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Primary Bladder Sarcoma: A multi-institutional experience from the Rare Cancer Network. | LitMetric

Primary Bladder Sarcoma: A multi-institutional experience from the Rare Cancer Network.

Arch Ital Urol Androl

Department of Radiation Oncology, Instituto de Radiomedicina, Santiago; Facultad de Medicina, Universidad Diego Portales, Santiago.

Published: July 2023

AI Article Synopsis

  • Primary sarcoma of the urinary bladder (SUB) is a rare and aggressive type of bladder cancer, with limited research available; this study aimed to analyze its clinical features, treatments, and outcomes across multiple institutions.
  • A total of 53 patients were reviewed, with the median age being 69 years, and common symptoms including hematuria and pelvic pain; treatments varied but often involved surgery, with significant recurrence rates.
  • Overall, the study found that SUB tends to present at advanced stages, with a 5-year overall survival rate of 66.5% and a progression-free survival rate of 37.6%, highlighting the complexity and aggressive nature of the disease.

Article Abstract

Purpose Or Objective: 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.

Materials And Methods: 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.

Results: 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.

Conclusions: 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.

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Source
http://dx.doi.org/10.4081/aiua.2023.11533DOI Listing

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