Ovarian-type epithelial tumors involving the testis and paratestis are rare, with clear cell carcinomas (CCC) one of the least frequent. We report our experience with 4 müllerian-type (MT) CCCs presenting as testicular/scrotal masses and arising in the paratestis (n=2) and seminal vesicle (n=2; well supported in 1 case and likely in the other). In addition, we document 3 cases of papillary CCC exclusively within the rete testis (RTCCC) and seminiferous tubules and differing from the MT tumors. The patients with MTCCC were 24 to 85 years old (median, 42 y), and 2 had metastases at presentation. The 2 originating in the paratestis were associated with other MT tumors, an endometrioid borderline tumor and a papillary serous borderline tumor. The other 2 MTCCCs likely involved the testis via extension from seminal vesicle primaries through the vasa deferentia. All MTCCCs showed typical features, including tubules, simple papillae with hyalinized cores, and solid nests of polygonal clear cells with occasional hobnail features. Both paratesticular primaries showed sarcomatoid foci with tumor-associated neutrophilic infiltrates. The 3 RTCCCs presented in 54-, 57-, and 60-year-old men as testicular masses; they showed intrarete arborizing papillary growth with nonhyalinized fibrous cores and piled-up, solid foci, lacked hobnail cells, and expressed carbonic anhydrase IX (2/2) and CD10 (2/2) but not CA125, unlike the MTCCCs. On follow-up, 2 patients with MTCCC died of metastatic tumor (4 and 13.5 mo), a third developed ileal and retroperitoneal metastases at 13 months; and the fourth died at 13.5 months of unspecified cause. Follow-up of 2 patients with RTCCCs showed 1 disease free at 8 months and another alive with unknown disease status at 13 years. We conclude that CCCs involving the testis may either be of MT with often aggressive courses or show some features of renal tumors, with confinement to the rete testis and indolent behavior.

中文翻译：

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苗勒管型和睾丸网起源的透明细胞癌表现为阴囊肿块：七例病例的研究。


累及睾丸和副睾丸的卵巢型上皮肿瘤很少见，透明细胞癌（CCC）是最不常见的肿瘤之一。我们报告了 4 例苗勒氏管型 (MT) CCC 的经验，表现为睾丸/阴囊肿块，并出现在睾丸旁 (n = 2) 和精囊 (n = 2；1 例中得到很好的支持，另一例也可能如此)。此外，我们记录了 3 例乳头状 CCC 病例，仅发生在睾丸网 (RTCCC) 和生精小管内，与 MT 肿瘤不同。 MTCCC 患者年龄为 24 至 85 岁（中位年龄 42 岁），其中 2 例就诊时已有转移。起源于睾丸旁的 2 例与其他 MT 肿瘤、子宫内膜样交界性肿瘤和乳头状浆液性交界性肿瘤相关。另外 2 个 MTCCC 可能通过从精囊初级延伸到输精管而累及睾丸。所有 MTCCC 均显示出典型特征，包括肾小管、具有透明核心的简单乳头以及具有偶尔平头钉特征的多边形透明细胞的实心巢。两个睾丸旁原发灶均显示肉瘤样病灶和肿瘤相关的中性粒细胞浸润。 3 个 RTCCC 在 54 岁、57 岁和 60 岁男性中表现为睾丸肿块；与 MTCCC 不同，它们表现出视网膜内树状乳头状生长，具有非玻璃样变的纤维核心和堆积的实心病灶，缺乏平头钉细胞，并表达碳酸酐酶 IX (2/2) 和 CD10 (2/2)，但不表达 CA125。随访时，2 名 MTCCC 患者死于转移性肿瘤（4 个月和 13.5 个月），第 3 名患者在 13 个月时出现回肠和腹膜后转移；第四个在 13.5 个月时死亡，原因不明。对 2 名 RTCCC 患者的随访显示，其中一名患者在 8 个月时已无疾病，另一名患者在 13 岁时仍然存活，但疾病状况未知。 我们的结论是，涉及睾丸的 CCC 可能是 MT，通常具有侵袭性过程，或者表现出肾肿瘤的某些特征，仅限于睾丸网和惰性行为。