|Year : 2016 | Volume
| Issue : 1 | Page : 36-38
Plasmacytoid urothelial carcinoma of the urinary bladder
Department of Pathology, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India
|Date of Web Publication||13-Jan-2016|
Type VI, Set No.14, IAS Colony, Maheli, Shimla - 171 009, Himachal Pradesh
Source of Support: None, Conflict of Interest: None
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 in a 78-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 with diffuse muscle and vascular invasion, in which almost of the areas studied on the tissue section showed the plasmacytoid appearance of tumor cells. The tumor cells showed positivity for CK7 and CD138. The diagnostic dilemmas of this unusual variant of urothelial malignancy and its clinical impact are discussed.
Keywords: Plasmacytoid variant, urinary bladder, urothelial carcinoma
|How to cite this article:|
Mardi K. Plasmacytoid urothelial carcinoma of the urinary bladder. Int J Health Allied Sci 2016;5:36-8
| Introduction|| |
Plasmacytoid urothelial carcinoma of the bladder (PUC) is a rare variant of bladder carcinoma which was included as a distinct variant of infiltrating urothelial carcinoma in World Health Organization (WHO) classification of bladder tumor in 2004. It is an aggressive variant with poor prognosis. Morphological distinction from other malignant neoplasms with plasmacytoid phenotype is critical for its clinical management. Treatment remains a challenge because of late presentation of the disease, and the presence of metastasis at the time of initial work up.,,,,,
| Case Report|| |
A 78-year-old male presented with pain in the right lumbar region for 1 year and an episode of hematuria 1 year back. The pain was insidious in onset and was radiating to the groin and right flank. He had a history of smoking 10 cigarettes per day for last 12 years. Cystoscopy revealed edema and ulceration of the entire bladder mucosa. CT scan of abdomen and pelvis showed nodular thickening of the right lateral wall of the urinary bladder with irregularity of the mucosa involving entire bladder. Other pelvic and abdominal organs were unremarkable. Transurethral bladder biopsy was done. Microscopic examination showed discohesive plasmacytoid tumor cells arranged in cords, single-file pattern, small nests, solid sheets, and diffuse discohesive pattern-less architecture in a loose mixed stroma [Figure 1]. These tumor cells revealed eccentric irregular, hyperchromatic nuclei, variably prominent nucleoli, and abundant eosinophilic cytoplasm with distinct cytoplasmic borders. Occasional tumor giant cells and frequent mitotic figures were seen. These tumor cells were diffusely infiltrating the lamina propria and were invading the muscle layer. There were areas revealing tumor necrosis and lymphovascular invasion by the tumor cells. On immunohistochemistry, (IHC) the tumor cells were positive for CK 7 [Figure 2] and CD138 [Figure 3]; while they were negative for leukocyte common antigen (LCA), CD20, k and λ light chains.
|Figure 1: The malignant cells have eccentric nuclei with eosinophilic cytoplasm giving them a plasmacytoid appearance. The stroma shows a myxoid character|
(H and E, ×40)
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|Figure 2: Tumor cells showing positivity for CK7 (Immunohistochemistry, ×40)|
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|Figure 3: Tumor cells showing positivity for CD138 (Immunohistochemistry, ×40)|
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| Discussion|| |
The PUC of the bladder represents one of several rare variants of urothelial carcinoma, which were included in the WHO classification in 2004. The earliest description of this variant came from the case reported by Sahin et al., in 1991 in which the tumor was mistakenly considered as multiple myeloma at first, because of multiple lytic tumors involving the bone of the ribs and skull. Since then, not more than 65 cases have been reported.
According to the reported cases, the mean age of initial diagnosis is 69 years (range 46–89), and the constituent ratio shows a male predominance (M: F = 3:1).,,,,,,,, The most common presenting symptom is hematuria. It is an aggressive variant associated with poor prognosis and presents at an advanced clinical stage. Treatment remains a challenge because of late presentation of the disease, and the presence of metastasis at the time of initial work up.,,,,,
Histopathologically, PUC is characterized by plasmacytoid tumor cells arranged in cords and single-file pattern, small nests, solid sheet-like growth, or diffuse discohesive pattern-less architecture. Cytoplasm is moderate to abundant and amphophilic to eosinophilic, with eccentrically placed nuclei. The nuclei vary from hyperchromatic to vesicular with evenly distributed chromatin and variability in the prominence of nucleoli. There is overall monotony of the nuclear feature., Stromal reaction in the form of prominent myxoid stroma is noted. Prominent retraction artifact around each tumor cell may also be seen. In the present case, almost all these findings were prominent. The PUC can coexist with noninvasive papillary urothelial cancer, carcinoma in situ,, or invasive high- grade urothelial carcinoma.
Morphological distinction from other malignant neoplasms with plasmacytoid phenotype is critical for its clinical management. One of these diseases is chronic bladder inflammation with abundant plasma cells, but the more prominent cytological anaplasia feature distinguishes PUC from chronic bladder inflammation. Another disease is primary signet ring cell adenocarcinoma because of its occasional presence of intracytoplasmic vacuoles, and the noncohesive nature of the tumor cells. The signet ring cells may permeate the wall of the urinary bladder in a manner similar to linitis plastica, but the lack of mucin staining helps to distinguish PUC from signet ring-cell carcinoma. Likewise, carcinoma with rhabdoid phenotype is considered in the differential diagnosis, its diagnosis is mostly based on cytoplasmic features and prominent nucleolus, and PUC in rhabdoid cells is mainly determined by positive immunostaining of vimentin. From the pathology point of view, caution should be taken in the evaluation of neoplasms with plasmacytoid morphology, and we suggest that ancillary tests such as IHC and electron microscopic studies are needed to document the cell of origin. From the clinical point of view, PUC have three salient features that are helpful to reach its diagnosis. These are high histological grade, high stage, and rapid progression to death.
When the histological appearance is highly suspicious for this variant of urothelial carcinoma, IHC is a key for making the diagnosis. Looking like plasma cells, these cells also stain positively for CD138, a plasma cell marker. However, they also stain positively for epithelial markers like cytokeratin and epithelial membrane antigen but not for hematopoietic markers such as CD79a, kappa, and lambda light chain. Zhang et al. studied 16 cases of PUC and concluded that IHC study with positivity for CK7, CK20, p63, and uroplakin III and negative staining for vimentin and LCA may be helpful in the differential diagnosis. In a recent case report by Shimada et al. was the first to show urothelial carcinoma with a plasmacytoid variant producing both CA19-9 and beta human chorionic gonadotropin in the absence of trophoblastic tissue.
Prognosis is extremely poor with an aggressive behavior of this variant. Studies comparing PUC and conventional UC usually show a worse prognosis of PUC than UC, if treated with cystectomy and adjuvant cisplatin-based chemotherapy. In the case series by Nigwekar et al., no patient with greater than 1-year follow-up survived the disease. Another report by Lopez-Beltran et al., showed that within 2 years of follow-up, all patients either died of cancer or had metastasis. In our case, the patient is free of disease since last 6 months after surgery.
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[Figure 1], [Figure 2], [Figure 3]