Holmäng Sten, Thomsen Jean, Johansson Sonny L
Department of Urology, Sahlgrenska University Hospital, Göteborg, Sweden.
J Urol. 2006 Feb;175(2):463-6; discussion 466-7. doi: 10.1016/S0022-5347(05)00153-9.
MPC located in the upper urinary tract is rare with only 2 cases reported to date. We report clinical and histopathological data on 26 patients to increase the knowledge of this rare entity.
A clinical and histopathological review was performed in 943 patients with a neoplasm in the renal pelvis or ureter, diagnosed between 1971 and 1998. We identified 26 patients with MPC. No patients were alive at the end of the study.
Of the patients 11 had greater than 50% MPC and 15 had focal MPC (at least 10%). The incidence was 2.8%. Median patient age at diagnosis was 69 years (range 54 to 88) and the male-to-female ratio was 17:9. All except 4 patients had stage T3 disease or higher. Carcinoma in situ was identified in 64% of cases and vascular invasion was present in 81%. A total of 20 patients (77%) died of disease and only 7 survived longer than 5 years.
The prognosis is poor since most patients with MPC of the renal pelvis and ureter initially present with advanced disease. Stage for stage the prognosis is not different from that in nonMPC urothelial cell carcinoma. Surgery is curative in less advanced cases. However, radiotherapy and systemic chemotherapy appear to be ineffective.
位于上尿路的黏液性梭形细胞癌(MPC)很罕见,迄今为止仅有2例报道。我们报告26例患者的临床和组织病理学数据,以增加对这种罕见疾病的认识。
对1971年至1998年间诊断为肾盂或输尿管肿瘤的943例患者进行了临床和组织病理学回顾。我们确定了26例MPC患者。研究结束时无患者存活。
患者中,11例MPC占比大于50%,15例为局灶性MPC(至少10%)。发病率为2.8%。诊断时患者的中位年龄为69岁(范围54至88岁),男女比例为17:9。除4例患者外,所有患者均为T3期或更高分期疾病。64%的病例发现原位癌,81%存在血管侵犯。共有20例患者(77%)死于疾病,仅7例存活超过5年。
由于大多数肾盂和输尿管MPC患者初诊时已为晚期疾病,预后较差。逐期来看,其预后与非MPC尿路上皮细胞癌无异。在疾病不太晚期的情况下,手术可治愈。然而,放疗和全身化疗似乎无效。