Soft tissue sarcomas arise primarily from what are called “connective tissues”. These tumors are usually discovered as a painless mass, which has grown subtly over time. Although patients often associate them with a traumatic bump or blow, they are not caused by such injury. Rather such an injury may make a person aware of an abnormal swelling in the same or adjacent location.

These tumors are often confused with truly benign conditions. For example, liposarcomas, which are relatively rare malignant fatty tumors, may easily be confused with lipomas which are very common benign fatty tumors. Because of their rarity, it is not surprising that sarcomas are frequently misdiagnosed and frequently mistreated. In the United States, the American Cancer Society estimates that there are only 13,000 new cases of soft tissue sarcoma, but these lead to over 5,300 deaths each year.

Careful surgery is the mainstay of soft tissue sarcoma treatment. For localized tumors of lower grade, cure is often completely dependent on how a biopsy, and how a resection, is managed. The biopsy must be sufficient to gain adequate tissue, as many of these tumors have variations within them that makes needle biopsy inaccurate. But the orientation of a biopsy incision, and avoidance of unnecessary bleeding or spreading of tumor during the procedure is critical. In some cases, with intraabdominal sarcoma, needle biopsy may be the only sampling option, and in some cases an aggressive resection may be necessary without a preceding biopsy.

Sarcomas do not grow within a “capsule” of fibrous tissue. Cells often trail off into the surrounding tissues. Thus, when primary sarcoma surgery is performed, the goal is to remove the entire tumor with a reasonable layer of normal tissue around it. Ideally, this would include a fibrous layer serving as a boundary. Accomplishing this goal may be a challenge if the tumor involves critical blood vessels or nerves. In some cases, there may be little choice but to perform an amputation if a sarcoma actually encircles major blood vessels or nerves in an arm or a leg. As sarcomas can spread along tissue planes, even an amputation may need to be more aggressive than anticipated.

Fortunately, studies done at the Surgery Branch of the National Cancer Institute during my tenure there, demonstrated the ability to combine proper local resection with radiation therapy in order to avoid amputation in the majority of localized soft tissue sarcomas of the arms and legs. In some cases, the use of preoperative chemotherapy may reduce the the size of the tumor, permitting resections, using limb preservation techniques, that would have otherwise been impossible.

In the trunk, removal of a soft tissue sarcoma may require removal of the tumor with adjacent intra-abdominal organs. As these tumors may be very large when discovered, this can result in very extensive surgery. However, because the opportunity for clear margins of resection is smaller in sarcomas of the abdomen or chest, these tumors recur more frequently.

When sarcomas recur, they usually do so within the first 2 to 3 years. The lungs are the most common site of spread or metastasis. However, data also generated in the Surgery Branch at the NCI established the value of metastasectomy: that is the removal of a limited number of metastatic lung nodules. When patients are carefully chosen, nearly a third of such individuals may be permanently cured of their disease.

With each passing year there are more active drugs, some of which may be valuable in the management of soft tissue sarcomas. These include chemotherapy drugs, with the expected toxicities and side effects. However, some are biologically active agents that modulate how tumor cells grow. While these may not always lead to cure, some these agents can produce long-lasting control and benefit, with limited side-effects.

Because of their rarity, and because of the complexity of their multi-disciplinary management, soft tissue sarcomas really require a knowledgeable and experienced surgeon and an integrated team of oncology consultants who can facilitate care. Most patients with soft tissue sarcomas can, and will be, cured of their disease. But it is an unforgiving disease in which early missteps have real consequences.

Sarcoma management by an experienced surgical oncologist is an essential part of curative care. However, the process of surgery and ancillary care is not easy and requires a team of providers. Having access to local care is very important, as mobility may be impacted after surgery, making long-distance commuting challenging. However, when required, advanced specialty care should be coordinated with national centers of excellence.

Having a primary surgical practitioner who understands the biology of these tumors, and who can integrate care across a team of nationally recognized consultants, provides the best strategy for both short and long term management.

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For additional information about adult soft tissue sarcomas, and their modern multidisciplinary management, please refer to the PDQ Website of the National Cancer Institute.