*Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, Los Angeles, CA †Department of Pathology, Indiana University School of Medicine, Indianapolis, IN ∥Department of Pathology, The University of Texas M. D. Anderson Cancer Center, Houston, TX ¶James Homer Wright Pathology Laboratories, Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA ‡Department of Pathology, The University of Hong Kong, Pokfulam §Department of Clinical Pathology, Tuen Mun Hospital, Tuen Mun, Hong Kong.
Am J Surg Pathol. 2014 Aug;38(8):1033-45. doi: 10.1097/PAS.0000000000000250.
Published experience remains limited for glandular neoplasms of the urachus, especially mucinous cystic tumors. We reviewed 55 glandular urachal neoplasms to evaluate their clinical features and histopathologic spectrum and to devise a classification system for the mucinous cystic forms. Within the 55 cases studied, we observed 2 groups with differing clinical, gross, and histopathologic features. The first group, invasive, noncystic adenocarcinomas (n=24), had clinicopathologic features in accord with the known spectrum of urachal adenocarcinoma (mean age 50 y, female:male ratio 1.7, with recurrence or death from disease in 9/16 cases over a 45 mo mean follow-up). The second group, mucinous cystic tumors (n=31), morphologically resembled mucinous cystic tumors of the ovary and appeared classifiable by the same approach (mean age 47 y, female:male ratio 1.4) and included mucinous cystadenoma (n=4), mucinous cystic tumor of low malignant potential (n=22, including 2 cases with intraepithelial carcinoma), and mucinous cystadenocarcinoma with microscopic (n=4) or frank invasion (n=1). Follow-up information was available for 13 patients with mucinous cystic tumors (mean 41 mo); we observed no local recurrence or distant metastasis. This experience suggests that there is a distinct group of glandular, cystic tumors of the urachus that is classifiable in a manner similar to ovarian neoplasms and that has a favorable prognosis after complete excision. As with cystic neoplasms of other organs, rigorous sampling is recommended to identify potentially small foci of carcinoma that could be missed by inadequate sampling. Accordingly, classification based on methods other than complete surgical excision may be hazardous.
脐尿管的腺性肿瘤,尤其是黏液性囊性肿瘤,其发表的经验仍然有限。我们回顾了 55 例脐尿管腺性肿瘤,以评估其临床特征、组织病理学表现,并为黏液性囊性肿瘤制定分类系统。在研究的 55 例病例中,我们观察到两组具有不同的临床、大体和组织病理学特征。第一组为侵袭性、非囊性腺癌(n=24),其临床病理特征与已知的脐尿管腺癌谱一致(平均年龄 50 岁,男女比例 1.7,在 45 个月的平均随访中,16 例中有 9 例复发或死于疾病)。第二组为黏液性囊性肿瘤(n=31),形态上类似于卵巢的黏液性囊性肿瘤,可通过相同的方法进行分类(平均年龄 47 岁,男女比例 1.4),包括黏液性囊腺瘤(n=4)、低度恶性潜能的黏液性囊腺瘤(n=22,包括 2 例上皮内癌)和微浸润(n=4)或明显浸润(n=1)的黏液性囊腺癌。13 例黏液性囊性肿瘤患者的随访信息可用(平均 41 个月);我们未观察到局部复发或远处转移。这一经验表明,脐尿管有一组明显的腺性、囊性肿瘤,可以类似于卵巢肿瘤的方式进行分类,并且在完全切除后具有良好的预后。与其他器官的囊性肿瘤一样,建议进行严格的取样,以识别可能因取样不足而遗漏的微小癌灶。因此,基于不完全手术切除以外的方法进行分类可能是危险的。