Introduction: Aggressive fibromatosis is a benign but locally aggressive process. It arises from musculo-aponeurotic tissues, and invades locally without respect for tissue planes, surrounding vessels and nerves, which makes treatment of local recurrences difficult.

Aims: Our aim is to review our experience in the management of aggressive fibromatosis, focussing on the cases of multiple recurrences, as well as to evaluate the need for disabling surgery.

Material and methods: We present the series of 33 patients (15 male and 18 female) diagnosed of aggressive fibromatosis treated between 1993 and 2003; the follow-up period was no less than two years. The locations were shoulder girdle (8), lower extremity (8), upper extremity (6), gluteus (5), paravertebral (4) and thorax (2). There were 6 cases with 3 or more episodes of local recurrences; in these cases, depending on location and size, and considering high surgical morbidity, associations of radiotherapy, chemotherapy and hormone therapy were given, avoiding disabling surgery.

Results: With an average follow-up of 32 months (25 to 50), there were no deaths and, in the 6 cases of multiple recurrences, there were no amputations. The control MRI demonstrated stability of the process in 5 out of 6 cases, and minimum growth without clinical correlation in the other one. In the other 27 cases, there were 14.8% surgical treated local recurrences, with no need for amputation.

Conclusion: We consider that cases of multiple recurrences of aggressive fibromatosis benefit from adjuvant treatment (radiotherapy, chemotherapy and hormone therapy), avoiding disabling surgery, which is unnecessary following our criteria. This requires strict clinical and radiological control.

Introduction: Synovial sarcoma ranks as the fourth most common sarcoma, but it is uncommon in the hand. Most Synovial Sarcomas arise in para-articular soft tissue such as tendon, tendon sheath and bursa adjacent to the large joints capsules. Arround 60–70% of these tumors involve lower extremity and they frequently affect knee, thigh and foot. The reported incidence for Synovial Sarcoma in hand is only 8.5%.

Material and methods: We carried out a retrospective study of 6 hand and forearm sarcomas, of a series of 35 synovial sarcomas surgically treated in our center from 1991 to 1997, with a 6 years follow up (3y.–11y.). 3 patients were male and 3 female, with a mean age of 54 years at the moment of initial diagnosis. Histologically all of them were synovial sarcomas: 4 monophasic and 2 biphasic. The inmunochemistry showed that the neoplasic cells were positive for vimentin, epithelial membrane antigen and cytokeratin. 2 of them were localized in the palmar aspect of the hand (2 ulnar cases and 2 eminence tenar cases), an 2 cases were dorsal. 2 cases were misdiagnosed as benign lesions and treated with tumor excision at another center.

Results: Primary treatment consisted of radical local excision of synovial sarcoma of the hand. 5 patients received adjuvant chemotherapy with CYVADIC and radiotherapy. 2 cases of recurrence received a second surgical treatment with forearm amputation. The mean time to recurrence was 12 months. At the end of the follow-up 3 patients developed metastatic disease and 2 of them died.

Discussion: Synovial Sarcoma in hand is a highly malignant tumor; due to its morphology they can be misdiagnosed as benign lesions such as aggressive fibromatosis or ganglion cysts. If we diagnose a soft tissue tumor in hand we must practice complementary tests to achieve early diagnosis. It is also important the multidisciplinary treatment of Synovial Sarcoma.

Aims: The purpose of this report is to asses the prognostic factors that could influence management and clinical outcome of malignant fibrous histiocytoma (MFH) of soft tissues. Methods: Between 1990 and 2000, 79 patients diagnosed with localized disease by MFH, seen at Santa Creu i Sant Pau Hospital, have been reviewed. 45 were women and 34 were men. The median age at presentation was 62 years (range: 20–85). The median follow-up was 60 months (range: 1–119). Low grade tumors constituted 8% and the remaining 92% were high grade. Several treatment modalities with or without radiotherapy and/or chemotherapy were associated and assessed. We used Kaplan-Meier method for calculating survival rates and Log-Rank test to assess presumed prognostic factors like sex, age, anatomic side, Enneking stage and associated quimio/radiotherapy. Results: Thigh were the most common location (50%). Isolated local recurrence occurred in 19 patients (24%), isolated meta-static disease without local recurrence in 4 patients (5%), and combined local and metastatic disease occurred in 4 patients (5%). The 2- and 5-year relapse-free survival (RFS) rates were 71% and 67%, respectively.

The 2- and 5-year overall survival (OS) rates were 92% and 86% respectively. We found that only Enneking stage was significant prognostic factor (p< 0.05). The best results in SLE were found in surgery + radiotherapy group but it was not statistically significant (p> 0.05). Conclusions: In our opinion, wide complete surgical resection at the time of primary tumor in combination with local radiotherapy is likely to afford the best chance for RFS and OS. Enneking stage seems to be the most important prognostic factor.

The role of adyuvant chemotherapy remains investigational.