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[睾丸女性化综合征(“雄激素不敏感”综合征)患者的肿瘤性疾病——两例病例描述]

[Tumorous diseases in patients with the testicular feminization syndrome ("androgen insensitivity" syndrome)--description of two cases].

作者信息

Hes O, Vanecek T, Síma R, Hora M, Velickinová H, Grossmann P, Kovár J, Michal M

机构信息

Oddelení speciální diagnostiky SPAU, FN Plzen.

出版信息

Ceska Gynekol. 2005 Mar;70(2):113-7.

Abstract

OBJECTIVE

To describe tumors occurring in two cases of testicular feminization syndrome.

SUBJECT

Case report.

SETTING

Dpt. of Special Diagnostics SPAU, University Hospital Plzen.

SUBJECT AND METHOD

Two cases of testicular feminization syndrome were selected from four cases in our registry. Patients were 45 and 84-year-old Caucasian "females". Resected material was fixed in formaldehyde, routinely processed and stained with hematoxiline-eosin, inhibin, cytokeratines 20, placentar alkaline phosphatase, CD 99, Melan A, hCG. Sertoli cell adenoma was diagnosed in both patients. Older patient had in addition unclassified sex cord tumor of Leydig cell type. The number of sex chromosomes was examined using FISH analysis in both patients.

CONCLUSION

Patients with testicular feminization syndrome are frequently affected by benign or malignant tumors in the cryptorchid testes. We documented two benign Sertoli cell adenomas and one sex cord tumor of uncertain biological behavior in our patients. The testes should be removed after puberty with subsequent estrogen therapy in patients with testicular feminization syndrome.

摘要

目的

描述两例睾丸女性化综合征患者所发生的肿瘤。

对象

病例报告。

地点

比尔森大学医院特殊诊断部SPAU。

对象与方法

从我们登记的4例病例中选取两例睾丸女性化综合征患者。患者为45岁和84岁的白种“女性”。切除的组织用甲醛固定,常规处理,并用苏木精-伊红、抑制素、细胞角蛋白20、胎盘碱性磷酸酶、CD 99、黑素A、人绒毛膜促性腺激素染色。两名患者均诊断为支持细胞瘤。年长患者还患有未分类的莱迪希细胞型性索肿瘤。两名患者均使用荧光原位杂交分析检测性染色体数量。

结论

睾丸女性化综合征患者的隐睾常受良性或恶性肿瘤影响。我们记录了两名患者的两例良性支持细胞瘤和一例生物学行为不确定的性索肿瘤。睾丸女性化综合征患者在青春期后应切除睾丸,随后进行雌激素治疗。

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