Article
Author: Dhillon, Jasreman ; Poch, Michael A ; Sexton, Wade J ; Spiess, Philippe E. ; Pow-Sang, Julio ; Zemp, Logan W ; Zhang, Jingsong ; Manley, Brandon J. ; Yamoah, Kosj ; Torres-Roca, Javier F ; Jain, Rohit K. ; Manley, Brandon J ; Zemp, Logan W. ; Linkowski, Lauren ; Grass, G Daniel ; Xu, Hongzhi ; Johnstone, Peter A.S. ; Yu, Alice ; Sim, Austin J. ; Liveringhouse, Casey ; Gilbert, Scott M ; Naidu, Shreyas U. ; Sexton, Wade J. ; Poch, Michael A. ; Jain, Rohit K ; Vosoughi, Aram ; Grass, G. Daniel ; Li, Roger ; Sim, Austin J ; Spiess, Philippe E ; Naidu, Shreyas U ; Gilbert, Scott M. ; Torres-Roca, Javier F. ; Johnstone, Peter A S
BACKGROUNDNeuroendocrine carcinoma of the bladder (NEC-bladder) is a rare disease with poor outcomes and variable treatment approaches.MATERIALS AND METHODSPatients with localized NEC-bladder treated with surgery or radiation between 2001-2021 were retrospectively identified. Rates of pathologic complete response (pCR) and downstaging were evaluated following NAC in surgically-treated patients. Progression-free survival (PFS) and overall survival (OS) were analyzed with univariable (log-rank) and multivariable (MVA; Cox regression) methods.RESULTSSixty-five patients were identified having a median age of 73. The tumor histology distribution was small cell (64.6%) or urothelial with NE differentiation (35.4%). Most patients (69.2%) received NAC. Patients received local therapy by surgery (78.5%) or chemoradiation (21.5%). The majority (62.7%) of surgical patients had ≥ pT2 with 37.3% having nodal involvement (pN+). The pCR and downstaging rates were 21.6% and 35.1%, respectively. At a median follow-up of 60 months (m), the median PFS and OS were 16.4m and 25.9m, respectively. NAC improved PFS (p=0.04) and downstaging improved PFS (p=0.012) and OS (p<0.001). Patients receiving NAC with ypN0 vs. ypN+ had median OS of 69.9m vs 15.3m, respectively (p<0.001). MVA identified receipt of NAC and pN as predictors of PFS; pN was predictive of OS. No differences in PFS or OS were seen between histology of primary tumor. The brain metastasis rate was 10.8% with all patients having small cell histology.CONCLUSIONSOptimized therapy in NEC-bladder includes NAC followed by local consolidation. Ascertainment of ypN0 is associated with long term survival, while pN+ remains associated with poor outcomes.