Synovial sarcoma of the foot and ankle.

Synovial sarcoma of the foot and ankle frequently is misdiagnosed, which leads to delays in treatment. The clinical records of 14 patients with synovial sarcoma of the foot and ankle were reviewed. Common findings at presentation were an enlarging mass with a variable incidence of pain, tenderness, and edema. The patients tended to be younger than patients with other soft tissue sarcomas (30 years) and had a median duration of symptoms of 14 months. Of the 14 patients, 12 underwent an attempted curative surgical procedure. Ten patients had partial foot amputations or below knee amputations, whereas two had an attempted limb salvage by wide resection. Of the 14 patients, one experienced regionally recurrent disease and eight had pulmonary metastasis developed. All patients who had metastasis develop died of their disease. Tumor size was not observed to be a prognostic variable in this group of patients. Patients with biphasic histologic features had a better outcome than did those with a monophasic subtype. Patients with a prolonged duration of symptoms before diagnosis had a better outcome, presumably because these tumors biologically were less aggressive. Wide resection can be considered in a select group of patients.     

Synovial sarcoma

The aim of this project was to investigate the diagnosis, treatment and consequences of local recurrence of soft tissue sarcoma (STS). It is based on patients reported to the Karolinska Hospital Sarcoma Register and the Scandinavian Sarcoma Group Register. Demographic and treatment data, based on 1613 adult patients reported to the Scandinavian Sarcoma Group Register by sarcoma centers in Norway, Sweden and Finland are presented. They all had STS of the extremities or trunk wall, and were diagnosed between 1986 and 1995. One third of the tumors were subcutaneous and two thirds deep-seated. The median size was 7 (1-35) cm and 75% were high grade. Metastases at presentation were diagnosed in 8% of the patients. Two thirds of the patients were referred to a sarcoma center before surgery. The preoperative morphologic diagnosis was made by fine-needle aspiration cytology in 72%. Among patients with final treatment for primary tumor at a sarcoma center (n=1331), the surgical margins were wide or better in 76% of subcutaneous lesions, and in 58% of deep-seated lesions. Adjuvant radiotherapy has not generally been considered indicated after wide or compartmental excisions in Scandinavia. Overall, 23% of patients managed by surgery had adjuvant radiotherapy. Among patients with an intralesional or marginal excision, 44% had postoperative radiotherapy. Patients treated outside of sarcoma centers were seldom referred for radiotherapy. The crude local recurrence rate was 225/1331 (17%) among the patients with final treatment for primary tumor at a sarcoma center. The local recurrence rate after local surgery for high-malignant deep-seated STS was 103/391 (26%). The rate was 25/64 (39%) after an intralesional/ marginal margin without postoperative radiotherapy versus 28/119 (24%) when radiotherapy was given. Fine-needle aspiration cytology (FNAC) was used to diagnose suspected local recurrences. 95 FNAC were performed in 86 patients from Karolinska Hospital. There were 47 local recurrences, of which 44 were diagnosed correctly by FNAC; one biopsy was inconclusive, and two lesions were incorrectly assessed as benign. 39 patients proved to have benign lesions in the scar examined cytologically on 50 occasions. None of the specimens was regarded as malignant, but in 4 cases FNAC was inconclusive. The inconclusive or false cytological diagnoses had no serious clinical consequences. Among 205 patients with local recurrence identified in the SSG Register 1987-1995, 169 patients were surgically treated. An intralesional or marginal margin was achieved in 110 of these patients, 59 of whom were also given radiotherapy. 54 of the 169 patients had a second local recurrence. The second local recurrence rate was 0.50 if the first local recurrence was treated using surgery with a marginal margin alone, compared to 0.28 if treated using either surgery with a marginal margin and radiotherapy, or a wide margin ( p = 0.0008). In extremity STS, the amputation rate for local recurrences was 0.22, compared to 0.09 for primary tumors. The overall 5-year MFS was 0.72 (95% CI 0.68-0.76). High histopathological malignancy grade (Relative Risk 3.0; 95% CI 1.5-6.3) and an inadequate surgical margin (2.9; 95% CI 1.8-4.6) were independent risk factors for local recurrence. High histopathological malignancy grade and large tumor size (>7 cm) were the most important risk factors for metastasis. Local recurrence was associated with an increased risk of metastasis (4.4; 95% CI 2.9-6.8), but an inadequate surgical margin was not a risk factor for metastasis (1.1; 95% CI 0.8-1.7). In conclusion, it is unlikely that local recurrence of STS is a major source of metastases. It nevertheless represents a costly, complicated and emotionally difficult problem. More radical surgical margins would improve the local recurrence rate, but this can hardly be achieved for centre-operated patients without increasing the amputation rate. Instead, local control will improve by giving radiotherapy to all patients after marginal surgery, and to selected patients with wide margins. Radiotherapy is indicated especially after a previous open biopsy or when a local recurrence might lead to an amputation. Furthermore, radiotherapy seems indicated after local recurrence, regardless of margin or grade. The most effective way of reducing costs and detriment associated with local recurrence is to increase referral to sarcoma centers before biopsy or surgery as primary surgical margins would then improve.     

Synovial cell sarcoma

Synovial sarcoma of the head and neck is a rare lesion. Based on previous literature reports, these lesions carry a poor prognosis. Case reports of two patients diagnosed and treated recently for head and neck lesions of this type are presented. A review of pathologic sections at the Armed Forces Institute of Pathology (AFIP) suggests that this lesion is more colmon than believed previously.     

Synovial chondromatosis of the foot

Synovial chondromatosis rarely occurs in the foot. Five patients with synovial chondromatosis in the foot were treated with excision. There were four men and one woman with a mean age of 37 years (range, 19-58 years). Mineral densities adjacent to the joint were seen on radiographs of all patients. Synovial chondromatosis occurred in the calcaneocuboid, tibiotalar, naviculocuneiform, and metatarsophalangeal joints. A painful mass was the common initial presentation in all patients. The patients were followed up for an average of 5 years (range, 3-16 years) after arthrotomy and excision. All patients were relieved of symptoms and retained normal function. There was no clinical or radiographic evidence of recurrence.     

Synovial sarcoma of the hand

Synovial sarcoma of the hand is a very rate tumor in an equally unusual location with a difficult histology of a fibrosarcoma combined with synovial-like areas that resemble joint endothelial lining. It has a poor prognosis prior to the current combined treatment of surgical ablation, radiation to the surgical field, and chemotherapy for microscopic metastases. It has an unexplained natural history of very late recurrence and distant spread, often 10 to 20 years following primary treatment.     

Synovial sarcoma of the extremities

Summary From May 1985 to March 1990, 18 cases of Enneking's stage II synovial sarcoma of the extremities were treated by a combination of surgery and CYVADIC chemotherapy with or without radiotherapy. The average follow up was 34.7 months. The estimated 5-year survival rate was 78.4% and the continuously disease-free 5-year survival rate was 44.4%. The results were improved by adequate surgery or chemotherapy when local recurrence or metastases had developed. The combination of surgery and CYVADIC chemotherapy with or without radiotherapy was considered to be an effective method of treating this tumour.

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Résumé Dix-huit synovialo-sarcomes des extrémités de stade II selon Enneking ont été traités entre mai 1985 et mars 1990 par un traitement combiné de chirurgie et de chimiothérapie associant cyclophosphamide — vincristine — adriamycin — dacarbazine (CYVADIC) en ajoutant ou non une radiothérapie. La chirurgie été une excision large (14 cas) ou une amputation (4 cas). La chimiothérapie par CYVADIC a été administrée en 6 cycles chez tous les patients. La radiothérapie (de 50 à 80 Gy, en moyenne de 62 Gy) a été utilisée chez les 14 patients opérés par excision large et chez 1 patient après amputation. Le suivi moyen des malades est de 34,7 mois avec un minimum d'un an. Le taux de survie estimé à 5 ans (méthode de Kaplan-Meier) est de 78,4% et le taux de patients sans signe de maladie est de 44,4%. La survie sans signe de maladie semble améliorée par une chirurgie ou une chimiothérapie adaptée quand il existe une récidive locale ou des métastases. L'association chirurgie et chimiothérapie par CYVADIC avec ou sans radiothérapie, s'avère une méthode efficace de traitement du synovialo-sarcome des extrémités.