Adult genitourinary sarcomas: A single-centre experience over 25 years

Abdul Baseet Arham; Michael A. O'Donnell; Sarah L. Mott

doi:10.1200/JCO.2024.42.16_suppl.e23532

e23532 Background: Genitourinary sarcomas are a rare entity. This study is a retrospective review of the patient characteristics, types of adult GU sarcomas, treatments and outcomes in a single institution over the past 25 years. Methods: Survival probabilities were estimated and plotted using the Kaplan-Meier method. Estimates along with 95% pointwise confidence intervals were reported. Recurrence-free survival (RFS) is defined as time from when the patient was disease-free to recurrence. Otherwise, patients were censored at last disease evaluation. OS was defined as time from when the patient was disease-free to death due to any cause. Patients still alive were censored at last known alive. Results: 29 patients presented with various types of GU sarcomas, with the majority being paratesticular tumors (n = 22, 75.9%). Others included bladder leiomyosarcomas (n = 5), prostatic leiomyosarcoma (n = 1), renal leiomyosarcoma (n = 1). Median age for diagnosis was 51 years (range: 21-79) for leiomyosarcoma vs 61 (range: 36-89) for liposarcoma. Among the paratesticular tumors majority were (n = 16) were liposarcoma, remaining 5 being leiomyosarcoma and 1 being rhabdomyosarcoma. Majority tumors were stage III and above (n = 16, 55.17%), high and intermediate grade (65.51%). All tumors were resected ± adjuvant therapy (n = 4). Leiomyosarcomas had a higher stage (66%) and grade (83%) at diagnosis, recurrence rate was also higher at 37.5% (vs 6.6% for liposarcoma) along with a higher mortality (41.6%). Commonest site of recurrence for leiomyosarcoma was the lungs (n = 3, 100%). Treatment for recurrence commonly involved radiotherapy, pembrolizumab etc. Paratesticular liposarcomas were found to be associated with a lipomatosis syndrome with n = 5 (31.25%) having lipomas elsewhere in the body and n = 2 (12.5%) patients having had previously resected spermatic cord lipoma. Paratesticular sarcomas often required (n = 15, 68%) revision surgery due to being initially diagnosed as a hernia, cord lipoma or hydrocele and requiring high ligation of the cord with hemiscrotectomy and radical orchiectomy ± LND on revision surgery, which often demonstrated a focus of residual tumor in liposarcomas (n = 5, 31%). N = 4 patients (25%) had positive margins in spite of revision surgery. Majority of these were MDM2+ positive on FISH (n = 9, 56%). Overall, GU sarcomas had an RFS of 63% (53-94%) and OS of 82% (53-94%) at 36 months since NED was achieved. The median length of follow-up was 19.9 months for leiomyosarcoma, 33.8 months for liposarcoma. Conclusions: Leiomyosarcomas in adults have worse outcomes compared to other GU sarcomas especially if it originates in the bladder. [Table: see text]

Adult genitourinary sarcomas: A single-centre experience over 25 years

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