Purpose: Paratesticular sarcomas are rare and frequently reported as isolated case reports. Studies evaluating the relative frequency of the paratesticular sarcomas are limited, and to the best of our knowledge, this is the first study of paratesticular sarcomas in the Brazilian population. Patients and Methods: Medical records of all patients undergoing treatment for paratesticular sarcomas between 1993 and 2006 were retrieved from the archives of our institution. Results: Complete data from 12 patients (39 ± 23 years, range 13–78) with paratesticular sarcomas were available, which represented 6.7% of all orchiectomies performed for testicular malignancies in the same period. At the time of diagnosis, 3 patients had retroperitoneal spread of the disease, all of which had elevated serum lactic dehydrogenase levels. The remaining 9 patients had normal serum markers. There were 6 rhabdomyosarcomas, 4 leiomyosarcomas, 1 liposarcoma and 1 undifferentiated sarcoma. Median follow-up was 31.4 months. Primary surgical excision by inguinal approach was performed in all cases (radical orchiectomy in 10 and preservation of the testis in 2). Retroperitoneal lymph node dissection was performed in 3 patients and excision of the hemiscrotum in 1. Eight patients received adjuvant chemotherapy. Mean overall survival time was 27.8 ± 6.2 months after orchiectomy. Conclusion: Patients with paratesticular sarcomas are at high risk of disease progression, and systemic relapse remains a significant problem, determining poor prognosis. The high risk of local recurrence demands long-term follow-up, and intraoperative frozen section analysis might be of benefit. Elevated lactic dehydrogenase might also be a marker of retroperitoneal disease and poor prognosis. Improvement in survival requires effective systemic adjuvant therapy.

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