Tian Yan, Ren Chao, Shi Lin, Guo Zhanlin
Hyperbaric Oxygen Therapy Center, The Affiliated Hospital of Inner Mongolia Medical University, Hohhot, China.
Department of Urology Surgery, Affiliated Hospital of Inner Mongolia Medical University, Hohhot, China.
Thorac Cancer. 2024 Dec;15(35):2509-2513. doi: 10.1111/1759-7714.15481. Epub 2024 Oct 27.
Arising from the urachal epithelial lining, the urachal carcinoma is a rare tumor, which accounts for 0.35%-0.7% of all bladder cancers. Urachal carcinoma has a higher predilection in men with median age around 50-60 years old. The most common clinical symptom is intermittent painless gross hematuria, and less-reported presentations include suprapubic mass, dysuria, lower abdominal pain, and frequent urination. The pathological study reveals that most cases (90%) are categorized as an intestinal adenocarcinoma subtype, while other morphological variants, including mucinous, enteric, signet ring cell subtype, not otherwise specified (NOS), squamous cell carcinoma, urothelial carcinoma, sarcoma, small cell carcinoma, and undifferentiated carcinoma, totally account for about 10%. The urachal carcinoma occurs mostly in the lower segment of urachal tube and bladder dome or anterior wall. However, due to the classically silent nature of the early lesions and high malignancy, urachal carcinoma patients are commonly diagnosed in advanced stage. Treatment modalities for local recurrence or metastatic urachal cancer include surgery and chemotherapy (cisplatin and 5-FU based-chemotherapy). Meanwhile, the EGFR-, PD-L1-, and MEK-targeted therapies in the metastatic urachal carcinoma cases showed satisfactory response. We presented a rare case of Sheldon stage IVB urachal adenocarcinoma with pulmonary metastasis, and the patient had no progression of disease 6 months following surgical treament without chemoradiotherapy.
脐尿管癌起源于脐尿管上皮，是一种罕见肿瘤，占所有膀胱癌的0.35%-0.7%。脐尿管癌在男性中更常见，中位年龄约为50-60岁。最常见的临床症状是间歇性无痛肉眼血尿，较少报告的表现包括耻骨上肿块、排尿困难、下腹痛和尿频。病理研究显示，大多数病例（90%）被归类为肠腺癌亚型，而其他形态学变异，包括黏液性、肠型、印戒细胞亚型（未另行说明，NOS）、鳞状细胞癌、尿路上皮癌、肉瘤、小细胞癌和未分化癌，总共约占10%。脐尿管癌大多发生在脐尿管下段及膀胱顶部或前壁。然而，由于早期病变通常无症状且恶性程度高，脐尿管癌患者通常在晚期才被诊断出来。局部复发或转移性脐尿管癌的治疗方式包括手术和化疗（以顺铂和5-氟尿嘧啶为基础的化疗）。同时，针对转移性脐尿管癌病例的表皮生长因子受体（EGFR）、程序性死亡受体1配体（PD-L1）和丝裂原活化蛋白激酶（MEK）靶向治疗显示出令人满意的反应。我们报告了一例罕见的谢尔顿IVB期脐尿管腺癌伴肺转移病例，该患者在未接受放化疗的手术治疗后6个月病情无进展。
