Plasmocytoid carcinoma of the bladder is a rare histological variant of urothelial carcinoma. We present a recent discovery clinical observation, referring to the data of the literature. The objective of this work is to define the clinical, histo-pathological and therapeutic characteristics of this pathology. . Treatment is usually based on total cystoprostatectomy followed by adjuvant cisplatin chemotherapy. The interest of neoadjuvant chemotherapy has not yet been demonstrated.

Plasmocytoid urothelial carcinoma of the bladder is a rare histological variant of urothelial carcinoma that has been included in whose classification of tumors since 2004 [1]. In the literature, only a hundred cases have been described to date. Available data suggest that this histological type is aggressive and most often diagnosed at an advanced stage. We report two new cases of urothelial plasmocytoid bladder carcinoma, in addition to a review of the literature and an analysis of different series dealing with this topic.

Observation
A case of urothelial plasmocytoid carcinoma was diagnosed and managed in our Urology Department. The patient was 65 years old. Hematuria was the tell-tale symptom of the disease in chronic smokers.A bladder ultrasound made discovered a large mass of budding tissue in the bladder(figure 1), practical cystoscopy reveals a large non-papillary tumor occupying the entire wall of the bladder.The patient underwent transurethral resection of the bladder in June 2019, a histopathologic result in favor of an undifferentiated tumor process, the immunohistochemical profile is compatible with a plasmocytoid variceal urothelial carcinoma. Tumor classed pTNIM = pT1 at least. The patient was returned to the emergency department again with hematuria with stable hemodynamic parameters and underwent a second transurethral resection of the bladder in September 2019, and the histopathological findings reveal this time urothelial carcinoma sarcomatoid. The patient was treated with total cystoprostatectomy. Histological analysis of the specimen, supplemented by an immunohistochemical study, confirmed the diagnosis of plasmacytoid urothelial carcinoma, and was sent to the cancer ward for further treatment by adjuvant chemotherapy. The fish died a few days later.

Plasmocytoid urothelial carcinoma of the bladder is a rare histological variant of urothelial carcinoma. This histological type has been described for the first time by Sahin et al. In 1991 [2] and since then, only a few cases have been reported in the literature, ranging from single cases to series of patients, the largest of which included 32 patients [3].

The average age at diagnosis is usually 64.77 years, with a clear male predominance. Clinical symptomatology is not specific, but is dominated by hematuria, with or without irritative signs of the lower urinary tract. At cystoscopy, the tumor can be single or multiple and can sit anywhere in the bladder.

Therefore, the diagnosis of plasmacytoid urothelial carcinoma is purely histological, with the presence of small cells with hyperchromatic eccentric nucleus and whose abundant eosinophilic cytoplasm contains very small mucous vacuoles resembling plasma cells [4]. This plasmocytoid cell morphology can be also observed in other bladder tumors such as lymphomas, plasmocytomas, neuroendocrine carcinomas, rhabdomyosarcomas, multiple myelomas, vesical metastases of breast or stomach cancer and chronic cystitis [5,8]. In case of doubt about the diagnosis, the use of an immunohistochemical examination is essential. The positivity of the cell staining with the anti-CK-7, CK-20, AE1 / AE3, EMA and CD-138 antibodies, associated with labeling negativity for LCA, S100, HMB45, κ, λ and CD79-α, confirms the diagnosis of urothelial plasmocytoid carcinoma [6,9].

Due to its rarity, the treatment of plasmocytoid urothelial carcinoma is still subject to debate. The disease is usually diagnosed at an advanced stage. Most authors propose cisplatin-based chemotherapy [7,10,]. In spite of an initial response to this chemotherapy, the results remain pejorative, since the median overall survival is 13.4 months [11]. For patients with non-metastatic resectable tumor at the time of diagnosis, total cystoprostatectomy or anterior pelvectomy in women, followed by adjuvant cisplatin-based chemotherapy, appears to be optional.In the Lopez-Beltran series [7], 7 patients treated with cystectomy alone died of the disease (within 2 to 11 months); Only 1 of 3 patients who received adjuvant chemotherapy after cystectomy died of the disease (after 8 months), while the 2 others were in remission (8 and 16 months later). In the Fritsche series [10], the patient treated with cystectomy alone died of his disease after 14 months. Two patients in this series received adjuvant chemotherapy: the first one still lives without disease recovery (at 16 months), while the other died (of pulmonary embolism) after 29 months. Finally, Dayyani [11] reports a median overall survival of 45.8 months in 14 patients who received neo-adjuvant or adjuvant chemotherapy for cystectomy.In this last series, the authors did not note any significant difference between the survival time of patients who received neo-adjuvant chemotherapy and patients initially treated with cystectomy [11]. All of these results suggest a benefit. potential of adjuvant chemotherapy for non-metastatic resectable forms.In our series, the patient did not present lymph node metastases or remotely, which is why after radical surgery he was sent to the cancer ward for further treatment.

Plasmocytoid urothelial carcinoma of the bladder is a rare and aggressive histological form of bladder tumors. The diagnosis is often made at an advanced stage, and the prognosis is not very encouraging. Treatment is usually based on total cystoprostatectomy followed by adjuvant cisplatin chemotherapy. The interest of neoadjuvant chemotherapy has not yet been demonstrated.