10093 Background: PCS are very rare tumors with poor prognosis. The current study aimed to evaluate clinico-pathological characteristics, treatment and survival in a cohort of patients (pts) referred to the FSG Network. Methods: From June 1977 to January 2010, 123 pts with PCS were identified. Medical records and clinicopathologic data were collected. Results: There were 54 men (44%) and 69 females (56%) with a median age of 47 years (range 3-82). Median time between first symptoms and diagnosis was 1 months (mo) (range 0-53). The most frequent revealing clinical signs were dyspnea (41%), thoracic pain (22%), heart failure (13%) and pericarditis (5%). The median tumor size was 60 mm (range 8-100). The pathological diagnoses included poorly differentiated sarcoma (S) (n=45), angioS (n=40), leiomyoS (n=16), synovialoS (n=7), rhabdomyoS (n=6), Ewing S (n=4), lipoS (n=3) and chondroS (n=2). At diagnosis, 99 pts (80%) had a localized disease and 24 pts had metastases in one site (n=14) or in multiple sites (n=10). The most frequent metastatic sites were lung (n=9), liver (n=7), bone (n=7), brain (n=4). Tumors were mainly located in the atrial cavities (right atrium n=47; left atrium n=44). Other localization included right ventricle (n= 6), left ventricle (n=9) and pericardium (n=3). In 11 cases, the tumor was multifocal. A surgical approach was considered in the majority of pts with localized disease. Resection alone or in combination with doxorubicin-containing chemotherapy (CT) or with a radiotherapy and CT regimen was undertaken in 18, 43 and 16 pts respectively. Among these pts, surgical approaches included wide excision (n=10), marginal excision (n=22), intralesional excision (n=31) and heart transplant (n=5). The median follow up was 13 mo (0-153), the median overall survival (OS) for those who underwent R0 excision was 39 mo (range 8-96) versus 17 mo (range 0-153) for those with R1 or R2 resection. The median OS of pts with initial metastatic disease treated with doxorubicin-containing CT was 11 mo (range 2-95). Conclusions: Complete surgical excision is associated with long-term survival. However, the poor prognosis of this malignancy requires extensive multidisciplinary research studies.
Read full abstract