Plasmocytoid variant urothelial carcinoma of urinary bladder, series from Kaohsiung Veterans General Hospital
Bing-Tau Chen, Jen-Tai-Lin
Divsion of Urology, Depart of Surgery, Kaohsiung Veterans General Hospital
Introduction: Plasmocytoid variant urothelial carcinoma (PVUC) is a rare histological variant which was associated with aggressive disease. 57% of patients survived for only a short time (mean, 23 months), despite the multimodal therapy. We presented our series and some did well after immunotherapy.
Materials and Method:
From Decembr 2011 to October 2019, six cases of plasmacytoid variant urothelial carcinoma were diagnosed at our institution. The brief histories were summarized below.
Case 1 was a 69 y/o male with ESRD. He was diagnosed to have plasmacytoid variant UC over left ureter (pT3N0M0) and died of disease metastasis 5 months after the surgery. Case 2 was a 65 y/o female presenting with right hydronephorsis and multiple retroperioneal lymph adenopathies CT scan revealed subtle infiltrative UB lesions and plasmocytoid variant UC was confirmed after TURBT. Systemic dd-MVEC chemotherapy gained short-term response and she died of disease 15 months later. Case 3 was a 62-year-old male who was diagnosed as muscular invasive PVUC of UB at presentation of hematuria. He underwent radical cystectomy (RC) with neobladder followed by adjuvant chemotherapy (dd-MVEC). He remained disease-free at present for 26 months. Case 4 was a 64-year-old male who developed muscular invasive PUVC of UB as his UB UC recurred and progressed. He received neoadjuvant chemotherapy (dd-MVAC) followed by RC with neobladder. Par-aortic lymph node metastasis developed 9 months later. He then received Pembrolizumab immunotherapy, complete response was noted at present after 9 courese of Pembrolizumab. Case 5 was a 66-year-old female with cancers over left renal pelvis and urinary bladder. The pathologic report showed the UB cancer was PVUC of stage I after surgery. Adjuvant intravesical BCG instillation was done and she remained disease free at 14 months follow-up. Case 6 was a 66 y/o male presenting diffuse PVUC infiltration of UB causing bilateral hydronephrosis. The disease soon spreading to bilateral ureters and peritoneal cavity in 2 months and is receiving GC chemotherapy.
Three of our series had extravesical invasion at presentation and rapid disease progression. These were compatible with literature. Two had T2 or lower stage at resection and seemed to be good at 14 and 26 months follow-up. The rest one was diagnosed to have conventional UC in the beginning and plasmacytoid variant developed at his third recurrence. He had ever hesitancy to receive treatment and the disease was pT3N2 while he received radical surgery. Fortunately the disease seemed to response to pembrolizumab at present.
Conclusions: The plasmacytoid variant UC is rare but lethal especially it extends beyond the organ. There may be opportunity of multi-modality treatment while the disease remained organ-confined. Definitely aggressive plays a role.