Sarcoma

 

Sarcoma is a malignancy of the soft tissues. There are many subtypes of sarcoma, the most common of which are liposarcomas, malignant fibrous histiocytomas (MFH), and leiomyosarcomas. Average age at diagnosis is 40-50 years, with slight male predominance. Most lesions are located in the lower extremity (45%) or trunk (30%) and have a propensity to spread via the blood stream rather than the lymph nodes.

Most patients will present with a painless mass typically 4-6 months prior to diagnosis. About 20% of cases will actually have distant metastases at the time of diagnosis, with the risk 10% if low grade and 50% if high grade. Workup includes routine bloodwork, a CT or MRI of the primary site, CT of the chest to rule out metastases, and a CT of the abdomen to rule out retroperitoneal metastases, and incisional or core biopsy.

Treatment for sarcoma is variable. A common approach is preoperative radiation therapy followed by oncologic resection. An alternative is definitive surgery up front followed by postoperative radiation for high-risk features (high grade tumor, close or positive margins). At certain centers, intra-operative radiotherapy (IORT) is used to boost areas at risk near the tumor bed. The total amount of radiation is anywhere from 2 weeks (hypofractionated preoperative dose) to 7 weeks (definitive dose for unresectable cases). Chemotherapy, either in a preoperative or adjuvant (postoperative) setting, is of questionable benefit but newer agents are currently being studied.

Prognosis depends on a variety of factors, but in general stage I-II tumors have an excellent 5 year overall survival of 80-90% while more advanced tumors see a decrease down to 20-56%.