Di Carlo Daniela, Ferrari Andrea, Perruccio Katia, D'Angelo Paolo, Fagnani Anna Maria, Cecchetto Giovanni, Bisogno Gianni
Department of Women's and Children's Health, Hematology/Oncology Division, Padova University Hospital, Padova, Italy.
Pediatr Blood Cancer. 2015 Jun;62(6):1000-3. doi: 10.1002/pbc.25380. Epub 2014 Dec 24.
Urothelial neoplasms of the bladder (UNB) are rare in patients under 20 years of age, and even rarer in the first decade of life. The present series was investigated to provide recommendations on patient management in terms of therapeutic strategy and follow-up.
This is a retrospective analysis on 12 patients with UNB under 18 years of age. Data were extracted from the national database of the TREP (Tumori Rari in Età Pediatrica) Project.
Ten of the 12 patients presented with a single episode of hematuria, while the discovery of the lesion was incidental in two. Eleven of the 12 lesions were G1 and one was G2/G3; none of the lesions invaded the lamina propria. All lesions were removed completely by transurethral resection. No further treatment was administered in nine children but three received a single dose of intravesical chemotherapy (epirubicin in 2, mitomycin in 1). Only one patient experienced a recurrence and all patients are alive in complete remission with a median follow-up of 30 months (range 4-112). Follow-up investigations varied at the different centers and included abdominal ultrasound in nine patients, cystoscopy in seven, and additional radiological investigations in a few cases.
UNB in children seems to be a low-grade, scarcely aggressive disease with an excellent prognosis. The role of intravesical chemotherapy is debatable. Follow-up can be based on ultrasound. The adoption of shared recommendations should enable unnecessary treatment and invasive investigations to be avoided.
膀胱尿路上皮肿瘤(UNB)在20岁以下患者中罕见,在生命的第一个十年中更为罕见。本系列研究旨在就治疗策略和随访方面的患者管理提供建议。
这是一项对12例18岁以下UNB患者的回顾性分析。数据从TREP(儿童罕见肿瘤)项目的国家数据库中提取。
12例患者中有10例表现为单次血尿发作,2例病变为偶然发现。12个病变中有11个为G1级,1个为G2/G3级;所有病变均未侵犯固有层。所有病变均通过经尿道切除术完全切除。9名儿童未接受进一步治疗,但3名接受了单剂量膀胱内化疗(2例使用表柔比星,1例使用丝裂霉素)。仅1例患者复发,所有患者均存活且完全缓解,中位随访时间为30个月(范围4 - 112个月)。不同中心的随访检查各不相同,9例患者进行了腹部超声检查,7例进行了膀胱镜检查,少数病例进行了其他影像学检查。
儿童UNB似乎是一种低级别、侵袭性较弱且预后良好的疾病。膀胱内化疗的作用存在争议。随访可以基于超声检查。采用共同的建议应能避免不必要的治疗和侵入性检查。