small cell carcinoma of the bladder is a very rare and aggressive histological subtype , and accounts for less than 1% of all bladder tumors.its management is challenging because it presents in the same way of other more frequent histological types , but with a high metastatic power . the diagnosis demands that the pathologist be suspicious and accurate in the analysis of the fragments of a transurethral bladder resection ( tur ) . multimodal treatment , including radical cystectomy , chemotherapy and radiation therapy should be initiated as soon as possible for a chance of cure and to improve survival . this kind of carcinoma presents metastasis and has a poor prognosis . in the present article we report a case of a male patient with small cell carcinoma of the bladder and the development of the condition , and we aim to show the most current management of this tumor , which has no consensus for treatment in the international literature for being extremely rare . patient jhc , male , 61 years old , came to the medical service with a complaint of hematuria and hypogastric pain for one year . the urinary tract ultrasound revealed an intravesical tumor , and he was submitted to bladder tur , with a conclusive report for small cell carcinoma of the bladder . faced with the diagnosis , the attending team chose to perform chemotherapy ( received four sessions of cisplatin , gemcitabine and paclitaxel ) ( figure 1 ) . figure 1field with the presence of multiple mitoses , salt - and - pepper nuclei 
  when referred to our service , he was submitted to thorax and abdomen computerized tomography ( ct ) that showed a 6.2x6.0x5.5 cm neoplasm on the left anterolateral wall of the bladder , suggesting invasion of the prostatic urethra . the patent was then submitted to radical cystectomy , with a bricker ileal derivation procedure , performed uneventfully , lasting 5 hours , with no need for blood transfusion . it is important to underscore that the time between the last chemotherapy and surgery was 2 months . the pathology report of the surgical specimen proved small cell carcinoma of the bladder with extravesical extension , perineural and vascular infiltration , and one lymph node affected ( left obturator ) out of 16 dissected . the patient had pneumonia , was on antibiotics and was discharged 29 days after surgery . he was referred to oncology to discuss adjuvant therapy which was not initiated , because the patient died 4 months after surgery due to pulmonary thromboembolism ( figure 2 ) . the first report of small cell carcinoma of the bladder was made in 1981 by cramer et al . since then , approximately 600 cases were registered in the international literature . it is characterized by its high aggressiveness and poor prognosis , and by the development of metastic disease in about 67% of patients . the small cell carcinoma of the bladder usually affects individuals of the same age , sex and presents the same symptoms and morphology of urothelial carcinoma . that is why it is a diagnostic challenge , depending only on the skills of the pathologist to differentiate it from urothelial carcinoma , which beside the factors already mentioned , has the same radiological aspect of small cell carcinoma of the bladder . thus , recommendations are based on retrospective studies , reports of individual cases , and protocols for small cell lung carcinoma . frequently , it presents with mixed histology.molecular studies show a common origin for small cell carcinoma of the bladder and urothelial carcinoma , when coexisting . however , management should be different , since it frequently presents metastases and has a poor prognosis . the pathological diagnosis of this kind of tumor is challenging and requires that the pathologist use immunohistochemical techniques for histological confirmation . as they are histologically identical , the world health organization standardization for small cell lung carcinoma is used . it comprises the following criteria for diagnosis of small cell carcinoma of the bladder : presence of group of small cells with scarce cytoplasm , few organelles ,  salt and pepper  chromatin and high rate of mitoses ( > 10 mitoses/10 high power fields ) . 
  on immunohistochemistry , these tumors express quite an amount of neurospecific enolase , chromogranin , synaptophysin and n - cam ( cd-56 ) . the presence of one or more markers allows to establish the diagnosis of neuroendocrine tumor . pathological staging is based on the consensus for urothelial carcinoma of the bladder ( figure 3 ) . figure 3marker cd-56 that characterizes neuroendocrine tumors 
  isolated therapies  whether tur , partial cystectomy or radiation therapy radical cystectomy is considered the best method to eliminate small cell carcinoma of the bladder completely , but it alone is believed to only change survival in stage i and ii tumors . reviewed 64 cases and concluded that in patients submitted to radical surgery compared to those treated with combined treatment ( whether only chemotherapy or radiation therapy or both as adjuvant ) , there was no increase in survival . in contrast , chemotherapy adjuvant to radical cystectomy resulted in a favorable prognosis in other studies , including meta - analyses . another alternative is neoadjuvant chemotherapy , recommended by the md anderson cancer center , which reported a 5-year disease free survival in 36% of the group submitted to radical cystectomy alone and of 78% in the group submitted to chemotherapy neoadjuvant to surgery . on the other hand , a canadian group reported ten patients with pt3-t4 , n0 lesions with chemotherapy , attaining complete remission in nine of them . the 2-year survival rate for this group was 70% and , 44% in 5 years . another similar series reported four patients treated with chemotherapy followed by radiation therapy who survived 27 to 60 months . the advantage of this treatment is sparing the organ . therefore , it is a less invasive treatment when compared to radical surgery , which is related to important rates of morbidity and mortality . patients with metastatic disease should receive systematic chemotherapy , and the most common treatment regimens are platinum - based ( cisplatin and etoposide , carboplatin , etoposide and cyclophosphamide ) ( figure 2 ) . given the extremely aggressive and rare disease , little is known about pathogenesis and molecular biology . data on the ideal approach for this kind of tumor are scarce , showing the importance of reporting such cases and , in this way , defining the best diagnostic and treatment methods . o carcinoma de clulas pequenas da bexiga  um subtipo histolgico muito raro e agressivo , correspondendo a menos de 1% de todos os tumores de bexiga.sua abordagem torna - se desafiadora , pois se apresenta da mesma forma que outros tipos histolgicos mais frequentes , porm com alto poder metasttico . o diagnstico exige suspeio e preciso do patologista na anlise dos fragmentos de resseco transuretral ( rtu ) de bexiga . o rpido tratamento multimodal , incluindo cistectomia radical , quimioterapia e radioterapia , deve ser institudo o mais precocemente possvel , para haver chance de cura e melhora da sobrevida.esse tipo de carcinoma apresenta metstase e tem prognstico ruim . neste artigo , relatamos o caso de um paciente do sexo masculino com carcinoma de clulas pequenas de bexiga e sua evoluo , assim como buscamos trazer o que h de mais atual no manejo desse tumor que , por sua extrema raridade , no apresenta consenso para o tratamento na literatura mundial . paciente jhc , masculino , 61 anos de idade , procurou servio mdico com queixa de hematria e dor hipogstrica h um ano . evidenciada tumorao intravesical em ultrassonografia do aparelho urinrio , foi submetido a rtu de bexiga , cujo laudo foi conclusivo para carcinoma de clulas pequenas de bexiga . frente a esse diagnstico , a equipe que o acompanhava optou por realizar quimioterapia ( recebeu quatro sesses de cisplatina , gencitabina e paclitaxel ) ( figura 1 ) . figura 1presena de mltiplas mitoses no campo , ncleos em sal e pimenta 
  ao ser encaminhado ao nosso servio , foi submetido  tomografia ( tc ) de trax e abdome , a qual mostrou neoplasia de 6,2x6,0x5,5 cm em parede anterolateral esquerda da bexiga , com sugestiva invaso de uretra prosttica . foi , ento , submetido  cistectomia radical , com derivao ileal  bricker , procedimento realizado sem intercorrncias , com durao total de 5 horas , sem necessidade de transfuso sangunea . importante ressaltar que o tempo da ltima quimioterapia para a cirurgia foi de 2 meses . o laudo anatomopatolgico da pea cirrgica comprovou carcinoma de clulas pequenas de bexiga com extenso extravesical , infiltrao perineural e vascular presentes , comprometimento de um linfonodo ( obturatrio esquerdo ) em 16 dissecados . no ps - operatrio apresentou pneumonia , sendo submetido a antibioticoterapia e recebendo alta hospitalar 29 dias aps a cirurgia . foi encaminhado  oncologia para discusso de tratamento adjuvante , que no foi iniciado , pois o paciente faleceu 4 meses aps a cirurgia devido a tromboembolismo pulmonar ( figura 2 ) . o primeiro relato de carcinoma de clulas pequenas de bexiga foi feito em 1981 por cramer et al . desde ento , foram registrados aproximadamente 600 casos na literatura mundial. caracterizado por sua alta agressividade e mau prognstico , bem como pelo desenvolvimento de doena metasttica em cerca de 67% dos pacientes . o carcinoma de clulas pequenas da bexiga urinria costuma atingir pessoas da mesma idade , sexo e apresentar os mesmos sintomas e morfologia do carcinoma urotelial . por isso ,  um desafio diagnstico , dependendo apenas da habilidade do patologista em diferenci - lo de carcinoma urotelial , que , alm dos fatores j citados , apresenta o mesmo aspecto radiolgico do carcinoma de clulas pequenas de bexiga . assim , recomendaes so baseadas em estudos retrospectivos , relatos de caso individuais e protocolos para carcinoma de clulas pequenas de pulmo . frequentemente , apresenta - se com histologia mista.estudos moleculares mostram origem comum entre o carcinoma de clulas pequenas de bexiga e o carcinoma urotelial , quando coexistentes . no entanto , seu manejo deve ser diferente , pois , frequentemente , apresenta metstases e tem prognstico ruim . o diagnstico anatomopatolgico desse tipo de tumor  desafiador e exige do patologista o uso de tcnicas de imuno - histoqumica para a confirmao histolgica . como so histologicamente idnticos , no diagnstico de carcinoma de clulas pequenas de bexiga  utilizada a padronizao da organizao mundial da sade para carcinoma de clulas pequenas de pulmo , composta dos seguintes critrios : presena de grupos de clulas pequenas com citoplasma escasso , poucas organelas , cromatina em  sal e pimenta  , e alta taxa de mitoses ( > 10 mitoses/10 campos de alto poder ) . na imuno - histoqumica , esses tumores expressam muita enolase neuroespecfica , cromogranina , sinaptofisina e n - cam ( cd-56 ) . o estadiamento patolgico  feito com base no consenso para carcinoma urotelial de bexiga ( figura 3 ) . figura 3marcador cd-56 , que caracteriza tumores neuroendcrinos 
  terapias isoladas  seja a rtu , a cistectomia parcial ou a radioterapia  apresentam benefcio apenas em casos especiais de doena em estgio inicial . cistectomia radical  considerada o melhor mtodo para remover o carcinoma de clulas pequenas de bexiga completamente , mas acredita - se que ela sozinha s altera a sobrevida em tumores de estgios i e ii.cheng et al . revisaram 64 casos e concluram que , em pacientes submetidos  cirurgia radical comparados queles tratados com terapia combinada ( seja apenas quimioterapia e radioterapia ou ambas em adjuvncia ) , no houve aumento da sobrevida.em contrapartida , quimioterapia adjuvante  cistectomia radical resultou em prognstico favorvel em outros estudos , incluindo meta - anlises . outra alternativa  a quimioterapia neoadjuvante , recomendada pelomdanderson cancer center , o qual relatou sobrevida de 5 anos sem doena em 36% do grupo submetido  cistectomia radical isoladamente e de 78% no grupo submetido  quimioterapia neoadjuvante  cirurgia.de maneira semelhante , a radioterapia neoadjuvante tambm mostrou - se eficiente . por outro lado , um grupo canadense tratou dez pacientes com leses pt3-t4 , n0 com quimioradioterapia , obtendo remisso completa em nove deles . a taxa de sobrevida desse grupo no perodo de 2 anos foi de 70% e , em 5 anos , de 44%.outra srie na mesma linha relatou quatro pacientes tratados com quimioterapia seguida de radioterapia que sobreviveram de 27 a 60 meses . a vantagem desse tratamento  a preservao do rgo . portanto , trata - se de terapia menos invasiva quando comparada  cirurgia radical , a qual est relacionada a importantes taxas de morbidade e mortalidade . pacientes com doena metasttica devem receber quimioterapia sistemtica , sendo os esquemas teraputicos mais comuns os baseados em platina ( cisplatina e etoposida , carboplatina , etoposida e ciclofosfamida ) ( figura 2 ) . por se tratar de doena extremamente agressiva e rara , pouco se conhece da patognese e da biologia molecular . h escassez de dados para abordagem ideal desse tipo de tumor , mostrando a importncia de relatar tais casos e , desta forma , definir melhor os mtodos de diagnstico e tratamento .
small cell carcinoma of the urinary bladder is an extremely aggressive and rare tumor . even though small cell carcinoma most commonly arises from the lungs there 
 are several reports of small cell carcinoma in extrapulmonary sites . due to its low frequency 
 there is no well - established management for this disease . 
 we report the case of a 61 year - old man with small cell carcinoma of the bladder who underwent radical cystectomy following neoadjuvant chemotherapy . 
 we also reviewed the literature for the optimal treatment strategy .