Oliva E, Young R H
Department of Pathology, Harvard Medical School, Massachusetts General Hospital, Boston 02114, USA.
Mod Pathol. 1996 May;9(5):513-20.
Nineteen clear cell adenocarcinomas of the urethra, 18 from women and one from a man, are described. The patients ranged from 35 to 80 years of age (average, 58 yrs). The clinical presentation and gross findings were similar to those of urethral carcinomas, except that 12 tumors, all from women, arose within a urethral diverticulum. On microscopic examination, the neoplasms exhibited the classic triad of tubulocystic, papillary, and diffuse patterns that characterize this tumor. The tumors had the typical cytologic features of clear cell adenocarcinoma, including hobnail cells, flattened cells, and cells with abundant clear cytoplasm. Nuclear pleomorphism was typically at least moderate and was marked in almost half the specimens. Mitotic figures were easily found in almost all the specimens. These cytologic features should aid in distinction of this carcinoma from the benign nephrogenic adenoma, although one of our patients was initially misdiagnosed as having the latter. Immunostaining for prostate-specific antigen and prostatic acid phosphatase was performed on 13 tumors, and all were negative. Follow-up is available for 13 patients. Six of them had no evidence of recurrence up to 10 years postoperatively. Four patients died of disease from 5 months to 42 months postoperatively. Three additional patients have had recurrence but were alive up to 6.5 years after presentation. We conclude that urethral clear cell adenocarcinoma occurs in adults, and in women in the great majority of cases; has a particular association with a urethral diverticulum, which has been present in 56% of the patients; is indistinguishable from clear cell adenocarcinoma of the female genital tract but is not associated with endometriosis; probably does not arise by malignant transformation of nephrogenic adenoma; is usually readily distinguished from the latter because of greater cytologic atypicality and mitotic activity; and does not stain for prostate-specific antigen or prostatic acid phosphatase.
本文描述了19例尿道透明细胞腺癌，其中18例来自女性，1例来自男性。患者年龄在35岁至80岁之间（平均58岁）。临床表现和大体检查结果与尿道癌相似，不过有12例肿瘤（均来自女性）发生于尿道憩室内。显微镜检查显示，肿瘤呈现出该肿瘤典型的三联征，即小管囊性、乳头状和弥漫性模式。肿瘤具有透明细胞腺癌典型的细胞学特征，包括鞋钉样细胞、扁平细胞以及胞质丰富透明的细胞。核异型性通常至少为中度，近半数标本中核异型性明显。几乎所有标本中都很容易找到有丝分裂象。这些细胞学特征有助于将这种癌与良性肾源性腺瘤区分开来，尽管我们的1例患者最初被误诊为后者。对13例肿瘤进行了前列腺特异性抗原和前列腺酸性磷酸酶免疫染色，结果均为阴性。13例患者有随访资料。其中6例术后长达10年无复发迹象。4例患者术后5个月至42个月死于疾病。另有3例患者复发，但就诊后存活长达6.5年。我们得出结论，尿道透明细胞腺癌发生于成年人，绝大多数病例为女性；与尿道憩室有特殊关联，56%的患者存在尿道憩室；与女性生殖道透明细胞腺癌难以区分，但与子宫内膜异位症无关；可能并非由肾源性腺瘤恶变而来；通常因其更大的细胞学异型性和有丝分裂活性而易于与肾源性腺瘤区分；并且不表达前列腺特异性抗原或前列腺酸性磷酸酶。
