Bashour George, Hneino George, Zaher Zain Aldin, Dway Ali, Michael Georges, Alshehabi Zuheir
Cancer Research Center, Tishreen University Hospital.
Faculty of Medicine, Tishreen University.
Ann Med Surg (Lond). 2024 Aug 26;86(10):6236-6240. doi: 10.1097/MS9.0000000000002499. eCollection 2024 Oct.
Urachal carcinomas are uncommon malignant neoplasms comprising only 0.01% of all adult cancers. Most patients were aged from 58 to 64 years at diagnosis with 60 years being the median. It usually metastasizes to the lungs (22%), bones (22%), and liver (16%).
We report a case of a 71-year-old female patient who initially presented with two large liver masses and a small nodule on the anterior side of the bladder. The symptoms were nonspecific with abdominal discomfort. The final diagnosis of urachal adenocarcinoma was finalized with a biopsy of the bladder mass. The patient initially received six doses of FOLFOX6 without improvement and then Gem-Carbo, showing improvement after six doses. Finally, the patient received two doses of FOLFIRI-B with no response and kept deteriorating and died after 19 months of treatment.
About 90% of patients are symptomatic and hematuria is the most typical presenting symptom at diagnosis. The low incidence and the histopathologic similarities to adenocarcinoma from various sources pose a difficulty in recognizing the tumor. Our study presents the only case of a urachal carcinoma first manifesting with abdominal mass resulting from liver metastasis with no prior symptoms of urological origins. Also, our study presents the first attempt of using FLOFIRI-B to treat metastatic UraC.
This case highlights the necessity for clinicopathological correlation to make the correct diagnosis and the challenges in the treatment which urges the need for further research to identify more effective treatment strategies for this rare cancer.
脐尿管癌是一种罕见的恶性肿瘤，仅占所有成人癌症的0.01%。大多数患者在确诊时年龄为58至64岁，中位年龄为60岁。它通常转移至肺（22%）、骨（22%）和肝（16%）。
我们报告一例71岁女性患者，最初表现为肝脏有两个大肿块以及膀胱前壁有一个小结节。症状不具特异性，表现为腹部不适。通过对膀胱肿块进行活检最终确诊为脐尿管腺癌。该患者最初接受了6个周期的FOLFOX6方案治疗但病情无改善，随后接受吉西他滨联合卡铂治疗，6个周期后病情有所改善。最后，患者接受了2个周期的FOLFIRI-B方案治疗但无反应，病情持续恶化，治疗19个月后死亡。
约90%的患者有症状，血尿是确诊时最典型的症状。其发病率低且与各种来源的腺癌在组织病理学上相似，这给肿瘤的识别带来了困难。我们的研究呈现了唯一一例脐尿管癌首先表现为肝转移导致的腹部肿块且无先前泌尿系统起源症状的病例。此外，我们的研究首次尝试使用FOLFIRI-B治疗转移性脐尿管癌。
该病例凸显了临床病理相关性对于做出正确诊断的必要性以及治疗中的挑战，这促使我们需要进一步研究以确定针对这种罕见癌症更有效的治疗策略。
