Dataset Information

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.

SUBMITTER: Teo MY

PROVIDER: S-EPMC8007750 | biostudies-literature | 2021 Mar

REPOSITORIES: biostudies-literature

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Publications

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

Teo Min Yuen MY Al-Ahmadie Hikmat H Seier Kenneth K Tully Christopher C Regazzi Ashley M AM Pietzak Eugene E Solit David B DB Tickoo Satish S Reuter Victor V Cha Eugene K EK Herr Harry H Donahue Timothy T Donat Sherri M SM Dalbagni Guido G Bochner Bernard H BH Funt Samuel S Iyer Gopakumar V GV Bajorin Dean F DF Ostrovnaya Irina I Rosenberg Jonathan E JE

British journal of cancer 20210121 7

<h4>Background</h4>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.<h4>Methods</h4>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 pa ...[more]

PMID: 33473164

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Dataset Information

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

Background

Methods

Results

Conclusions

Publications

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

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