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 |
| PMCID: | PMC8007750 |
| DOI/URL: | |
| Notes: | Erratum issued, see DOI: [10.1038/s41416-022-01721-w] -- Source: Scopus |
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