Natural history, response to systemic therapy, and genomic landscape of plasmacytoid urothelial carcinoma Journal Article


Authors: Teo, M. Y.; Al-Ahmadie, H.; Seier, K.; Tully, C.; Regazzi, A. M.; Pietzak, E.; Solit, D. B.; Tickoo, S.; Reuter, V.; Cha, E. K.; Herr, H.; Donahue, T.; Donat, S. M.; Dalbagni, G.; Bochner, B. H.; Funt, S.; Iyer, G. V.; Bajorin, D. F.; Ostrovnaya, I.; Rosenberg, J. E.
Article Title: Natural history, response to systemic therapy, and genomic landscape of plasmacytoid urothelial carcinoma
Abstract: Background: Plasmacytoid urothelial carcinoma (PUC) is a rare, aggressive histologic variant of urothelial cancer characterised by a diffuse growth pattern and CDH1 mutation. We studied the efficacy of preoperative platinum-based chemotherapy in nonmetastatic PUC and immune checkpoint inhibitors (ICIs) in advanced PUC. Methods: Cases of nonmetastatic PUC and advanced PUC treated with ICIs at our institution were identified. Outcomes were compared to those of a published cohort of patients with urothelial carcinoma not otherwise specified. Results: We identified 81 patients with nonmetastatic PUC. Of the patients with localised disease who underwent neoadjuvant chemotherapy, pathologic complete response and downstaging rates were 12 and 21%, respectively. Pathologic downstaging was not associated with significant improvement in clinical outcomes. Up to 18% of localised disease and 28% of locally advanced cases had unresectable disease at the time of surgery. ICI-treated advanced PUC (N = 21) had progression-free and overall survival of 4.5 and 10.5 months, respectively, and a 38% response rate. FGFR3 and DNA damage response gene alterations were observed in 3 and 15% of cases, respectively. Conclusions: PUC is associated with high disease burden and poor chemosensitivity. Increased awareness and recognition of this disease variant will allow for new treatment strategies. © 2021, The Author(s), under exclusive licence to The Author(s), under exclusive licence to Cancer Research UK.
Keywords: adult; cancer survival; treatment response; aged; major clinical study; overall survival; mutation; clinical feature; cisplatin; advanced cancer; systemic therapy; gemcitabine; paclitaxel; cancer patient; carboplatin; progression free survival; multiple cycle treatment; cohort analysis; fibroblast growth factor receptor 3; genomics; platinum; dna damage response; clinical effectiveness; inoperable cancer; neoadjuvant chemotherapy; transitional cell carcinoma; clinical outcome; preoperative chemotherapy; immune checkpoint inhibitor; human; male; female; priority journal; article; plasmacytoid urothelial carcinoma; triplet chemotherapy
Journal Title: British Journal of Cancer
Volume: 124
Issue: 7
ISSN: 0007-0920
Publisher: Nature Publishing Group  
Date Published: 2021-03-30
Start Page: 1214
End Page: 1221
Language: English
DOI: 10.1038/s41416-020-01244-2
PUBMED: 33473164
PROVIDER: scopus
DOI/URL:
Notes: Article -- Export Date: 3 May 2021 -- Source: Scopus
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MSK Authors
  1. Dean Bajorin
    563 Bajorin
  2. Guido Dalbagni
    289 Dalbagni
  3. Sherri M Donat
    148 Donat
  4. David Solit
    618 Solit
  5. Satish K Tickoo
    416 Tickoo
  6. Gopakumar Vasudeva Iyer
    205 Iyer
  7. Bernard Bochner
    388 Bochner
  8. Victor Reuter
    1120 Reuter
  9. Harry W Herr
    535 Herr
  10. Ashley Regazzi
    58 Regazzi
  11. Jonathan Eric Rosenberg
    362 Rosenberg
  12. Timothy Francis Donahue
    32 Donahue
  13. Eugene K. Cha
    77 Cha
  14. Samuel Aaron Funt
    70 Funt
  15. Min Yuen   Teo
    58 Teo
  16. Eugene J Pietzak
    49 Pietzak
  17. Kenneth Seier
    53 Seier