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主要的封印环细胞腺癌是由于尿道膀的外而产生的 (epispadias-exstrophy复合物)

Gwendolyn Fernandes¹, Gloria Khumanthem, Nupur Harinkhede

¹Department of Pathology, Seth G.S. Medical College and K.E.M Hospital, Parel, Mumbai, Maharashtra, India.

Indian journal of pathology & microbiology

|February 23, 2024

概括

初级封印环细胞腺癌 (PSRCA) 是一种罕见的癌症,在患有膀外缩的患者中发现. 这一案例凸显了它的攻击性和早期手术干预的必要性以及膀外缩患者的终身监测.

科学领域:

在瘤学瘤学.
泌尿器科泌尿器科泌尿器科泌尿器科
遗传异常是一种先天性异常.

背景情况:

膀外缩是一种罕见的先天性异常,其中尿膀在体外发育.
它可以是膀表皮病-外-水综合体的一部分,涉及多个系统.
膀外缩中的初级封印环细胞腺癌 (PSRCA) 异常罕见.

研究的目的:

在未经治疗的膀外缩症患者中报告一种罕见的原发性封印环细胞腺癌病例.
在这种情况下,强调PSRCA的攻击性行为和转移潜力.
建议早期手术管理和膀外收缩的长期随访.

主要方法:

一个32岁的男性患有先天性膀外缩的病例介绍.
诊断成像包括超声波和对比增强计算机断层扫描 (CECT).
活检,根性囊切除与阴道管, inguinal 淋巴结剖析,和免疫组织化学 (IHC).

主要成果:

在腹壁上发现了一大块功能性质,取代了泌尿膀.
活检证实了封印环细胞腺癌; 组织病理学揭示了PSRCA与 inguinal 淋巴结转移.
IHC显示CDX2和CK20阳性,CK7阴性,表明胃肠道来源.

结论:

膀外缩症中的PSRCA具有侵略性,并可能出现转移.
差异性诊断必须排除从其他部位的转移性封印环细胞腺癌.
早期的手术治疗和终身监测对于管理膀外缩和相关恶性瘤至关重要.

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