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婴儿和儿童睾丸卵黄囊瘤:33例临床病理特征分析

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

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.
作者报道33例从1918-2014年期间诊断的发生在5-71个月(平均20.7个月)男孩睾丸的纯卵黄囊瘤。除1例外所有患者均接受睾丸切除,18例同时行淋巴结清扫(全部阴性);21例接受化疗,12例接受放疗。瘤体直径1.6-7.0cm(平均3.7cm),无包膜,灰色到黄色,切面常常黏液样。最常见的结构是网状-微囊型,但是大囊型、乳头状、内胚窦样型(S-D小体)、迷路样、黏液样、腺管状或实性的结构同样可以见到。32例患者可获得随访资料(平均随访100.5个月,范围3-456个月)。24例患者(包括4例未接受辅助治疗的患者)无病生存;8例发生转移性疾病,其中5例死于疾病,1例死于治疗并发症,2例给予放疗伴或不伴化疗。I期病例中如果出现以下两种或两种以上因素,与患者预后差密切相关,包括肿瘤大小>4.5 cm4/6肿瘤[67%])、浸润睾丸网和/或附睾(3/7 肿瘤[43%])和坏死(6/17 肿瘤 [35%])。在非转移性肿瘤组,2个或2个以上的不利因素仅出现在3/2413%)肿瘤中(P=0.0001)。卵黄囊瘤与幼年性颗粒细胞瘤的鉴别也非常重要,后者发病年龄稍微更年轻,尽管形态学有些重叠,但后者具有一些独特的特征。发生在年轻男孩睾丸卵黄囊瘤的存活情况非常好,可能由于该年龄段本组肿瘤的固有特性和有效的化疗造成的。

标签:睾丸 卵黄囊瘤 临床病理
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