Thanks to popular nonprofits like Feel Your Boobies and Get a Grip, we all know what to do when we detect a lump or bump on our reproductive parts. But what happens when a person notices a Superball-sized growth deep under the skin or they start having a lot of bone pain when trying to sleep at night? If symptoms persist for more than a week or two, an appointment to be examined should be considered. Why? Because it could be a tumor.
Both bone and muscle tumors can occur in people of any age. Usually, most persistent masses are benign, meaning that they won’t spread to another part of the body. But it is not unusual for patients to need some tests or treatments to confirm this diagnosis. Malignant or cancerous bone and muscle tumors account for only about 1% of all cancers, but they are very aggressive, require intensive treatment, and can be lethal (Figures 1 and 2). There are very few factors that predispose a patient to a bone or soft-tissue tumor. There are slightly more males than females diagnosed each year, and age is relevant only in the incidence of the type of tumor or tissue where it arises from (known as histology).
Depending on their insurance situation, patients may have to see their primary care doctor first, but they should always be referred to an orthopedic surgeon who specializes in tumors, called an orthopedic oncologist, if there is any question of there being an abnormality. Most orthopedic oncologists are affiliated with larger hospital centers or universities, where patients can have all of their additional tests performed.
Patients with persisting mass or bone pain will need x-rays and very often CT scans and MRIs, which show what the inside of our soft tissues look like. If any of these tests, known as imaging studies, show an abnormality, a biopsy will be required.
The biopsy must be performed by an orthopedic oncologist or an interventional radiologist who is very experienced in performing tumor biopsies that avoid contaminating surrounding tissue. Poorly performed biopsies, in the case of malignant tumors, can mean the difference between limb salvage and amputation. Biopsies collect samples of abnormal tissues, for microscopic evaluation. This will definitively determine what disease is causing the abnormality and help the orthopedic oncologist determine a plan of action.
Some benign tumors require little or no surgical intervention. Some benign-aggressive tumors can grow to a large size or destroy bone and will require surgical removal and additional treatment such as freezing with liquid nitrogen.
Tumors revealing malignant cells are called sarcomas, classified as low or high grade, and are treated accordingly. About 65-70% of patients diagnosed with sarcomas are cured. Low-grade sarcomas are treated with surgery to remove the tumorous cells as well as a rim of about 2 inches of normal tissue surrounding the tumor. During surgery, the pathologist will look at the tissue to ensure that no abnormal cells remain.
For unfortunate patients with high-grade sarcomas, very often treatment is complex and requires an oncology team made up of medical oncologists (chemotherapy doctors), orthopedic oncology surgeons, occasionally radiation oncology physicians, nurses, and rehabilitative medicine specialists. Chemotherapy and radiation therapy should be performed by oncologists who specialize in treating sarcomas. Most patients will receive several months of preoperative chemotherapy to reduce the size of the tumor before surgery. Surgical resection of the tumor with concurrent salvage of the limb can be achieved in about 95% of cases. Joints are removed when treating a bone sarcoma and are replaced with chromium-cobalt metal replacements, which offer the patient good function post operatively (Figure 3). Children older than 5 are eligible for limb salvage procedures, but their surgeries are more challenging because of their continuously growing bones. Expandable prosthetic implants for growing patients have been developed, and can be lengthened at regular intervals. Children and adolescents will also receive psychological counseling or exposure to a child life specialist to discuss issues such as cosmetic appearance (like losing hair and surgical scars), physical limitations, potential future fertility issues, etc.
Additional chemotherapy or in rare circumstances, radiation therapy, will resume following surgical healing to kill remaining tumor cells that have broken away from the original tumor or been displaced during surgery. Because most extremity lumps are transient, patients typically do not become alarmed if they find an abnormal muscle swelling or some bone pain, chalking it up to a sports or overuse injury. However, if these symptoms persist, we strongly recommend a referral to an orthopedic oncologist.
Figure 1. This is a clinical photograph of a large soft-tissue sarcoma arising from the leg. If the tumor does not involve the arteries or nerves of the leg, a limb-sparing surgery can most likely be performed.
Figure 2. This is a plain X-ray showing a primary bone tumor (osteosarcoma) of the proximal tibia bone. The dense white ball (labeled osteoblastic) shows an overabundance of production of abnormal bone. The portion of the x-ray under (labeled osteolytic) shows the destructive nature of the tumor. Osteosarcomas may be osteoblastic, osteolytic, or mixed (as demonstrated in this x-ray).
Figure 3. The Modular Replacement System, developed by Stryker Osteonics, Inc. of Mahwah, NJ in collaboration with the senior author for this article, is routinely used for reconstruction following a limb sparing surgery. Most major joints can be replaced with a chromium-cobalt modular system.