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plasmacytoid urothelial carcinoma

Tuesday 2 November 2010

CD138-positive plasmacytoid urothelial carcinoma

Definition: 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.

Images

- https://twitter.com/jpett88/status/723588707006222337
- https://twitter.com/Gleason4plus5/status/718180200211734528

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

Differential diagnosis

- tumoral plasmacytoid cells

  • mammary lobular carcinoma
  • diffuse gastric adenocarcinoma

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