核型为 46,XX 的处女膜青少年中的性腺母细胞瘤伴精子异常瘤:病例报告和文献综述。

IF 1.5 4区 医学 Q4 ENDOCRINOLOGY & METABOLISM
Tuğçe Kandemir, Esin Karakilic Ozturan, Özlem Dural, Ayça Dilruba Aslanger, Elif İnan Balcı, Aysel Bayram, Semen Önder, Aslı Derya Kardelen Al, Melek Yıldız, Şükran Poyrazoğlu, Firdevs Baş, Feyza Darendeliler
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引用次数: 0

摘要

性腺母细胞瘤是一种罕见的卵巢肿瘤,由性索细胞和原始生殖细胞组成。虽然大多数性腺母细胞瘤发生在 46,XY 性腺发育不良的患者身上,但也很少见于 46,XX 核型的患者。我们报告了一例 14 岁零 6 个月的女孩,她因男性化卵巢肿瘤而导致男性化,这种病因并不常见。患者接受了双侧输卵管切除术。经组织病理学检查,确认切除的肿瘤为双侧性腺母细胞瘤,左侧为发育不良性生殖器瘤。原发性性腺发育不全伴有 46,XX 核型和进行性男性化时,应考虑恶性性腺肿瘤。即使实验室和影像学检查未发现异常,也应考虑进行性腺活检。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Gonadoblastoma with Dysgerminoma in a Virilized Adolescent with Karyotype 46,XX: A Case Report and Review of the Literature.

Gonadoblastoma is a rare ovarian tumor composed of sex cord cells and primitive germ cells. While the majority of gonadoblastomas are found in individuals with 46,XY gonadal dysgenesis, they are also rarely seen in patients with a 46,XX karyotype. We report a case of a fourteen-year-and-six-month-old girl presenting with an uncommon cause of virilization due to a virilizing ovarian tumor. The patient underwent bilateral salpingo-oophorectomy. Upon histopathological examination, the excised tumor was confirmed to be bilateral gonadoblastoma, with dysgerminoma on the left side. Malignant gonadal tumors should be considered in cases of primary gonadal insufficiency with a 46,XX karyotype and progressive virilization. Even when laboratory and imaging tests show no abnormalities, a gonadal biopsy should be considered.

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来源期刊
Journal of Clinical Research in Pediatric Endocrinology
Journal of Clinical Research in Pediatric Endocrinology ENDOCRINOLOGY & METABOLISM-PEDIATRICS
CiteScore
3.60
自引率
5.30%
发文量
73
审稿时长
20 weeks
期刊介绍: The Journal of Clinical Research in Pediatric Endocrinology (JCRPE) publishes original research articles, reviews, short communications, letters, case reports and other special features related to the field of pediatric endocrinology. JCRPE is published in English by the Turkish Pediatric Endocrinology and Diabetes Society quarterly (March, June, September, December). The target audience is physicians, researchers and other healthcare professionals in all areas of pediatric endocrinology.
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