title: [Neuroendocrine small-cell bladder cancer: our experience].
abstract: BACKGROUND
  Neuroendocrine bladder cancer is extremely rare, with an estimated incidence of 0.35-0.70% of all bladder tumors. The small-cell carcinoma represents the most frequent histologic variant described. Small-cell carcinoma is an epithelial tumor associated with a more aggressive behavior and poorer prognosis than transitional cell bladder carcinoma. The overall survival rate at 5 years does not exceed 8%. At the time of presentation 59% of patients have clinical stage >T2 and 56% show metastatic disease. In 50% of the patients, fatal progression occurs within 6 months. Local recurrence after radical surgery occurred in 50-70% of cases.
  
  
  METHODS
  We report three cases of pure neuroendocrine small-cell bladder cancer. Hematuria was the most common presenting symptom. Local advanced disease was present in all the cases with stage >T2, metastatic disease in 1 case, lymph node involvement and ureteral bilateral obstruction in 2. Two patients were treated by radical cystectomy, bilateral pelvic limph node resections and urinary derivation. Platinum-based adjuvant chemotherapy was proposed but only two patients received the treatment. One patient with liver metastasis was managed only by extensive TUR and support regimen.
  
  
  RESULTS
  In 2 patients residual or relapsed cancer reappered within 2 months after surgery. All of the three patients died of metastatic disease at 5, 7, and 13 months. Median overall survival was 7 months. The most common site of relapse and spread of disease was the peritoneum and intestinal tract, and the reason of death was uncontrolled acute hemorrhage from gastro-intestinal district.
  
  
  CONCLUSIONS
  In the absence of a prospective study, and because of the rarity of the disease, the best treatment for small-cell bladder cancer remains uncertain. Neoadjuvant chemotherapy with platinum regimen plus aggressive surgical approach will be the treatment of choice. The association of chemotherapy and radiotherapy should also be considered.
authors.affiliation: UO Urologia, Azienda Ospedaliera Ospedale Treviglio Caravaggio (Bergamo), Italy. micheleruoppolo@tiscalinet.it
authors.forename: M
authors.initials: M
authors.lastname: Ruoppolo
authors.forename: E
authors.initials: E
authors.lastname: Pezzica
authors.forename: R
authors.initials: R
authors.lastname: Milesi
authors.forename: D
authors.initials: D
authors.lastname: Corti
authors.forename: P
authors.initials: P
authors.lastname: Mercurio
authors.forename: G
authors.initials: G
authors.lastname: Fragapane
mesh_terms: D000230:Adenocarcinoma; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D016190:Carboplatin / Q000008:administration & dosage; D018278:Carcinoma, Neuroendocrine / Q000150:complications / Q000188:drug therapy / Q000401:mortality / Q000473:pathology* / Q000556:secondary / Q000601:surgery; D018288:Carcinoma, Small Cell / Q000150:complications / Q000188:drug therapy / Q000401:mortality / Q000473:pathology* / Q000556:secondary / Q000601:surgery; D003131:Combined Modality Therapy; D015653:Cystectomy; D003841:Deoxycytidine / Q000008:administration & dosage / Q000031:analogs & derivatives; D018450:Disease Progression; D017809:Fatal Outcome; D006471:Gastrointestinal Hemorrhage / Q000209:etiology; D006417:Hematuria / Q000209:etiology; D006801:Humans; D007414:Intestinal Neoplasms / Q000150:complications / Q000556:secondary; D015451:Leukemia, Lymphocytic, Chronic, B-Cell; D008113:Liver Neoplasms / Q000556:secondary; D008197:Lymph Node Excision; D008297:Male; D008875:Middle Aged; D016609:Neoplasms, Second Primary; D010534:Peritoneal Neoplasms / Q000556:secondary; D011471:Prostatic Neoplasms; D013274:Stomach Neoplasms; D015996:Survival Rate; D001749:Urinary Bladder Neoplasms / Q000150:complications / Q000188:drug therapy / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D000093542:Gemcitabine
chemical_list: D003841:Deoxycytidine; D016190:Carboplatin; D000093542:Gemcitabine