[Synovial sarcoma of the extremities. Results of surgical and multimodal therapy].
BACKGROUND
Synovial sarcoma is a rare tumor frequently occurring in adolescents and young adults. The preferred location, at the distal parts of the extremities, and the high incidence of distant metastases represent major challenges for treatment. The purpose of this study is to analyze the indications for surgery in the context of a multimodal therapy.


METHODS
Between October 1992 and December 2000, 41 patients were treated surgically for synovial sarcoma of the extremities. Their mean age was 35 years (range: 11-75 years). Extracompartmental tumor growth was present in more than 90% of the patients. Fourteen patients (34%) presented with a tumor size of less than 5 cm (T1) while 27 patients (66%) showed lesions greater than 5 cm (T2). At the time of presentation, seven patients had distant metastases; three located in the lung and four in lymph nodes.


RESULTS
Due to a locally unresectable tumor or synchronous distant metastases, 28 patients (68%) underwent preoperative systemic chemotherapy (n=9) or isolated limb perfusion (n=18) or both (n=1). In 29 patients, limb-conserving surgery was possible. Of the 12 patients (29%) who required amputation, 85% had a distal tumor. Sixteen patients received additional postoperative radio- and/or chemotherapy. After a median follow-up of 30 months, only two patients (7%) had developed a local recurrence, while seven patients (20%) suffered from new, distant metastases. The 5-year overall survival rate was 82%.


CONCLUSIONS
With a combined modality treatment, it is possible to achieve excellent local control rates and long-term survival in patients with synovial sarcoma of the extremities. Neoadjuvant treatment can enable limb-sparing surgery in most patients, even if the tumors are located in anatomically difficult areas.
Ulmer
C
C
Klinik für Chirurgie und Chirurgische Onkologie,Charité Campus Berlin-Buch, Humboldt Universität Berlin,Robert-Rössle-Klinik im Helios Klinikum Berlin.
Kettelhack
C
C
Tunn
P U
PU
Reichardt
P
P
Hohenberger
P
P
Schlag
P M
PM
D000293:Adolescent; D000328:Adult; D000368:Aged; D000671:Amputation, Surgical / Q000401:mortality; D017024:Chemotherapy, Adjuvant; D002648:Child; D003131:Combined Modality Therapy / Q000379:methods / Q000401:mortality; D005121:Extremities / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D023821:Limb Salvage / Q000401:mortality; D008175:Lung Neoplasms / Q000401:mortality / Q000473:pathology / Q000556:secondary / Q000601:surgery; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D020360:Neoadjuvant Therapy; D009364:Neoplasm Recurrence, Local / Q000401:mortality / Q000473:pathology / Q000601:surgery; D009367:Neoplasm Staging; D009360:Neoplastic Cells, Circulating; D018714:Radiotherapy, Adjuvant; D013584:Sarcoma, Synovial / Q000401:mortality / Q000473:pathology / Q000556:secondary / Q000601:surgery*; D012983:Soft Tissue Neoplasms / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D015996:Survival Rate