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Nonchoriocarcinomatous Trophoblastic Tumors of the Testis: The Widening Spectrum of Trophoblastic Neoplasia.

睾丸非绒毛膜癌性滋养细胞肿瘤:拓宽了滋养细胞肿瘤谱系

Idrees MT,Kao CS,Epstein JI,Ulbright TM

Abstract

Tumors of trophoblastic derivation other than choriocarcinoma are very rare in the testis but have been reported on occasion in association with other germ cell tumors. Their morphologic spectrum is analogous to the trophoblastic tumors of the female genital tract including epithelioid trophoblastic tumor (ETT) and placental site trophoblastic tumor (PSTT). Herein we report our experience with 8 cases of trophoblastic tumors of testicular origin that lacked the features of choriocarcinoma; these included 4 ETTs, 1 PSTT, 1 unclassified trophoblastic tumor (UTT), 1 partially regressed choriocarcinoma with a monophasic morphology, and 1 hybrid tumor showing a mixture of adenocarcinoma and a UTT. All tumors occurred in young men 19 to 43 years old. Five arose de novo within the testis (2 ETTs, 1 UTT, 1 regressing choriocarcinoma, and the hybrid tumor) as a component of mixed germ cell tumors, and 3 (2 ETTs and 1 PSTT) were found in metastatic sites after chemotherapy. The trophoblastic component was minor (5% to 10%) in 6 tumors but was 95% of 1 metastatic tumor (ETT) and 50% of the hybrid tumor. Other germ cell tumor elements were identified in all cases, most commonly teratoma. The ETTs consisted of nodules and nests of squamoid trophoblast cells showing abundant eosinophilic cytoplasm, frequent apoptotic cells, extracellular fibrinoid material, and positivity for p63 and negativity for human placental lactogen (HPL). The PSTT showed sheets of discohesive, pleomorphic, mononucleated trophoblast cells that invaded blood vessels with fibrinoid change and were p63 negative and HPL positive. The UTT showed a spectrum of small and large trophoblast cells, some multinucleated but lacking distinct syncytiotrophoblasts, and was patchily positive for both p63 and HPL. The hybrid tumor had ETT-like and adenocarcinomatous areas that coexpressed inhibin and GATA3 but were negative for p63 and HPL, leading to classification of the trophoblastic component as UTT. Seven of the patients were alive and well on follow-up (8 to 96 mo; median, 39 mo), whereas the patient with the hybrid tumor died of liver metastases at 2 years. Our study verifies that trophoblastic neoplasms often having the features of nonchoriocarcinomatous gestational trophoblastic tumors may arise from the testis, occur either in the untreated primary tumor or in metastases after chemotherapy, and should be distinguished from choriocarcinoma given what appears to be a less aggressive clinical course.

摘要

除了绒毛膜癌,睾丸中滋养细胞来源的肿瘤很少见,但有报道它有时会伴随其它生殖细胞肿瘤。其形态学谱系类似于女性生殖道滋养细胞肿瘤,其中包括上皮样滋养细胞肿瘤(ETT)、胎盘部位滋养细胞肿瘤(PSTT)。我们报道了8例睾丸缺乏绒毛膜癌特征的滋养细胞肿瘤,包括4例ETTs、1例PSTT、1例未分类滋养细胞肿瘤、1例形态单一有部分退变的绒毛膜癌以及1例混合有腺癌和UTT的杂交瘤。所有肿瘤发生于年轻人,年龄19—43岁。5例发现于睾丸(2例ETTs、1例UTT、1例退变绒毛膜癌和1例杂交瘤),是混合性生殖细胞肿瘤的一部分;3例(2例ETTs和1例PSTT)发现于化疗后的转移部位。滋养细胞成分,6例显示少量(5%—10%),1例转移性肿瘤(ETT)中为95%,1例杂交瘤中为50%。所有病例中的其它生殖细胞肿瘤明确,畸胎瘤最常见。ETTs由鳞状上皮样滋养细胞结节和滋养细胞巢组成,胞质丰富嗜酸,常见凋亡细胞,细胞外有纤维素样物质,p63阳性,人类胎盘泌乳素(HPL)阴性。PSTT显示滋养细胞失黏附、多形性、单核,片状排列,浸润纤维素样变的血管,p63阴性,HPL阳性。UTT显示相对窄的形态学谱系,滋养细胞体大,部分呈多核,但缺乏明显的合体滋养细胞,P63和HPL均斑片状阳性。杂交瘤中见ETT样区域和腺癌样区域,两种成分共同表达inhibin和GATA3,但P63和HPL均阴性,所以将其中的滋养细胞成分归类于UTT。病人随访8—96个月,中位数39个月,其中7例存活,而杂交瘤病人死于2年后的肝转移。我们的研究证实,无妊娠绒毛膜癌特征的滋养细胞肿瘤可以发生于睾丸,可以表现为未经治疗的睾丸原发肿瘤,或者是化疗后的转移性肿瘤,应该与绒毛膜癌区分开来,前者的临床病程侵袭性较弱。

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