plasmacytoid urothelial carcinoma is a rare malignant neoplasm in the urinary bladder recognized by recent who classification of urothelial carcinoma .
it is an aggressive variant associated with poor prognosis that presents at an advanced clinical stage .
limited data is available about the pathological , immunohistochemical characteristic as well as clinical behavior of this rare variant .
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 presence of metatstasis at the time of initial work up.[27 ] we present here a case of 54-year - old male patient who was referred to our hospital for diagnostic work - up of bladder cancer .
ct scan of abdomen and pelvis showed thickened urinary bladder wall with irregularity of the mucosa involving entire bladder .
radical cystectomy was done which on gross examination showed a grey white firm to hard , ill - circumscribed ulcerative tumor involving almost the entire bladder measuring 2 cms in thickness .
microscopic examination showed a high - grade tumor composed of discohesive plasmacytoid cells replacing the lamina propria [ figures 1 and 2 ] .
the tumor cells were seen extending from the mucosal aspect and invaded through the detrusor muscle to invade the perivesical fat and the serosa [ figure 3 ] .
the tumor cells were seen extending along the adventitia of both the ureters even up to the surgical cut margins .
on immunohistochemistry the tumor cells were positive for ck , ck 7 [ figure 4 ] and cd 138 ; while they were negative for lca , cd20 ,  and  light chains .
plasmacytoid urothelial carcinoma : the tumor cells are seen undermining the normal urothelium with retraction spaces around them .
immunohistochemical staining with antibody to ck7 marks the tumor cells as well the overlying urothelium thereby confirming their epithelial origin .
the patient is on regular follow - up and is doing fine for last 6 months without any evidence of recurrence or metastasis elsewhere in the body .
recent years have witnessed the illustrations and documentation of several morphological variants of urothelial carcinomas , which have significance from therapeutic and prognostic perspective .
the plasmacytoid variant is an unusual variant that has been recognized in the latest who classification of urothelial carcinomas .
earliest description of this variant came from the case reported by sahin et al , in 1991 and since then , not more than 40 cases have been reported,[26 ] there is very limited data available about the pathological , immunohistochemical characteristic as well as clinical behavior of this rare variant .
most common presenting symptom is hematuria.[48 ] histopathologically , plasmacytoid urothelial carcinoma 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 .
the nuclei vary from hyperchromatic to vesicular with evenly distributed chromatin and variability in the prominence of nucleoli .
prominent retraction artifact around each tumor cell may also be seen . in the present case
the plasmacytoid urothelial carcinoma can coexist with non - invasive papillary urothelial carcinoma , urothelial carcinoma in situ or invasive high - grade urothelial carcinoma .
however , in our case there were no areas of either papillary or in situ urothelial malignancy .
plasmacytoid appearance of this tumor can lead to diagnostic dilemma , especially in smaller biopsies .
differential diagnosis ranges from benign conditions like chronic cystitis with prominent plasma cell infiltrate to malignant tumors like plasmacytoma and signet ring carcinoma . in our case
some cells also had focal signet ring - like appearance which led to confusion with signet ring cell carcinoma .
however , the nuclei were eccentric rather then being peripherally compressed as in true signet ring cell carcinoma .
metastatic carcinoma from breast or stomach , malignant melanoma and rhabdomyosarcoma can have striking plasmacytoid morphology and can cause considerable diagnostic difficulties . in such situations , it is imperative to employ a panel of antibodies for confirmation of diagnosis .
immunohistochemical studies have shown that plasmacytoid urothelial carcinoma cells to be positive for ck-7 , ck-20 , ck , ae1/ae3 , ema and cd-138 , but negative for lca , s 100 , hmb 45 ,  ,  and cd 79- , as was seen in our case also . because of late presentation , biopsy usually reveals a high - grade tumor infiltrating the lamina propria and muscularis , with high potential of metastasis .
some reports have shown good response to neoadjuvant chemotherapy , but this aspect needs further evaluation .
our patient also had high - stage disease ( pt3 ) and underwent radical cystectomy with ileal conduit . on final histopathology
, the tumor cells were seen extending along the ureteric adventitia till the surgical resection margins .
prognosis is extremely poor with an aggressive behavior of this variant . in the case series by nigwekar et al , no patient with greater than 1-year
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 . in conclusion ,
plasmacytoid variant of urothelial carcinoma is a rare and important variant form diagnostic , therapeutic and prognostic point of view .
it can be mistaken for other pathologies of bladder having plasmacytoid features and should be differentiated from them because of the difference in the therapeutic approach .
 plasmacytoid urothelial carcinoma is an uncommon and aggressive variant of urothelial carcinoma associated with late presentation and poor prognosis . 
 we discuss here the first reported case from india of a 54-year - old male who presented with hematuria . 
 cystoscopy showed edematous and ulcerated mucosa throughout the bladder . 
 a transurethral biopsy revealed urothelial carcinoma with plasmacytoid appearance . 
 he underwent a radial cystectomy which on histopathology showed plasmacytoid urothelial carcinoma of the bladder of high stage with involvement up to bladder serosa and adventitial walls of the ureter . 
 the diagnostic dilemmas of this unusual variant of urothelial malignancy and its clinical impact are discussed . 
INTRODUCTION
CASE REPORT
DISCUSSION