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Yolk Sac Tumor of the Testis in Infants and Children: A Clinicopathologic Analysis of 33 Cases.

婴幼儿睾丸卵黄囊瘤:33例临床病理学分析

Cornejo KM,Frazier L,Lee RS,Kozakewich HP,Young RH

Abstract

We report 33 pure yolk sac tumors of the testis from boys 5 to 71 months of age (mean 20.7 mo) diagnosed from 1918 to 2014. All except 1 underwent orchiectomy, with lymph node dissections (all negative) performed in 18; 21 also received chemotherapy and 12 radiotherapy. The tumors were 1.6 to 7.0 cm (mean 3.7 cm) and were nonencapsulated, with a gray to yellow, often mucoid, cut surface. The commonest pattern was reticular-microcystic, but macrocystic, papillary, endodermal sinus (Schiller-Duval bodies), labyrinthine, myxomatous, glandular, and solid patterns were also observed. Follow-up was available for 32 patients (mean 100.5 mo; range, 3 to 456 mo). Twenty-four patients (including 4 who did not receive adjuvant therapy) were without evidence of disease, 8 had metastatic disease; 5 of the latter died of tumor and 1 of treatment complications. Two patients with metastasis were cured with radiation with or without chemotherapy. Two or more of the following were associated with a poor outcome in patients presenting with stage I cases: tumor size >4.5 cm (4/6 tumors [67%]), invasion of rete testis and/or epididymis (3/7 tumors [43%]), and necrosis (6/17 tumors [35%]). In the nonmetastasizing group, 2 or more unfavorable features occurred in only 3/24 tumors (13%) (P=0.0001). It is crucial that this tumor be distinguished from the juvenile granulosa cell tumor, which occurs at a slightly younger age and has distinctive features, although there may be some morphologic overlap. The survival of young boys with testicular yolk sac tumor is very good because of both effective chemotherapy and likely, the inherent characteristics of the tumor in this age group.

摘要

1918年至2014年间,我们诊断报告了33例发生于男孩睾丸的卵黄囊瘤,这些男孩年龄5-71月(平均20.7月)。除1例之外,其他所有的患者都进行了睾丸切除术,其中18例进行了淋巴结清扫(均为阴性);21例也进行了化疗,12例进行了放疗。肿瘤大小1.6-7.0cm(平均3.7cm),无包膜,切面灰色到黄色,通常为黏液样。最常见的结构为网状-微囊,但是也可以见到较大囊性、乳头状、内胚窦(Schiller-Duval小体)、迷宫样、黏液瘤样、腺样及实性结构。32例患者进行了随访(平均存活时间100.5月;范围3-456月)。24例患者(包括4例未接受辅助治疗患者)无疾病表现,8例发生转移性疾病;5例最近因肿瘤死亡,1例因治疗并发症死亡。2例发生转移的患者采用放疗联合/不联合化疗进行治疗。含有下面所列2个或更多,则与I期患者的不良预后相关:肿瘤大小>4.5cm(4/6,67%)、睾丸网和/或附睾浸润(3/7,43%)以及坏死(6/17,35%)。未发生转移患者,肿瘤具有2个或更多不利特征的仅为3/24(13%)(P=0.0001)。这一肿瘤与幼年性粒层细胞瘤鉴别非常重要,两者可能有一些形态学的重叠,但是后者发生的年龄较其稍年轻并且具有特征性的特点。由于有效的化疗以及这一年龄组肿瘤可能固有的特性,患有睾丸卵黄囊瘤的幼年男孩的存活率非常高。
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