PubMed2001
Soft tissue sarcoma surgery is based on techniques that are in the process of ongoing development. In this study, the case is reported of a female patient who was operated on at the age of 14 years for a primary synoviosarcoma of the dominant hand, which was treated by conservative surgery and 60 Gy adjuvant radiotherapy. Twenty-two years later, she presented with a clinical picture of recurrence, but with no detectable metastases. Amputation of the distal third of the forearm was performed. The anatomopathological examination showed the presence of a myxoid malignant fibrous histiocytoma. It was considered that this tumor had been radiation-induced. All the distinct diagnostic criteria were met, i.e., a latency of over two years, different diagnosis and the appearance of the tumor within (or next to) the irradiated field. Both the diagnostic methods (MRI, tumor biopsy, and CT scan of the chest to investigate possible tumor spread) and the surgical approach have been discussed. The latter has altered over the years from being systematically radical (amputation or compartimental resection) to systematically conservative (good local control via radiotherapy). The modern attitude reflects a return to a more balanced approach. The frequency of such tumors has probably been underestimated in reports in the literature, as the latency period can sometimes exceed 30 years, and an accurate determination of the etiology is not always possible. Two main factors should be taken into account in treatment strategy: i) distant metastases of high-grade soft tissue sarcomas often appear early in the course of the disease, and are not affected by surgery at the primary site; ii) local recurrence, which is associated with high morbidity, and is directly connected with incomplete resection. Technical progress and a multidisciplinary approach have resulted in more sophisticated treatment (allowing a larger surgical resection area, and better residual function). Surgical management remains the treatment of choice, as radiotherapy and chemotherapy have not demonstrated any positive effect on patient survival.