183P Surgical management and long-term outcomes of spinal and sacral chordomas: A 19-year single-institution experience

therapy, and treatment responses were documented.Data were analyzed using SPSS v.30. Results:Of 20 patients analyzed, 2 were secondary (IVC leiomyomatosis and dermatofibrosarcoma protuberans).Among 18 primary cardiac sarcomas, the median age at diagnosis was 33.5 years and 11 (61%) were male.Dyspnea (61%) and chest pain (39%) were the most common symptoms.The index presentation involved pericardial involvement (effusion 50%, tamponade 11.1%), congestive cardiac failure (27.7%), and arrhythmias (11.1%).9 (50%) patients were misdiagnosed, including tubercular pericarditis (n=7), hydatid disease (n=1), skeletal tuberculosis (n=1).Metastatic disease at presentation was in 6 patients (lungs: 4/6, 66.6%).Right-sided tumors predominated (n=13) (right atrium: 10, 76%; right ventricle: 3, 23%).Most common histological subtypes were angiosarcoma 8 (44.4%), leiomyosarcoma 2 (11.1%) and synovial sarcoma 2 (11.1%).Surgical resection was done in 7 patients, (R2 resection: 6, 85.7%).Systemic therapy was administered to 11 patients [median number of lines: 1(range: 1-4)].Among these, 6 (54.5%) received gemcitabine, doxorubicin 4 (36.3%),pazopanib 3 (27.2%),nab-paclitaxel 3 (27.2%)and ifosfamidedoxorubicin 3 (27.2%).Median OS was 5.1 months with a 12-month OS of 31%.OS did not differ significantly for metastatic vs. non-metastatic subgroups (7.0 vs. 5.0 months) or for angiosarcoma vs. non-angiosarcoma subgroup (5.0 vs. 5.1 months).Conclusions: Primary cardiac sarcomas often presents late with frequent msisdiagnosis and poor survival.Angiosarcoma predominated.Outcomes remained poor despite surgery and systemic therapy, underscoring the urgent need for earlier detection and better treatment strategies.