Primary sarcoma of the urinary bladder (SUB) is a rare but aggressive form of bladder cancer (BCa). Available evidence on SUB is limited to case reports and small series. The aim of the present multi-institutional study was to assess the clinical features, treatments, and outcomes of patients with SUB.
Using a standardized database, 7 institutions retrospectively collected the demographics, risk factors, clinical presentation, treatment modalities and follow-up data on patients with SUB between January 1994 and September 2021. The main inclusion criteria included BCa with soft tissue tumor histology and sarcomatoid differentiation.
Fifty-three patients (38 men and 15 women) were identified. Median follow-up was 18 months (range 1-263 months). Median age at presentation was 69 years (range 16-89 years). Twenty-six percent of patients had a prior history of pelvic radiotherapy (RT), and 37% were previous smokers. The main presenting symptoms at diagnosis were hematuria (52%), pelvic pain (27%), and both hematuria and pelvic pain (10%). American Joint Committee on Cancer (AJCC) 8 th edition stage II, III and IV at diagnosis were 21%, 63% and 16%, respectively. Treatment modalities included surgery alone (45%), surgery plus neo- or adjuvant-chemotherapy (17%), surgery plus neo- or adjuvant-RT (11%), RT with concurrent chemotherapy (4%), neo-adjuvant chemotherapy plus surgery plus adjuvant RT (2%) and palliative treatment (21%). Rates of local and distant recurrences were 49% and 37%, respectively. Five-year overall survival and progression-free survival (PFS) were 66.5% and 37.6%, respectively. No statistically significant differences in PFS between the treatment modalities were observed.
Primary SUB is a heterogeneous disease group, commonly presenting at advanced stages and exhibiting aggressive disease evolution. In contrast to urothelial carcinoma, the primary pattern of recurrence of SUB is local, suggesting the need for multimodal approaches. Continuous international collaborative efforts seem warranted to provide guidance on how to best tailor treatments based on SUB-specific indices.
Archivio italiano di urologia, andrologia : organo ufficiale [di] Societa italiana di ecografia urologica e nefrologica. 2023 Jul 25 [Epub ahead of print]
Piero Bettoli, ZhihuiAmy Liu, Natalia Jara, Federico Bakal, William Wong, Mario Terlizzi, Paul Sargos, Thomas Zilli, Juliette Thariat, Sebastian Sole, Guilaume Ploussard, Sharad Goyal, Peter Chung, Alejandro Berlin, Claudio Sole
Department of Radiation Oncology, Fundación Arturo López Pérez, Santiago; Facultad de Medicina, Universidad de Los Andes, Santiago. ., Radiation Medicine Program, Princess Margaret Cancer Centre, University Health Network, University of Toronto, Toronto, ON. ., Department of Radiation Oncology, Instituto de Radiomedicina, Santiago. ., Department of Radiation Oncology, Fundación Arturo López Pérez, Santiago. ., Department of Radiation Oncology, Mayo Clinic Arizona, Phoenix. ., Department of Radiation Oncology, Institute Bergonie, Bordeaux. ., Department of Radiation Oncology, Institute Bergonie, Bordeaux. ., Department of Radiation Oncology, Geneva University Hospital, Geneva. ., Department of Radiation Therapy, Centre Francoise Baclese, Caen. ., Department of Radiation Oncology, Instituto de Radiomedicina, Santiago; Facultad de Medicina, Universidad Diego Portales, Santiago. ., Department of Urology, La Croix du Sud Hospital, Toulouse. ., Department of Radiation Oncology, George Washington University Hospital, Washington DC. ., Radiation Medicine Program, Princess Margaret Cancer Centre, University Health Network, University of Toronto, Toronto, ON. ., Radiation Medicine Program, Princess Margaret Cancer Centre, University Health Network, University of Toronto, Toronto, ON. ., Department of Radiation Oncology, Instituto de Radiomedicina, Santiago; Facultad de Medicina, Universidad Diego Portales, Santiago. .
PubMed http://www.ncbi.nlm.nih.gov/pubmed/37491942