primary small - cell carcinoma of the bladder ( smccb ) is extremely rare , accounting for less than 1% of all bladder cancer cases [ 1 , 2 ] . smccb is much more aggressive than urothelial carcinoma ( uc ) , the most common type of bladder cancer , and it has a worse prognosis . herein , we report a case of smccb that progressed rapidly after radical cystectomy ( rc ) . most cases of smccb are histologically diagnosed on final pathologic examination after rc or transurethral resection of bladder tumor ( turbt ) . alternatively , it is rare that suspected smccb is diagnosed on preoperative urine cytology , as in the presented case . a 61-year - old japanese woman presented to our urology outpatient clinic with the chief complaint of gross hematuria . the urine cytology was class v. urinary cytology findings revealed clusters of small tumor cells , resembling neuroendocrine carcinoma ( fig 1 ) . cystoscopy revealed a broad - based solitary nodular bladder tumor on the anterior bladder wall . plain and enhanced computed tomography ( ct ) scans and magnetic resonance imaging ( mri ) showed a broad - based solitary nodular tumor on the anterior wall of the bladder with a diameter of 54  50  24 mm , indicating extracystic invasion without distant or lymph node metastasis ( fig 2 ) . microscopically , the specimen displayed the features of a neuroendocrine neoplasm or small - cell carcinoma . it displayed small , round or oval - shaped tumor cells , a nest - like structure , little cytoplasm , marked hyperchromatic nuclei , a rough granular karyosome , frequent massive mitotic figures , and extensive necrosis . immunohistochemically , the tumor cells stained positive for cluster of differentiation ( cd ) 56 , chromogranin a , and synaptophysin , ( fig 3a  c ) partially positive for cytokeratin , and positive for ki-67 . in addition , a brain ct was performed to rule out potential brain metastasis , a characteristic of small - cell carcinoma , but brain metastasis was not observed . as a result the patient underwent rc and ileal conduit urinary diversion and standard lymph node dissection under general anesthesia . the results of the final pathological examination of the bladder tumor were as follows : primary smccb on cystectomy specimen , pt3b , n1 , lymphovascular invasion , and negative resection margins . the patient had no complications after rc and she was discharged on day 24 after rc . immediate adjuvant systemic cisplatin - based chemotherapy was planned for the patient , but she was admitted to our hospital with back pain in an emergency . the patient died from disease just 2 months after rc , without being able to receive adjuvant systemic chemotherapy due to the rapid progression and highly aggressive behavior of the disease . it accounts for less than 1.0% of all primary bladder carcinomas [ 1 , 2 ] . in a retrospective study of 3,778 bladder cancer cases , the male : female prevalence ratio of smccb is 5 : 1 , and the average age of onset for smccb is approximately 67 years ( range : 3291 years ) [ 3 , 4 ] . symptoms of smccb are similar to those of uc , especially hematuria , which occurs at a rate of 90% . other common symptoms include dysuresia , urinary obstruction , chronic pelvic pain , and urinary tract infection . the diagnosis mainly depends on the pathological diagnosis and the immunohistochemical analysis of turbt or rc specimens . preoperative urine cytology may also be beneficial in the diagnosis , although the specificity of this method is low . reported that 67.4% of 43 smccb patients had class iv or v preoperative urine cytology , but smccb was suspected in only three cases ( 6.9% ) , based on the urine cytology . in the present case , smccb was initially suspected based on urine cytology features ; these included clusters of small tumor cells with little cytoplasm that resembled neuroendocrine carcinoma and marked hyperchromatic nuclei ( fig 1 ) . therefore , preoperative urine cytology may be an important auxiliary diagnostic tool for smccb , contributing to the early diagnosis of smccb . early diagnosis of smccb is very important because , due to the rapid progression and poor prognosis of the disease , treatment must be initiated as soon as possible . smccb is biologically much more aggressive than uc , with early metastasis and poor prognosis . .   showed that 98% of smccb patients had muscle - invasive disease , and 66% had lymph node metastasis at the time of rc . in a study of 27 patients with smccb , choong et al . demonstrated that the distribution of stage ii , iii , and iv disease was 27.3 , 29.6 , and 43.2% , respectively . in that study , the 3- and 5-year survival rates were 20 and 813.5% , respectively , and the mean survival time was only 9.419.6 months . the characteristics and prognosis for patients with smccb at our institution are presented in table 1 . between 2000 and 2015 , a total of 263 patients underwent rc at our institution , and of these , four patients ( 1.5% ) had smccb . the mean survival time in those four cases was 9.75 months , which is consistent with previous reports . surprisingly , two of the four patients with smccb died just 2 months after undergoing rc , although there was no evidence of metastasis on ct or mri prior to surgery . in the present case , persistent gross hematuria with high t stage was identified using ct and mri . in such cases , when systemic chemotherapy is not effective , it was expected that the patient 's quality of life would be markedly worsened due to bladder tamponade or frequent transfusions . therefore , rc was performed as the initial treatment , after which immediate adjuvant chemotherapy was scheduled . options for local treatment include turbt , rc , and radiation , similar to those for uc . it has been reported that simple turbt treatment may result in uncontrolled disease , high recurrence rates , and poor survival periods of 36 months . in addition , cheng et al .   demonstrated that there was no difference in the 5-year survival rate between patients who underwent rc and those who did not ( 15 vs. 18% , p = 0.65 ) . these studies showed that surgery alone , even rc , does not confer any survival benefit for the majority of patients with smccb . on the other hand , multimodal therapies using a combination of neoadjuvant chemotherapy ( nac ) and rc may improve long - term survival . smccb is a systemic disease , even if it does not initially manifest as such , and its cells respond to platinum - based chemotherapy . it can be down - staged by nac and chemosensitive micrometastases can be treated , resulting in extended survival periods . siefker - radtke et al .   showed that patients with smccb who received a combination of nac and rc had longer survival periods than the patients who underwent rc alone ( 5-year survival rate : nac + rc , 78% ; rc alone , 36% ) . in another study by the same authors , it was demonstrated that pathological downstaging occurred via nac in 78% of patients , resulting in a median overall survival of 58 months . demonstrated even more favorable results for the smccb patients at their institution , with a median survival of 159.5 months and a 5-year survival rate of 79% . some studies reported that chemoradiotherapy may be used as an alternative to nac and rc . reported that the 5-year survival rate in the 10 smccb patients in their study was 44% after chemoradiotherapy . reported complete response in 88% of 17 smccb patients who underwent chemoradiotherapy after turbt , with a median overall survival time of 32.5 months . in summary , based on these previous studies , systemic chemotherapy before local treatments such as rc and radiation may represent a key treatment strategy for patients with smccb . in fact , in the present study , adjuvant chemotherapy after rc had been scheduled for the two smccb patients ( 50% ) at our institution who died only 2 months after rc , but the disease progressed too rapidly and the adjuvant therapy could not be administered . systemic chemotherapy as soon as possible after a diagnosis of smccb appears to be an important parameter in reducing chemosensitive micrometastases , even if metastasis is not observed at diagnosis and the patients appear suitable for surgery . in that regard , this resulted in a survival period of 13 months after rc , which is shorter than that reported in previous studies . early initiation of systemic chemotherapy before local treatment such as rc or radiation is a key treatment strategy for smccb . consent for publication of this case report was obtained from the patient at the outpatient clinic at our institution .
primary small - cell carcinoma arising from the bladder ( smccb ) is uncommon . 
 it differs from urothelial carcinoma ( uc ) , the most common type of bladder cancer , with respect to its cell of origin , biology , and prognosis . 
 biologically , prostatic smccb is much more aggressive than uc , and the prognosis for cases with distant metastasis is especially poor . 
 we report here a case of primary smccb ( ct3bn1m0 ) treated with radical cystectomy .