[神经内分泌小细胞膀胱癌：我们的经验]

Ruoppolo M, Pezzica E, Milesi R, Corti D, Mercurio P, Fragapane G

UO Urologia, Azienda Ospedaliera Ospedale Treviglio Caravaggio (Bergamo), Italy.

Urologia. 2010 Oct-Dec;77 Suppl 17:64-71.

INTRODUCTION

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.

PATIENTS AND 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.

引言

神经内分泌性膀胱癌极为罕见，估计占所有膀胱肿瘤的0.35 - 0.70%。小细胞癌是最常见的组织学变异类型。小细胞癌是一种上皮性肿瘤，与移行细胞膀胱癌相比，其行为更具侵袭性，预后更差。5年总生存率不超过8%。就诊时，59%的患者临床分期>T2，56%有转移性疾病。50%的患者在6个月内出现致命进展。根治性手术后局部复发率为50 - 70%。

患者与方法

我们报告3例纯神经内分泌性小细胞膀胱癌病例。血尿是最常见的首发症状。所有病例均为局部晚期疾病，分期>T2，1例有转移性疾病，2例有淋巴结受累及双侧输尿管梗阻。2例患者接受了根治性膀胱切除术、双侧盆腔淋巴结切除术及尿流改道。建议采用铂类辅助化疗，但仅2例患者接受了该治疗。1例有肝转移的患者仅接受了广泛经尿道膀胱肿瘤切除术及支持治疗。