood vessels and the fatty and fibrous tissues. In addition, it may also occur in the bones, which means sarcomas can arise anywhere, because, these tissues are found everywhere on the body. There are more than 70 sub-types of sarcoma. As different types of sarcomas exist, the diagnosis, treatment, research and billing surrounding them can be challenging. Medical billing services for this particular condition requires better understanding of the existing codes and documentation/reimbursement guidelines.

Sarcomas, which are considered to be the “forgotten cancers”, are cancers that arise from the cells that hold the body together. It can invade surrounding tissue and can metastasize (spread) to other organs of the body, forming secondary tumors, referred to as “metastatic sarcoma”. They are named as metastatic because, the cells of secondary tumors are similar to those of the primary (original) cancer and are part of the same cancer and not a new disease. It is a rare cancer in adults (1% of all adult cancers), but rather prevalent in children. This disease affects more than 50,000 Americans and the families and friends who are by their side. However, due to a lack of awareness and understanding, the efforts to encourage Sarcoma research and drug development are more challenging. According to the American Cancer Society, in 2020, it is estimated that about 13,130 new soft tissue sarcomas will be diagnosed and about 5,350 people are expected to die of soft tissue sarcomas in the United States. These statistics include both adults and children, as it can touch lives no matter what age and at any location on the body.

Types of Sarcoma

In general, Sarcomas are divided into two main groups:

  • Soft Tissue Sarcomas
  • Bone Sarcomas

Tissues that connect, support, or surround other structures and organs of the body are referred to as soft tissues. It includes muscles, tendons, fibrous tissues, fat, blood vessels, nerves, and synovial tissues. However, cancerous tumors that develop in these tissues are called Soft Tissue Sarcomas. Due to the certain microscopic characteristics, similar symptoms, and similar treatment methods, these sarcomas are grouped together.

Even though bone tumors are also called sarcomas, they are categorized separately because they have different clinical and microscopic characteristics, and are treated differently. The most common type of bone cancer is osteosarcoma, which develops in new tissue in growing bones. Another form of bone cancer, Ewing’s sarcoma begins in immature nerve tissue in bone marrow. Both Osteosarcoma and Ewing’s sarcoma tend to occur more frequently in children and adolescents, while another type of cancer, chondrosarcoma, arises in cartilage and occurs more often in adults.


According to the American Cancer Society, most people notice a lump in an arm or leg that’s grown over time (weeks to months). The lump may or may not hurt. However, sarcomas can also grow in the back of the abdomen (the retroperitoneum) pressing nerves, blood vessels, or nearby organs. This type of sarcoma displays symptoms often that come from other problems the tumor is causing such as blockage or bleeding of the stomach or bowels. Sometimes, these tumors can grow large enough to be felt in the abdomen and can cause pain.

General signs and symptoms of sarcoma include:

  • A new lump or a lump that’s growing anywhere on your body
  • A lump that can be felt through the skin that may or may not be painful
  • Abdominal pain that’s getting worse
  • Blood in your stool or vomit
  • Black, tarry stools
  • Bone pain
  • A broken bone that happens unexpectedly, such as with a minor injury or no injury at all
  • Abdominal pain
  • Weight loss

Even though these symptoms might be caused due to other things, consulting a doctor is highly recommended.

Causes & Risk Factors

It’s still not fully understood why some people develop sarcomas while the vast majority do not. Generally, the cancer forms when changes (mutations) happen in the DNA within cells. By identifying common characteristics in groups with unusually high occurrence rates, researchers have been able to identify some factors that may play a role in increasing the risk of sarcoma. They are:

  • Inherited syndromes.
  • Radiation therapy for cancer may pose a risk of developing a sarcoma later.
  • Chronic swelling (lymphedema) may increases the risk of a type of sarcoma called angiosarcoma.
  • Exposure to chemicals, such as some industrial chemicals and herbicides, can increase the risk of sarcoma that affects the liver.
  • Exposure to viruses, especially, human herpesvirus 8 can increase the risk of a type of sarcoma called Kaposi’s sarcoma in people with weakened immune system

Diagnosis & Treatment

According to the National Cancer Institute, sarcomas are difficult to distinguish from other cancers. Their occurrence is probably underestimated when they are found within organs. Therefore, people with lumps and bumps, should have them looked at by a doctor at an early stage in case it is sarcoma.

Some diagnosis methods used to identify sarcoma and determine its extent (stage) include:

  • A physical exam: With this diagnosis method, your doctor will be able to understand your symptoms and look for other clues that will help with your diagnosis.
  • Imaging tests: Imaging tests used to diagnose sarcomas include X-ray, MRI, ultrasound, CT, bone scans and positron emission tomography (PET) scans. X-rays are better for seeing bone problems, whereas, other tests, such as MRI, are better for seeing connective tissue problems.
  • Biopsy: If a soft tissue sarcoma is suspected based on a physical exam and imaging tests, a biopsy would be needed to confirm if it’s a sarcoma and not another type of cancer or a benign (not cancer) disease. A surgical biopsy is a procedure that removes a piece of suspicious tissue for testing. The piece of tissue is removed with a needle passed through the skin or cut away during an operation. Sometimes a biopsy is done at the same time as surgery to remove the cancer. Sophisticated lab tests can determine whether the cells are cancerous and what kind of cancer they represent. This test can also reveal information that’s helpful for choosing the best treatments.

Once sarcoma is diagnosed, your doctor would recommend additional tests to look for signs if the cancer has spread or not.

The best method to remove sarcoma is through surgery. The Sarcoma Foundation of America (SFA) estimates that about 20% of sarcoma cases are curable by surgery while another 30% may be effectively treated by surgery with chemotherapy and/or radiation.

In general, treatment for sarcomas depends on the stage of the cancer, which is based on the size and grade of the tumor, and whether the cancer has spread to the lymph nodes or other parts of the body. Apart from surgery, other treatments for sarcoma are:

  • Radiation therapy: This treatment uses high-powered energy beams, such as X-rays and protons, to kill cancer cells.
  • Chemotherapy: Some types of sarcoma are more likely to respond to chemotherapy treatment than others, which is a drug treatment that uses chemicals to kill cancer cells.
  • Targeted therapy: If your sarcoma cells are likely to respond to targeted therapy drugs, a drug treatment that uses medicines to attack specific weaknesses in cancer cells, then it could be a better treatment method.

Other treatments include Immunotherapy and ablation therapy.

The screening and diagnostic tests performed as part of the treatment procedures must be correctly documented using the right medical codes. Professional medical billing companies can ensure this. Billers and coders in such reputable companies will have a thorough knowledge about the related medical codes and payer-specific guidelines essential for correct billing and optimal reimbursement.

ICD-10 diagnostic codes for Sarcoma are:


  • C49: Malignant neoplasm of other connective and soft tissue
    • C49.1: Malignant neoplasm of connective and soft tissue of upper limb, including shoulder
    • C49.2: Malignant neoplasm of connective and soft tissue of lower limb, including hip
    • C49.4: Malignant neoplasm of connective and soft tissue of abdomen
    • C49.9: Malignant neoplasm of connective and soft tissue, unspecified.

Bone Sarcoma

C41.9: Malignant neoplasm of bone and articular cartilage, unspecified

Other types of sarcoma include:

  • C46: Kaposi’s sarcoma
    • C46.1: Kaposi’s sarcoma of soft tissue
    • C46.2: Kaposi’s sarcoma of palate
    • C46.3: Kaposi’s sarcoma of lymph nodes
    • C46.4: Kaposi’s sarcoma of gastrointestinal sites
    • C46.5: Kaposi’s sarcoma of lung
    • C46.7: Kaposi’s sarcoma of other sites
    • C46.9: Kaposi’s sarcoma, unspecified

Sarcoma Awareness Month (SAM) is celebrated in July every year. It is dedicated to those who had and have sarcoma. SAM also highlights the extraordinary challenges that sarcoma patients face, the need for more sarcoma research and better sarcoma therapies. They allow each person in the sarcoma community to make an impact and help increase awareness for this relentless cancer.

As this year the entire world is hit by the COVID-19 pandemic, the Sarcoma Foundation of America (SFA) has arranged “National Race to Cure Sarcoma Virtual event” for Sarcoma Awareness Month (SAM) celebration and invites everyone to join. With this virtual event, the SFA wants us to gather together near and far to raise awareness and funds in the hope of finding a cure for sarcoma.