Guerin M, Miran C, Colomba E, Cabart M, Herrmann T, Pericart S, Maillet D, Neuzillet Y, Deleuze A, Coquan E, Laramas M, Thibault C, Abbar B, Mesnard B, Borchiellini D, Dumont C, Boughalem E, Deville J L, Cancel M, Saldana C, Khalil A, Baciarello G, Flechon A, Walz J, Gravis G
Department of Medical Oncology, Institut Paoli-Calmettes, Marseille, France.
Department of Medical Oncology, Centre Leon-Berard, Lyon, France.
Front Oncol. 2023 Jan 19;13:1110003. doi: 10.3389/fonc.2023.1110003. eCollection 2023.
Urachal cancer (UrC) is a rare, non-urothelial malignancy. Its natural history and management are poorly understood. Although localized to the bladder dome, the most common histological subtype of UrC is adenocarcinoma. UrC develops from an embryonic remnant, and is frequently diagnosed in advanced stage with poor prognosis. The treatment is not standardized, and based only on case reports and small series. This large retrospective multicentric study was conducted by the French Genito-Urinary Tumor Group to gain a better understanding of UrC.
data has been collected retrospectively on 97 patients treated at 22 French Cancer Centers between 1996 and 2020.
The median follow-up was 59 months (range 44-96). The median age at diagnosis was 53 years (range 20-86), 45% were females and 23% had tobacco exposure. For patients with localized disease (Mayo I-II, n=46) and with lymph-node invasion (Mayo III, n=13) median progression-free-survival (mPFS) was 31 months (95% CI: 20-67) and 7 months (95% CI: 6-not reached (NR)), and median overall survival (mOS) was 73 months (95% CI: 57-NR) and 22 months (95% CI: 21-NR) respectively. For 45 patients with Mayo I-III had secondary metastatic progression, and 20 patients were metastatic at diagnosis. Metastatic localization was peritoneal for 54% of patients. Most patients with localized tumor were treated with partial cystectomy, with mPFS of 20 months (95% CI: 14-49), and only 12 patients received adjuvant therapy. Metastatic patients (Mayo IV) had a mOS of 23 months (95% CI: 19-33) and 69% received a platin-fluorouracil combination treatment.
UrC is a rare tumor of the bladder where patients are younger with a higher number of females, and a lower tobacco exposure than in standard urothelial carcinoma. For localized tumor, partial cystectomy is recommended. The mOS and mPFS were low, notably for patients with lymph node invasion. For metastatic patients the prognosis is poor and standard therapy is not well-defined. Further clinical and biological knowledge are needed.
脐尿管癌（UrC）是一种罕见的非尿路上皮恶性肿瘤。对其自然病史和治疗方法了解甚少。尽管脐尿管癌局限于膀胱顶部，但其最常见的组织学亚型是腺癌。脐尿管癌起源于胚胎残余，常于晚期被诊断出，预后较差。其治疗方法不规范，仅基于病例报告和小样本系列研究。这项大型回顾性多中心研究由法国泌尿生殖肿瘤组开展，旨在更好地了解脐尿管癌。
回顾性收集了1996年至2020年间在22家法国癌症中心接受治疗的97例患者的数据。
中位随访时间为59个月（范围44 - 96个月）。诊断时的中位年龄为53岁（范围20 - 86岁），45%为女性，23%有吸烟史。对于局限性疾病患者（梅奥I - II期，n = 46）和有淋巴结侵犯的患者（梅奥III期，n = 13），中位无进展生存期（mPFS）分别为31个月（95%置信区间：20 - 67）和7个月（95%置信区间：6 - 未达到（NR）），中位总生存期（mOS）分别为73个月（95%置信区间：57 - NR）和22个月（95%置信区间：21 - NR）。45例梅奥I - III期患者出现继发性转移进展，20例患者在诊断时已有转移。54%的患者转移部位在腹膜。大多数局限性肿瘤患者接受了部分膀胱切除术，mPFS为20个月（95%置信区间：14 - 49），仅有12例患者接受了辅助治疗。转移性患者（梅奥IV期）的mOS为23个月（95%置信区间：19 - 33），69%的患者接受了铂类 - 氟尿嘧啶联合治疗。
脐尿管癌是一种罕见的膀胱肿瘤，患者较年轻，女性比例较高，吸烟率低于标准尿路上皮癌。对于局限性肿瘤，建议行部分膀胱切除术。mOS和mPFS较低，尤其是有淋巴结侵犯的患者。对于转移性患者，预后较差，标准治疗方法尚不明确。需要进一步的临床和生物学知识。
