Ni Y P, Zhang Z H, Chen X Y, Liu J S, Yang X Q
Department of Pathology, Ruijin Hospital, Shanghai Jiaotong University Medical College, Shanghai 200025, China.
Zhonghua Bing Li Xue Za Zhi. 2023 Apr 8;52(4):347-352. doi: 10.3760/cma.j.cn112151-20221124-00990.
To investigate the clinicopathological features and possible mechanisms of burned-out testicular germ cell tumors. The clinical and imaging data, histology and immunophenotypic characteristics of three cases of burned-out testicular germ cell tumors diagnosed at the Ruijin Hospital, Medical College of the Shanghai Jiaotong University, from 2016 to 2020 were retrospectively analyzed. The relevant literature was reviewed. The mean age of the three patients was 32 years. Case 1 had an elevated preoperative alpha-fetoprotein level (810.18 μg/L) and underwent "radical pancreaticoduodenectomy and retroperitoneal lesion resection" for a retroperitoneal mass. Postoperative pathology showed embryonal carcinoma, which needed to exclude gonadal metastasis. Color Doppler ultrasound showed a solid mass of the right testis, with hypoechoic lesion and scattered calcification in some areas. Case 2 was a "right supraclavicular lymph node biopsy specimen." Chest X-ray showed multiple metastases in both lungs. The biopsy showed metastatic embryonic carcinoma and bilateral testicular color Doppler ultrasound revealed abnormal calcifications in the right testicle. Case 3 showed a cystic mass of the right testis with calcification and solid areas. All 3 patients underwent radical right orchiectomy. Grossly, borders of the testicular scar areas were well defined. Cross sectioning of the tumors showed a gray-brown cut surface and single focus or multiple foci of the tumor. The tumor maximum diameter was 0.6-1.5 cm. Microscopically, lymphocytes, plasma cells infiltration, tubular hyalinization, clustered vascular hyperplasia and hemosiderin laden macrophages were found in the scar. Atrophic and sclerotic seminiferous tubules, proliferation of clustered Leydig cells and small or coarse granular calcifications in seminiferous tubules were present around the scar. Seminoma and germ cell neoplasia in situ were seen in case 1, germ cell neoplasia in situ was seen in case 2 and germ cells with atypical hyperplasia were seen in case 3. Immunohistochemistry showed that embryonic carcinoma expressed SALL4, CKpan(AE1/AE3) and CD30, seminoma and germ cell tumor in situ expressed OCT3/4, SALL4 and CD117, and spermatogenic cells with atypical hyperplasia expressed CD99 and SALL4. The Ki-67 positive index was about 20%, while OCT3/4 and CD117 were both negative. Burned-out testicular germ cell tumors are rare. The possibility of gonad testicular metastasis should be considered first for extragonadal germ cell tumor. If fibrous scar is found in testis, it must be determined whether it is a burned-out testicular germ cell tumor. The burned-out mechanisms may be related to the microenvironment of tumor immune-mediated and local ischemic injury.
探讨消退型睾丸生殖细胞肿瘤的临床病理特征及可能机制。回顾性分析2016年至2020年在上海交通大学医学院附属瑞金医院确诊的3例消退型睾丸生殖细胞肿瘤的临床及影像资料、组织学和免疫表型特征。并复习相关文献。3例患者的平均年龄为32岁。病例1术前甲胎蛋白水平升高（810.18μg/L），因腹膜后肿块行“根治性胰十二指肠切除术及腹膜后病变切除术”。术后病理显示为胚胎癌，需排除性腺转移。彩色多普勒超声显示右侧睾丸实性肿块，部分区域为低回声病变并伴有散在钙化。病例2为“右锁骨上淋巴结活检标本”。胸部X线显示双肺多发转移灶。活检显示为转移性胚胎癌，双侧睾丸彩色多普勒超声显示右侧睾丸有异常钙化。病例3显示右侧睾丸囊性肿块伴钙化及实性区域。所有3例患者均行右侧根治性睾丸切除术。大体上，睾丸瘢痕区域边界清晰。肿瘤切面呈灰棕色，可见单个或多个肿瘤病灶。肿瘤最大直径为0.6 - 1.5cm。显微镜下，瘢痕区可见淋巴细胞、浆细胞浸润，小管玻璃样变，成簇的血管增生及含铁血黄素巨噬细胞。瘢痕周围可见萎缩硬化的生精小管、成簇的Leydig细胞增生以及生精小管内小的或粗大的颗粒状钙化。病例1可见精原细胞瘤及原位生殖细胞肿瘤，病例2可见原位生殖细胞肿瘤，病例3可见具有非典型增生的生殖细胞。免疫组化显示胚胎癌表达SALL4、CKpan(AE1/AE3)和CD30，精原细胞瘤及原位生殖细胞肿瘤表达OCT3/4、SALL4和CD117，具有非典型增生的生精细胞表达CD99和SALL4。Ki-67阳性指数约为20%，而OCT3/4和CD117均为阴性。消退型睾丸生殖细胞肿瘤罕见。对于性腺外生殖细胞肿瘤，应首先考虑性腺睾丸转移的可能性。如果在睾丸发现纤维瘢痕，必须确定其是否为消退型睾丸生殖细胞肿瘤。消退机制可能与肿瘤免疫介导的微环境及局部缺血损伤有关。
