In most cases, a combined modality approach of preoperative radiation therapy (preRX) or postoperative radiation therapy (PORT) is used, rather than the radical surgical procedures, such as amputation, that were used in the past. It may even be possible to use surgery without PORT in selected cases. For example, a case series was reported from a specialized sarcoma treatment referral center in which 74 selected patients with primary extremity and trunk tumors 5 cm or less in size were found to have no histologic involvement of the surgical margins. The patients were observed without radiation therapy, and the estimated local recurrence rate after 10 years was 11%.[Level of evidence: 3iiiDiv] The role of chemotherapy is not as well defined as is the role for radiation therapy. Because of the evolving nature of the treatment options for this disease, patients should be offered the option of clinical trials when available. Information about ongoing clinical trials is available from the NCI Web site.
The histiocytic diseases in children and adults include three major classes of disorders. Only Langerhans cell histiocytosis (LCH), a dendritic cell disorder, is discussed in detail in this summary. Erdheim-Chester disease (primarily found in adults) and juvenile xanthogranuloma (diagnosed in children and adults) are macrophage disorders. Other disorders of the macrophage/monocytoid lineages include Rosai-Dorfman disease and hemophagocytic lymphohistiocytosis. Malignant disorders include malignant...
Surgical resection is the mainstay of therapy for soft tissue sarcomas. When feasible, wide-margin function–sparing surgical excision is the cornerstone of effective treatment for extremity tumors. This may be facilitated by soft tissue reconstructive surgery, which generally permits wider margins than those obtained when the surgical plan involves direct closure of the excision site. Cutting into the tumor mass or shelling out the gross tumor along the plane of the pseudocapsule of compressed tumor cells and reactive tissue that often surrounds soft tissue sarcomas are associated with an elevated risk of local recurrence. Even high-grade soft tissue sarcomas of the extremities can usually be effectively treated while preserving the limb with combined-modality treatment consisting of preRX or PORT to reduce local recurrence. (Refer to the Role of Radiation Therapy section of this summary for more information.)
Only one small, single-institution randomized trial has directly compared amputation to limb-sparing surgery for soft tissue sarcomas of the extremities. In a 2:1 randomization ratio, 27 patients with high-grade extremity sarcomas were assigned to a wide excision plus PORT (45 Gy–50 Gy to the wide local excision area, and a total of 60 Gy–70 Gy to the tumor bed over 6–7 weeks), and 16 were assigned to amputation at or above the joint proximal to the tumor. Both groups received adjuvant chemotherapy (i.e., doxorubicin, cyclophosphamide, and high-dose methotrexate). At 63 months, with a median follow-up of 56 months, there were four local recurrences in the 27 patients who underwent limb-sparing surgery and no recurrences in the 16 patients who underwent amputation P2 = .12. Overall survival (OS) rates were not statistically significantly different (actuarial 5-year survival rate, 83% vs. 88%, P2 = .99).[Level of evidence: 1iiA]