editorial comment
TRANSCRIPT
PLASMACYTOID UROTHELIAL CANCER AND PERITONEAL CARCINOMATOSIS1660
term survival is seen in only relatively few patientswith T4a or less nonmetastatic tumors (stage I–III).These outcomes are much worse than would typicallybe expected in patients with a more traditional urothe-lial histology. In such patients 5-year OS and diseasespecific survival are around 63% and 68%, respec-tively, even in those with clinical features that placethem at high risk for stage III or greater disease.15
In patients with resectable bladder cancer whounderwent cystectomy without chemotherapy a me-dian OS of 5 to 15 years was reported depending ontumor T stage.16 Although it is difficult to comparesurvival rates between our cohort and the reportedcohort due to sample size and other factors, it isremarkable that despite neoadjuvant chemotherapyin our cohort the patients with resectable diseasehad a median OS of only less than 4 years. Com-pared with nonPUC bladder cancer, there was alsoinferior survival for stage IV. While up to 20% ofpatients with stage IV conventional urothelial car-cinoma might be alive at 5 years,16 none with stageIV PUC in our study survived beyond 24 months.
The most common site of recurrence in these pa-
tients was the peritoneum and in some an initialREFERENCES
EDITORIAL COMMENT
largest series to date of PUC, a rare variant with
surge in serum CA-125 preceded radiological andsymptomatic findings of progression. Thus, our de-scription of the peritoneum as the primary site ofrecurrence might suggest followup with serial se-rum CA-125 measurement.17 This would aid in rec-ognizing early disease progression and possibly inbeginning second line therapy sooner, before pa-tients become symptomatic.
CONCLUSIONS
PUC is a rare tumor with a locally infiltrative pattern.The prognosis remains poor with few long-term survi-vors despite neoadjuvant chemotherapy. Patients areat a high risk for relapse in the peritoneal lining. Thediagnosis of peritoneal carcinomatosis should be con-sidered in patients who present with abdominal symp-toms. Tumor markers, including CEA, CA-125 andCA19-9, may be useful in this disease. Responses totraditional urothelial cancer regimens, including dosedense MVAC, are of short duration. To our knowledgeit is still unknown whether that is the optimal chemo-
therapy regimen for this cancer.1. Montironi R and Lopez-Beltran A: The 2004 WHOclassification of bladder tumors: a summary andcommentary. Int J Surg Pathol 2005; 13: 143.
2. Mai KT, Park PC, Yazdi HM et al: Plasmacytoidurothelial carcinoma of the urinary bladder reportof seven new cases. Eur Urol 2006; 50: 1111.
3. Keck B, Stoehr R, Wach S et al: The plasmacytoidcarcinoma of the bladder—rare variant of ag-gressive urothelial carcinoma. Int J Cancer 2011;129: 346.
4. Kohno T, Kitamura M, Akai H et al: Plasmacytoidurothelial carcinoma of the bladder. Int J Urol2006; 13: 485.
5. Lopez-Beltran A, Requena MJ, Montironi R et al:Plasmacytoid urothelial carcinoma of the bladder.Hum Pathol 2009; 40: 1023.
6. Nigwekar P, Tamboli P, Amin MB et al: Plasma-cytoid urothelial carcinoma: detailed analysis ofmorphology with clinicopathologic correlation in17 cases. Am J Surg Pathol 2009; 33: 417.
7. Raspollini MR, Sardi I, Giunti L et al: Plasmacy-
clinicopathologic, immunohistochemical,ultrastructural, and molecular analysis of a caseseries. Hum Pathol 2011; 42: 1149.
8. Ro JY, Shen SS, Lee HI et al: Plasmacytoidtransitional cell carcinoma of urinary bladder: aclinicopathologic study of 9 cases. Am J SurgPathol 2008; 32: 752.
9. Kaplan E and Meier P: Nonparametric estimationfrom incomplete observations. J Am Stat Assoc1958; 53: 457.
10. Cox D: Regression models and life tables (withdiscussion). J Roy Stat Soc B 1972; 34: 187.
11. Siefker-Radtke AO, Dinney CP, Abrahams NA etal: Evidence supporting preoperative chemother-apy for small cell carcinoma of the bladder: aretrospective review of the M.D. Anderson cancerexperience. J Urol 2004; 172: 481.
12. Siefker-Radtke AO, Kamat AM, Grossman HB etal: Phase II clinical trial of neoadjuvant alternatingdoublet chemotherapy with ifosfamide/doxorubicinand etoposide/cisplatin in small-cell urothelial can-
cytoid component
13. McConkey DJ, Lee S, Choi W et al: Moleculargenetics of bladder cancer: emerging mecha-nisms of tumor initiation and progression. UrolOncol 2010; 28: 429.
14. Fritsche HM, Burger M, Denzinger S et al: Plas-macytoid urothelial carcinoma of the bladder:histological and clinical features of 5 cases.J Urol 2008; 180: 1923.
15. Siefker-Radtke AO, Dinney CP, Shen Y et al: Aphase 2 clinical trial of sequential neoadjuvantchemotherapy with ifosfamide, doxorubicin, andgemcitabine followed by cisplatin, gemcitabine,and ifosfamide in locally advanced urothelial can-cer: final results. Cancer 2012; 119: 540.
16. Stein JP, Lieskovsky G, Cote R et al: Radicalcystectomy in the treatment of invasive bladdercancer: long-term results in 1,054 patients. J ClinOncol 2001; 19: 666.
17. Topalak O, Saygili U, Soyturk M et al: Serum,pleural effusion, and ascites CA-125 levels inovarian cancer and nonovarian benign and malig-nant diseases: a comparative study. Gynecol On-
toid urothelial carcinoma of the urinary bladder: cer. J Clin Oncol 2009; 27: 2592. col 2002; 85: 108.
Urothelial carcinoma represents greater than 90%of bladder cancer diagnoses with variants such asPUC reported. These authors describe one of the
more than 100 cases in the literature (references 3and 7 in article). This retrospective analysis of 31patients diagnosed with greater than a 50% plasma-
confirms the aggressive nature of
PLASMACYTOID UROTHELIAL CANCER AND PERITONEAL CARCINOMATOSIS 1661
this disease with a median overall survival of 17.7months despite neoadjuvant chemotherapy. Definedby invasive single cells that mimic plasma cells,these carcinomas are diagnostically challengingsince they express plasma cell markers in additionto cytokeratins.1 The authors identified multiplechallenges in bladder cancer diagnosis and treat-ment, including 1) the importance of recognizing
REFERENCE
ior, 2) the necessity of developing new therapiestailored to the distinct appearance of and/or molec-ular alterations in bladder cancer variants, and 3)the relationship between variant morphology andthe background bladder cancer subtype.
Donna E. Hansel
The Cleveland Clinic
rare variants that may have distinct clinical behav- Cleveland, Ohio1. Grignon D and El-Bolkainy MN: Infiltrating Urothelial Carcinoma: Lymphoma-Like and Plasmacytoid Variants. Lyon: IARC Press 2004.