Division of Urology, Ivrea Hospital (ASL TO4), Ivrea (Turin).
Arch Ital Urol Androl. 2020 Oct 2;92(3). doi: 10.4081/aiua.2020.3.211.
Neuroendocrine tumours (NET) are extremely rare and aggressive. Although they commonly affect intestine, many organs may be involved such as pancreas, lung or urinary tract. Bladder is rarely involved. Actually, two main forms of bladder NET have been described: small-cell and large-cell. The first one is considered highly agressive since it shows poor oncologic outcomes being mainly diagnosed at advanced stage: the second one is extremely rare and equally aggressive.
A 78-years-old Caucasian male presented to our facility for lower urinary tract symptoms and gross hematuria recently occurred. He was a strong smoker since many years. No familiarity for urothelial cancer was referred nor previous episodes of hematuria until that time. Citology was negative; outpatient ultrasound of the bladder revealed a 3 cm bladder thickening highly suspicious for bladder cancer; patient underwent TC scan that confirmed the bladder lesion. A transurethral resection of the bladder (TURB) was performed. After 3 months total body TC showed multiple visceral metastases also involving brain and lymph nodes. Best supportive care was offered but the patient died 6 months later.
Pathology revealed a mixed bladder tumor: 30% of the specimen resulted as an high-grade urothelial cancer (G3) and 70% as small-cell neuroendocrine variant.Microscopic muscle involvement was excluded.
Neuroendocrine tumors are uncommon entities which origin from cells of neuro-endocrine system and may potentially involve all human tissues. Neuroendocrine smallcell carcinoma of the bladder is a non-urothelial histotype: it is highly aggressive and diagnosed mainly at advanced stages. Whenever considering the high risk of metastatic spread and the poor prognosis, a multimodal approach is highly suggested. TURB alone is uneffective in disease control due to its aggressive nature. Unless metastatic, radical cystectomy and adjuvant chemotherapy represent the gold standard.
神经内分泌肿瘤(NET)非常罕见且具有侵袭性。尽管它们通常影响肠道,但许多器官也可能受到影响,如胰腺、肺或泌尿道。膀胱很少受到影响。实际上,已经描述了两种主要形式的膀胱 NET:小细胞和大细胞。第一种被认为具有高度侵袭性,因为它主要在晚期诊断,预后较差;第二种则极为罕见且同样具有侵袭性。
一名 78 岁白人男性因下尿路症状和近期出现的肉眼血尿到我院就诊。他多年来一直是重度吸烟者。没有尿路上皮癌的家族史,也没有之前出现血尿的病史。细胞学检查为阴性;门诊膀胱超声显示膀胱 3 厘米增厚,高度怀疑膀胱癌;患者接受了 TC 扫描,证实了膀胱病变。随后进行了经尿道膀胱肿瘤切除术(TURB)。3 个月后,全身 TC 显示多个内脏转移,还累及脑部和淋巴结。提供了最佳支持性治疗,但患者在 6 个月后死亡。
病理显示混合性膀胱肿瘤:30%的标本为高级别尿路上皮癌(G3),70%为小细胞神经内分泌变体。显微镜下排除了肌肉侵犯。
神经内分泌肿瘤是罕见的实体瘤,起源于神经内分泌系统的细胞,可能潜在地涉及所有人体组织。膀胱神经内分泌小细胞癌是一种非尿路上皮组织学类型:它具有高度侵袭性,主要在晚期诊断。鉴于转移扩散的高风险和不良预后,强烈建议采用多模式方法。由于其侵袭性,单独 TURB 对疾病控制无效。除非发生转移,根治性膀胱切除术和辅助化疗是金标准。