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| Year : 2014 | Volume
: 57
| Issue : 2 | Page : 320-322 |
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| Plasmacytoid urothelial carcinoma of the bladder |
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Meihua Qin1, Gangping Wang2, Yanqing Sun1, Qun He3
1 Department of Urology, Rizhao People's Hospital, Rizhao, China
2 Department of Pathology, Rizhao People's Hospital, Rizhao, China
3 Department of Pathology, Peking University First Hospital; Institute of Urology, Peking University, Beijing, China
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| Date of Web Publication | 19-Jun-2014 |
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 Abstract | | |
Plasmacytoid urothelial carcinoma (PUC) of the urinary bladder is an uncommon and aggressive variant of urothelial carcinoma associated with late presentation and poor prognosis. Immunohistochemical examination showing expression of epithelial markers, CD138, and losing the membranous expression of E-cadherin confirms evidence of PUC. Here, we report a case of bladder PUC of a 74-year-old male who presented with hematuria. A transurethral biopsy revealed urothelial carcinoma with plasmacytoid appearance. The diagnostic dilemmas of this unusual variant of urothelial malignancy and its clinical impact are discussed. The pathological diagnosis was PUC (High-grade, pT4N2M0) with diffuse muscle, small tracts, and vascular invasion, in which almost of the areas studied on the tissue section showed Plasmacytoid differentiation. Keywords: CD138, E-cadherin, plasmacytoid, urothelial carcinoma
How to cite this article:
Qin M, Wang G, Sun Y, He Q. Plasmacytoid urothelial carcinoma of the bladder. Indian J Pathol Microbiol 2014;57:320-2 |
| Introduction | |  |
Plasmacytoid urothelial carcinoma (PUC) is an uncommon and aggressive variant of urothelial carcinoma (UC) associated with late presentation and poor prognosis. PUC of the bladder is a recently identified rare variant of UC with histological characteristics similar to plasma cells. [1],[2] Very few cases of PUC have been reported in the literature.
| Case report | | |
A 74-year-old Chinese male presented with the chief complaint of a 3-week history of diffuse lower abdominal pain, associated with 4 days of urological symptoms including macroscopic hematuria, urgency, and frequency. The patient was a cigarette smoker with a history of smoking 20 cigarettes per day for 40 years. Urine cytology revealed a scant number of atypical cells, with frequent presence of tumor diathesis [Figure 1]a]. Urinary ultrasonography and computerized tomography showed a large intravesical mass associated with irregular thickening of the left lateral wall and the trigone of the bladder with possible extravesical extension [Figure 1]b]. Transurethral bladder biopsy revealed a malignant urothelial tumor with plasmacytoid appearance [Figure 1]c]. Immunohistochemical staining of the tumor sections showed positive for CK7 [Figure 1]d], weakly positive for CD138, but negative for several lymphoid markers including CD38, MUM-1, LCA. The patient was therefore diagnosed as a high-grade UC with plasmacytoid differentiation. Radical cystectomy was done which on gross examination showed an ulcerated irregular growth neoplasm measuring 6.4 × 4.3 × 2.5 cm. Cut surface of the tumor was variegated with solid gray-white areas, yellowish areas as well as friable necrotic area. Histologically, the tumor was found to penetrate throughout the entire bladder wall into the serosa, and the infiltrating malignant epithelial cells had mainly eccentric nuclei and abundant eosinophilic cytoplasm with characteristic plasmacytoid morphology with scattered atypical cells arranged in loose clusters on high power. The proportion of plasmacytoid variant UC differentiation was about 90%. The obturator lymph nodes and right iliac artery lymph nodes were found to be metastatic. The pathological diagnosis was PUC (high-grade, pT4N2M0) with diffuse muscle, small tracts, and vascular invasion. Immunohistochemical studies showed that the plasmacytoid tumor cells were positive for cytokeratin (CK) (including CK7, CK20 PCK, AE1/AE3) with different staining intensity ranging from slight to strong positivity. The tumor cells were also positive for epithelial membrane antigen (EMA) and weakly positive for CD138, while the tumor lost the membranous expression of E-cadherin as a molecular hall mark of PUC. The tumor was negative for several lymphoid markers (LCA, CD38, MUM-1, kappa, and lambda), and markers of embryonal rhabdomyosarcoma (desmin, SMA, myoglobin, MyoD1, and myogenin), malignant melanoma (HMB45, Melan A, S-100), as well as neuroendocrine carcinoma (ChrA, Syn, NSE, CD56). beta subunit of human chorionicgonadotropin (β-hCG), placental alkaline phosphatase (PLAP), and WT1 staining were also negative. Unfortunately, the patient died 8 months after the surgery and two cycles of adjuvant chemotherapy with MVAC (methotrexate, etoposide, vinblastine, and cisplatin). | Figure 1: (a) Urine cytology revealing a scant number of atypical cells, with frequent tumor diathesis (hematoxylin and eosin, ×400). (b) Pelvic computerized tomography scan showing a large intravesical mass associated with thickening of the left lateral wall of the bladder (Nonenhanced). (c) The tumor from the biopsy sample showing an invasive carcinoma mimicking plasmacytoma, and was mainly composed of round cells with abundant eosinophilic cytoplasm and eccentric nuclei (hematoxylin and eosin, ×200, Inset ×400). (d) Plasmacytoid urothelial carcinoma of the bladder showing CK7 positivity (DA B ×200)
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| Discussion | | |
PUC is a rare malignant neoplasm in the urinary bladder recognized by recent World Health Organization (WHO) classification of UC. PUC was reported mainly in Western countries in White race and described mostly in small case series, with the largest series having 32 cases reported by Keck et al., 19 cases reported by Gonzales-Roibon et al., and 17 cases reported by Nigwekar et al. [3],[4],[5],[6] The mean age of initial diagnosis is 68 years (range 42-89). [5] PUC is more common in males than in females (the constituent ratio shows a male predominance ratio of about 3:1). To the best of our knowledge, there are only 13 cases of PUC reported in cases without series in the Chinese literature with detailed pathological information. [7] The cases in China have the same clinical features as all other reported cases in Western countries in White race. However, the male-to-female ratio is 12:1 in China. Therefore, it is necessary for further study.
This variant has displayed aggressive behavior in the cases described thus far, usually diagnosed in advanced pathological stage (64% pT3, 23% pT4), showing metastases in 60% of the patients, although there is insufficient evidence to make any concrete recommendations from these small case series. [1],[4],[8] Studies comparing PUC and conventional UC usually show a worse prognosis of PUC than UC, if treated with cystectomy and adjuvant cisplatin-based chemotherapy. [4] Systemic cisplatin-based chemotherapy is regarded as the therapy of choice in metastatic UC. However, the role of adjuvant or neoadjuvant chemotherapy remains under debate. Most urological and oncological guidelines recommend neoadjuvant cisplatin-based chemotherapy as the therapy of choice in locally advanced bladder cancer. [9]
The patient is a tobacco smoker with history of smoking for more than 30 years. It is unknown whether smoking is also a potential risk factor for PUC, and more studies are needed to evaluate the potential risk of smoking for PUC. PUC is included in the divergent differentiation patterns. As a rare variant of UC with histological characteristics similar to plasma cells, however, most of the reports failed to quantify the divergent differentiation present within the specimen.
PUC is difficult to differentiate from signet ring cell adenocarcinoma of the urinary bladder due to overlap in clinical, morphological, and immunohistochemical presentation. These tumors initially present as a high-grade, high-stage lesion, and diffusely invade the bladder wall, and the patients have no specific symptoms, which leads to delayed diagnosis and poor prognosis. From the pathology point of view, caution should always be taken in evaluation of neoplasms with plasmacytoid morphology, and immunohistochemical study is needed to document the cell of origin. The individual tumor cells of PUC had striking morphologic overlap with plasma cells with an eccentrically placed nucleus and abundant amphophilic to eosinophilic cytoplasm. The nuclei of PUC were of low to intermediate nuclear grade with minimal nuclear pleomorphism. [6] Immunohistochemical staining plays an important role in the diagnosis of PUC. The panel of antibodies for immunophenotyping of the atypical neoplastic cells comprises EMA and CKs. The CKs with various molecular weights are expressed at different levels in PUC-diffuse positive for CK7, slightly focal positive for CK20, but negative for CK5/6. PUC is reported to be positive for CD138, a plasma cell marker. [10] In the Nigwekar's report, CD138 was positive in 94% of cases. Therefore, tumor cells positive for CD138 provides an evidence to support the diagnosis of PUC. This often has been utilized as a surrogate marker for PUC along with CK reactivity, which would help to rule out PUC from signet ring cell adenocarcinoma to the bladder. [6] In the Keck's report, 87% of the PUCs showed a negative or strongly reduced membranous staining of E-cadherin. β-Catenin staining was negative in 22.5%, and 16.7% of the remaining tumors showed nuclear accumulation. Aberrant CK20 expression (negative or >10% of cells stained) and negative CK7 staining was found in 100% and 22.6%, respectively. It has been noted that PUC has loss of E-cadherin expression, and the studies suggest that loss of E-cadherin expression is probably associated with increased cellular invasiveness or correlated with muscularis mucosal involvement and tumor recurrence. [4],[9] However, loss of E-cadherin is not entirely specific for PUC. It has been also reported in UC showing signet ring cell differentiation, a mimic of PUC. [10] The other differential diagnoses including small-cell carcinoma, malignant melanoma, and embryonal rhabdomyosarcoma are all ruled out because of the mucosal origin of the tumor.
| References | | |
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| 7. | Wang Z, Lu T, Du L, Hu Z, Zhuang Q, Li Y, et al. Plasmacytoid urothelial carcinoma of the urinary bladder: A clinical pathological study and literature review. Int J Clin Exp Pathol 2012;5:601-8.
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| 8. | Chalasani V, Chin JL, Izawa JI. Histologic variants of urothelial bladder cancer and nonurothelial histology in bladder cancer. Can Urol Assoc J 2009;3(6 Suppl 4):S193-8.
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| 9. | Stenzl A, Cowan NC, De Santis M, Kuczyk MA, Merseburger AS, Ribal MJ, et al. Treatment of muscle-invasive and metastatic bladder cancer: Update of the EAU guidelines. Eur Urol 2011;59:1009-18.
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| 10. | Lim MG, Adsay NV, Grignon DJ, Osunkoya AO. E-cadherin expression in plasmacytoid, signet ring cell and micropapillary variants of urothelial carcinoma: Comparison with usual-type high-grade urothelial carcinoma. Mod Pathol 2011;24:241-7.
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Correspondence Address:
Gangping Wang
Department of Pathology, Rizhao People's Hospital, 126 Taian Road, Rizhao, Shandong province, 276826
China
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0377-4929.134730
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