International Journal of Clinical Oncology

, Volume 16, Issue 6, pp 759–762

Two cases of plasmacytoid variant of urothelial carcinoma of urinary bladder: systemic chemotherapy might be of benefit

Authors

  • Takuji Hayashi
    • Department of UrologyOsaka General Medical Center
  • Go Tanigawa
    • Department of UrologyOsaka General Medical Center
    • Department of UrologyOsaka General Medical Center
  • Ryoichi Imamura
    • Department of UrologyOsaka General Medical Center
  • Shigeaki Nakazawa
    • Department of UrologyOsaka General Medical Center
  • Yoshiyuki Yamamoto
    • Department of UrologyOsaka General Medical Center
  • Masahiro Hosomi
    • Department of UrologyOsaka General Medical Center
  • Kohki Shimazu
    • Department of PathologyOsaka General Medical Center
  • Hiroaki Fushimi
    • Department of PathologyOsaka General Medical Center
  • Seiji Yamaguchi
    • Department of UrologyOsaka General Medical Center
Case Report

DOI: 10.1007/s10147-011-0240-4

Cite this article as:
Hayashi, T., Tanigawa, G., Fujita, K. et al. Int J Clin Oncol (2011) 16: 759. doi:10.1007/s10147-011-0240-4

Abstract

We report two cases of the plasmacytoid variant of urothelial carcinoma of urinary bladder in which systemic chemotherapy was effective. In the first case, a 76-year-old man presented with dysphasia. Magnetic resonance imaging (MRI) and computed tomography revealed a brain tumor and a bladder tumor. Resection of the brain tumor and transurethral resection of the bladder tumor were performed. The pathological diagnosis was plasmacytoid variant of urothelial carcinoma of urinary bladder with brain metastasis (pT1N0M1). Three cycles of adjuvant MVAC (methotrexate, vinblastine, adriamycin, and cisplatin) chemotherapy were performed. He has no evidence of recurrence 96 months after resection of brain metastasis. In the second case, a 76-year-old man presented with hematuria. MRI revealed a bladder tumor with abdominal wall invasion, and a transurethral biopsy was performed. The pathological diagnosis was plasmacytoid variant of urothelial carcinoma of urinary bladder (cT4bN0M0). After three cycles of neoadjuvant GC (gemcitabine and cisplatin) chemotherapy, MRI demonstrated a complete response. Radical cystectomy was performed, and the pathological diagnosis was pT0pN0. Although there was no evidence of recurrence 9 months after radical cystectomy, he died from other causes. Our two cases suggest that systemic chemotherapy might be effective for the plasmacytoid variant of urothelial carcinoma.

Keywords

Urothelial carcinomaPlasmacytoid variantChemotherapy

Introduction

The plasmacytoid variant of urothelial carcinoma is a rare histological variant, which is included in the 2004 WHO classification of urothelial carcinoma [1]. The prognosis of the variant is uniformly poor with most patients having an advanced stage of disease at presentation and metastatic disease progression. A treatment strategy for the variant has not been established. We present two cases of plasmacytoid variant of urothelial carcinoma of urinary bladder in which systemic chemotherapy was effective.

Case reports

Case 1

A 76-year-old man was admitted to our hospital with dysphasia. Brain magnetic resonance imaging (MRI) revealed an enhanced tumor in the left parietal lobe (Fig. 1a). Abdominal computed tomography (CT) revealed a bladder tumor (diameter 2.5 cm) (Fig. 1b). Urine cytology showed discohesive cells which had eccentrically located nuclei with increased nuclear/cytoplasmic (N/C) ratio. Cystoscopy revealed a solid tumor in the right wall of urinary bladder. Resection of the brain tumor and transurethral resection of the bladder tumor were performed. The pathological findings in the brain tumor were discohesive proliferation of tumor cells which had eccentric nuclei (Fig. 2a). The tumor cells were positive for cytokeratin AE1/AE3, and negative for CEA, vimentin, desmin, αSMA, and S-100 protein. The pathological findings in the bladder tumor were the same plasmacytoid cells as the brain tumor cells (Fig. 2b) in major part, and poorly differentiated urothelial carcinoma in minor part. There was no evidence of invasion across the muscle layer. The bladder tumor cells were positive for cytokeratin 7 and negative for E-cadherin. CT and bone scintigraphy revealed no sign of metastases except for brain. He was diagnosed as plasmacytoid variant of urothelial carcinoma of urinary bladder with brain metastasis (pT1N0M1), and three cycles of adjuvant MVAC (methotrexate, vinblastine, adriamycin, and cisplatin) chemotherapy were performed. Since carcinoma in situ (CIS) of urinary bladder occurred 2 months after chemotherapy, he had undergone eight procedures of Bacillus Calmette-Guérin (BCG) instillation into the urinary bladder. Eight years after chemotherapy, he has no evidence of recurrence or metastases.
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Fig. 1

Enhanced MRI of brain (T1-weighted image) showed a tumor in the left parietal lobe (a) in case 1, and CT revealed a bladder tumor at the right wall (b)

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Fig. 2

Histological findings of brain (a) and bladder tumor (b) showed discohesive proliferation of tumor cells with eccentric nuclei in case 1. H&E ×200

Case 2

A 76-year-old man presented with hematuria. Urine cytology showed discohesive cells which had eccentrically located nuclei with increased N/C ratio. Cystoscopy revealed a solid tumor in the right wall of the urinary bladder. MRI showed a massive tumor at the right wall of the urinary bladder with invasion to the abdominal wall (Fig. 3a). CT showed no swelling of lymph nodes and no distant metastases. He was diagnosed as invasive bladder carcinoma (cT4bN0M0), and a transurethral biopsy was performed. The pathological findings were discohesive proliferation of tumor cells which had eccentric nuclei in major part (Fig. 4), and poorly differentiated urothelial carcinoma in minor part, with infiltration of tumor cells into smooth muscle bundles. The tumor cells were positive for cytokeratin 7 and negative for E-cadherin. The pathological diagnosis was plasmacytoid variant of urothelial carcinoma. Three cycles of neoadjuvant GC (gemcitabine and cisplatin) chemotherapy were performed. MRI demonstrated marked size reduction of the tumor (Fig. 3b), and a complete response (CR) was achieved. Radical cystectomy was performed, and the pathological findings were no residual tumor in urinary bladder and no metastases of pelvic lymph nodes. Although there was no evidence of recurrence 9 months after radical cystectomy, he died from other causes.
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Fig. 3

MRI (T2-weighted image) revealed a massive tumor at the right wall of urinary bladder with invasion to the abdominal wall (a) at presentation in case 2. After neoadjuvant chemotherapy, MRI (T2-weighted image) demonstrated no residual tumor in the bladder (b)

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Fig. 4

Histological findings of bladder tumor showed that tumor cells with eccentric nuclei proliferated discohesively in case 2. H&E ×40

Discussion

The histological features of the plasmacytoid variant of urothelial carcinoma are characterized by the presence of discohesive oval or round malignant cells. The tumor cells have eccentrically placed nuclei and eosinophilic cytoplasm. The first case was reported by Sahin et al. [2], and there was up to 3% incidence of the variant in a large series of muscle-invasive urothelial carcinomas [3, 4].

The variant is positive for cytokeratin 7 and 20 in the differential diagnosis with metastatic carcinoma, and loss of E-cadherin is described [4]. Recent studies have indicated that the loss of E-cadherin expression was associated with a loss of cellular differentiation and increased cellular invasiveness, and might correlate with muscular involvement and tumor recurrence [5].

Seventy-one cases of the plasmacytoid variant of urothelial carcinoma have been reported in the English literature. The clinical stages of 30 cases were unclear, 5 cases were clinical stage III, 35 cases were clinical stage IV, and most patients had advanced stage at presentation. Radical cystectomy was performed in 32 cases, and systemic chemotherapy was performed in 40 cases. Among the 71 cases, 25 patients died within 12 months, though the outcomes of 15 cases were unclear. The prognosis of the variant is poor [4].

The therapeutic approach in patients with the variant remains debatable because of the rarity of the tumor [4]. Radical cystectomy is warranted as the first-line treatment for muscle-invasive nonmetastatic urothelial carcinoma, including the variant of urothelial carcinoma. Disseminated distant disease carries a poor prognosis and no therapy has proved to be effective.

The data in published cases of the variant suggested a possible benefit of systemic chemotherapy [4]. There were 3 cases of the plasmacytoid variant in which systemic chemotherapy was reported to be effective. Sahin et al. [2] reported a case with bone metastasis in which there had been no signs of recurrence for 18 months after MVAC chemotherapy. Zukerberg et al. [6] reported a case in which there had been no signs of recurrence for 18 months after systemic chemotherapy. Kohno et al. [7] reported a case of clinical T4N0M0 in which neoadjuvant MVAC chemotherapy was very effective, resulting in pathological CR, and there had been no signs of recurrence for 36 months after radical cystectomy.

Our two cases also suggest that systemic chemotherapy might be effective for the plasmacytoid variant of urothelial carcinoma. In case 1 with brain metastasis, there have been no signs of recurrence for 96 months after MVAC chemotherapy. In case 2 of clinical T4N0M0, neoadjuvant GC chemotherapy resulted in pathological CR. More cases would be required to evaluate the effectiveness of systemic chemotherapy for the plasmacytoid variant of urothelial carcinoma.

Conclusions

The prognosis of the plasmacytoid variant has been uniformly poor in most patients with advanced stage of disease at presentation. However, we experienced two cases of plasmacytoid variant of urothelial carcinoma of urinary bladder in which cisplatin-based systemic chemotherapy was effective. Systemic chemotherapy might be effective for urothelial carcinoma with the plasmacytoid variant.

Conflict of interest

No author has any conflict of interest.

Copyright information

© Japan Society of Clinical Oncology 2011