Soft tissue sarcomas (as
distinct from sarcomas of bone) are fairly common and tend to occur
in the extremities in 50% of cases, trunk and retroperitoneal area in 40% of cases,
and in the head or neck in 10% of cases. Sarcomas tend to occur in patients with von
Recklinghausen’s disease, Gardner syndrome and basal cell nevus syndrome, as
well as other syndromes. Sarcomas have also been associated with a history of
previous radiation exposure.
In-depth
Information
Patients with extremity sarcomas often present with a painful mass and swelling. A previous history of trauma is often elicited. The workup to evaluate soft tissue sarcoma should include a CT scan and an MRI of the extremity. The biopsy should be either core needle or incisional, with the incision or needle track planned with consideration of future resection or radiation therapy. The workup also should include a chest x-ray or CT scan for high grade lesions.
There are several subtypes of soft tissue sarcomas. Most tend to remain localized, but a few subtypes have a propensity for regional lymph node spread: rhabdomyosarcoma, angiosarcoma, clear cell sarcoma, epitheliod sarcoma, and synovial sarcoma. The lung is the most common site of distant metastasis.
TNM Staging System
| Primary Tumor | |
| T1 | Tumor less than or equal to 5.0 cm in greatest dimension |
| T1A | Superficial tumor |
| T1B | Deep tumor |
| T2 | Tumor greater than 5.0 cm in greatest dimension |
| T2A | Superficial tumor |
| T2B | Deep tumor |
| Lymph Nodes | |
| N0 | No regional lymph node involvement |
| N1 | At least one regional lymph node involved |
| Metastasis | |
| M0 | No distant metastasis |
| M1 | Distant metastasis |
The treatment
of soft tissue sarcomas depends on the size, grade, and location of these
tumors. Surgery is the primary treatment for cure in non-metastatic cases. In general,
superficial, low-grade tumors can be treated with surgery only. Deep muscle-invasive tumors are generally
treated with surgical resection followed by external beam radiation therapy; in some cases when a tumor is large and high-grade, radiation
is utilized prior to surgery and adriamycin/ifosfamide-based chemotherapy also
may be given. Chemotherapy is the principal treatment for stage IV
sarcomas.
Research
& Clinical Trials
If you are diagnosed with sarcoma, you should consider participating in a research protocol. Protocols often evaluate new agents that may have beneficial effects in the treatment of sarcoma. There are ongoing studies at the Emory Clinic. Click here to visit our clinical trial center.