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plasmacytoid urothelial carcinoma

Tuesday 2 November 2010

CD138-positive plasmacytoid urothelial carcinoma

Plasmacytoid urothelial carcinoma is a rare form of invasive urothelial carcinoma first described in 1991 by Sahin et al. [Acta Cytol 1991;35:277-280]. Since this original publication, over 70 cases of plasmacytoid urothelial carcinoma have been described.

The WHO classification of 2004 defines new histological and molecular variants of urothelial carcinoma. However there are limited data available on the clinico-pathological characteristics or prognosis of these variants.

In summary, PUC is a aggressive variant of bladder cancer with molecular features of advanced bladder cancer and evidence of WNT pathway activation in some of the cases.

Synopsis

- high-grade tumor
- molecular features of aggressive urothelial carcinoma
- usually diagnosed in advanced pathological stage (64% pT3, 23% pT4)
- metastases in 60 % of the patients
- CD138 is positive in 78 %
- typical dyscohesive tumor cells with perinuclear clearing reminiscent of plasma cells
- cohesive nests of tumor cells with prominent nuclear pleomorphism and conspicuous nucleoli

Prognosis

Average survival of PUC treated with radical cystectomy and adjuvant chemotherapy is lower than what is typically seen for comparable conventional urothelial carcinomas.

PCV exhibits a unique pattern of spread along the ureter. This proposes a new mode of invasion along the fascial sheath. It may lead to a paradigm shift, with surgeons and pathologists being more vigilant with surgical margins. (24582117)

Immunochemistry (20878954)

- CD138 is positive in 78 %

  • One of the diagnostic pitfalls in the cytologic evaluation of this rare malignancy is the positive staining with CD138.
  • While CD138 is a marker for plasma cell differentiation, it is also positive in plasmacytoid urothelial carcinoma.

- 87% of the PUCs show a negative or strongly reduced membranous staining of E-cadherin. (20878954, 20818341)
- Beta-catenin staining is negative in 22.5 % and 16.7% of the remaining tumors show nuclear accumulation.
- Aberrant CK20 expression (negative or >10% of cells stained) and negative CK7 staining is found in 100% and 22.6 %, respectively.
- 97% reveal positive staining for PAN-CK.
- MUM-1 expression is negative in all cases.

Cytogenetics (20878954)

Multi-target fluorescence in situ hybridization showed all PUCs to be highly aneuploid and polysomic.

Deletions on chromosome 9p21 seem to play an important role in this variant.

Molecular biology (20878954)

- FGFR3 and PIK3CA mutation analyses yield no mutations in any of the PUCs analyzed.
- TP53 mutation analysis show mutations in 29%.

Open references

- Plasmacytoid variant of bladder cancer defines patients with poor prognosis if treated with cystectomy and adjuvant cisplatin-based chemotherapy. Keck B, Wach S, Stoehr R, Kunath F, Bertz S, Lehmann J, Stöckle M, Taubert H, Wullich B, Hartmann A. BMC Cancer. 2013 Feb 8;13:71. doi : 10.1186/1471-2407-13-71 PMID: 23394492 [Free]

- Plasmacytoid urothelial carcinoma, a chemosensitive cancer with poor prognosis, and peritoneal carcinomatosis. Dayyani F, Czerniak BA, Sircar K, Munsell MF, Millikan RE, Dinney CP, Siefker-Radtke AO. J Urol. 2013 May;189(5):1656-61. doi : 10.1016/j.juro.2012.11.084 PMID: 23159581 [Free]

- Plasmacytoid urothelial carcinoma of the bladder metastatic to the stomach: a case report. Nabbout P, Furr J, Paari M, Slobodov G. Case Rep Urol. 2012;2012:715951. doi : 10.1155/2012/715951 PMID: 22953154 [Free]

- Isolated cardiac metastasis from plasmacytoid urothelial carcinoma of the bladder. Peck JR, Hitchcock CL, Maguire S, Dickerson J, Bush C. Exp Hematol Oncol. 2012 Jun 24;1(1):16. doi : 10.1186/2162-3619-1-16 PMID: 23210798 [Free]

References

- Plasmacytoid bladder cancer: variant histology with aggressive behavior and a new mode of invasion along fascial planes. Kaimakliotis HZ, Monn MF, Cheng L, Masterson TA, Cary KC, Pedrosa JA, Foster RS, Koch MO, Bihrle R. Urology. 2014 May;83(5):1112-6. doi : 10.1016/j.urology.2013.12.035 PMID: 24582117

- Plasmacytoid urothelial carcinoma, a chemosensitive cancer with poor prognosis, and peritoneal carcinomatosis. Dayyani F, Czerniak BA, Sircar K, Munsell MF, Millikan RE, Dinney CP, Siefker-Radtke AO. J Urol. 2013 May;189(5):1656-61. doi : 10.1016/j.juro.2012.11.084 PMID: 23159581

- Plasmacytoid urothelial carcinoma of the bladder: a rare malignancy. Philippou P, Kariotis I, Volanis D, Ploumides A, Delakas D. Urol Int. 2011;86(3):370-2. doi : 10.1159/000322389 PMID: 21325783

- The plasmacytoid carcinoma of the bladder - rare variant of aggressive urothelial carcinoma. Keck B, Stoehr R, Wach S, Rogler A, Hofstaedter F, Lehmann J, Montironi R, Sibony M, Fritsche HM, Lopez-Beltran A, Epstein JI, Wullich B, Hartmann A. Int J Cancer. 2010 Sep 28. PMID: 20878954

- E-cadherin expression in plasmacytoid, signet ring cell and micropapillary variants of urothelial carcinoma: comparison with usual-type high-grade urothelial carcinoma. Lim MG, Adsay NV, Grignon DJ, Osunkoya AO. Mod Pathol. 2010 Sep 3. PMID: 20818341

- Plasmacytoid urothelial carcinoma of the bladder. Lopez-Beltran A, Requena MJ, Montironi R, Blanca A, Cheng L. Hum Pathol. 2009 Jul;40(7):1023-8. PMID: 19297008

- Plasmacytoid urothelial carcinoma: detailed analysis of morphology with clinicopathologic correlation in 17 cases. Nigwekar P, Tamboli P, Amin MB, Osunkoya AO, Ben-Dor D, Amin MB. Am J Surg Pathol. 2009 Mar;33(3):417-24. PMID: 19011563