Departments of Pathology.
Urology, and Oncology, Johns Hopkins Medical Institutions, Baltimore, MD.
Am J Surg Pathol. 2018 Nov;42(11):1513-1521. doi: 10.1097/PAS.0000000000001108.
Skene's (periurethral) gland adenocarcinoma is very rare, with only 7 cases reported in the literature. This is the first series of cases on this entity. We describe the histologic, immunohistochemical, and clinical findings of 4 patients with Skene's gland adenocarcinoma retrieved from the Johns Hopkins Urologic Pathology Consult Service from 1984 to 2017. The average age at diagnosis of the 4 women was 74.5 years (range, 61 to 87 y). Tumors were treated by limited resections with negative margins. Tumor size ranged from 1.0 to 2.0 cm (mean, 1.5 cm). Average follow-up time was 40.7 months (range, 4 to 132 mo). Three of our cases were morphologically consistent with prostatic acinar adenocarcinoma with variable cribriform, fused, and poorly formed glands, analogous to Gleason score 4+4=8. Of these, one had mixed ductal features with neoplastic cells showing papillary carcinoma with columnar cytology. These 3 lesions were positive for PSA, P501S, NKX3.1, and AMACR. Focal goblet cells positive for CK20 and negative for prostatic markers were seen in one of these cases, suggesting intestinal differentiation (although negative for CDX2 and SATB2). A fourth case had glandular and papillary formations with pseudostratified columnar epithelium and mucin secretion, showing positivity for CK7, ER, and P16, and negativity for prostatic markers, suggesting serous differentiation (although negative for PAX8 and WT1). PIN4 cocktail confirmed the origin in preexisting paraurethral glands in 3 of the cases. All patients were alive and free of recurrence or metastatic disease at the time of last follow-up. Because of the rarity of Skene's gland adenocarcinomas, there is no consensus regarding their treatment. Our findings demonstrate that Skene's gland adenocarcinomas recapitulate morphologies and immunohistochemical markers seen in prostatic adenocarcinoma. However, it is unknown whether applying the same grading criteria for prostatic adenocarcinomas to Skene's gland adenocarcinoma is valid given the small number of cases with variable treatment and limited follow-up.
斯基恩氏（尿道旁）腺腺癌非常罕见，文献中仅报道了 7 例。这是首例关于该实体的病例系列。我们描述了从 1984 年至 2017 年约翰霍普金斯泌尿科病理咨询服务中检索到的 4 例斯基恩氏腺腺癌患者的组织学、免疫组织化学和临床发现。4 名女性的平均诊断年龄为 74.5 岁（范围，61 至 87 岁）。肿瘤通过边缘阴性的局限性切除术进行治疗。肿瘤大小从 1.0 至 2.0 厘米（平均，1.5 厘米）。平均随访时间为 40.7 个月（范围，4 至 132 个月）。我们的 3 例病例在形态上与前列腺腺泡腺癌一致，具有不同程度的筛状、融合和形成不良的腺体，类似于 Gleason 评分 4+4=8。其中 1 例具有混合管状特征，肿瘤细胞表现出柱状细胞学的乳头状癌。这些病变均对 PSA、P501S、NKX3.1 和 AMACR 呈阳性。在其中 1 例中，可见局灶性杯状细胞 CK20 阳性，前列腺标志物阴性，提示肠分化（尽管 CDX2 和 SATB2 阴性）。第 4 例病例具有腺状和乳头状形成，假复层柱状上皮和粘液分泌，对 CK7、ER 和 P16 呈阳性，对前列腺标志物呈阴性，提示浆液分化（尽管 PAX8 和 WT1 阴性）。PIN4 鸡尾酒在 3 例病例中证实了起源于原尿道旁腺。所有患者在最后一次随访时均存活且无复发或转移疾病。由于斯基恩氏腺腺癌非常罕见，因此对于其治疗方法尚无共识。我们的发现表明，斯基恩氏腺腺癌再现了前列腺腺癌的形态和免疫组织化学标志物。然而，鉴于病例数量少、治疗方法多样且随访时间有限，对于将前列腺腺癌的相同分级标准应用于斯基恩氏腺腺癌是否有效尚不清楚。
