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伴有性腺器官罕见恶性转化的男性两性畸形:一例报告

Male hermaphroditism with rare malignant transformation of gonadal organs: a case report.

作者信息

He Zhengyu, Li Zhiyuan, Chen Mei

机构信息

Department of Urology, Ya'an People's Hospital, No. 9 Ankang Road, Yucheng District, Ya'an City, Sichuan, China.

出版信息

Discov Oncol. 2024 Aug 16;15(1):351. doi: 10.1007/s12672-024-01222-7.

DOI:10.1007/s12672-024-01222-7
PMID:39147989
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11327227/
Abstract

A 27-year-old man was admitted to the hospital after a year of marriage due to infertility. During laparoscopic exploratory surgery, the presence of a retrovesical uterus was clearly observed, and the gonadal organs were visible on both sides. However, the testicles or ovaries were not identifiable, nor were the spermatic vessels and fallopian tubes at the joint. Intraoperative bilateral gonad biopsy was performed. Cryopreservation of the right gonadal gland revealed gonadoblastoma and malignant germinoma (asexual tumor/seminoma) with sclerosis and atrophy of testicular tissue. No proliferation of germ cells and sertoli cells was observed in spermatic tubule. The left gonad was diagnosed as a gonadoblastoma. Finally, total hysterectomy and bilateral gonadal tumor organectomy were performed to seal the vaginal stump. Local radiotherapy was administered after surgery. In general, tumors were found on both sides of the gonads, especially gonadoblastoma and malignant germinoma on the right side and gonadoblastoma on the left side.

摘要

一名27岁男性在婚后一年因不育入院。在腹腔镜探查手术中,清晰观察到膀胱后位子宫,双侧性腺器官可见。然而,未发现睾丸或卵巢,关节处的精索血管和输卵管也未发现。术中进行了双侧性腺活检。右侧性腺冷冻保存显示为性腺母细胞瘤和恶性生殖细胞瘤(无性细胞瘤/精原细胞瘤),伴有睾丸组织硬化和萎缩。在精曲小管中未观察到生殖细胞和支持细胞的增殖。左侧性腺被诊断为性腺母细胞瘤。最后,进行了全子宫切除术和双侧性腺肿瘤器官切除术以封闭阴道残端。术后进行了局部放疗。总体而言,性腺两侧均发现肿瘤,尤其是右侧的性腺母细胞瘤和恶性生殖细胞瘤以及左侧的性腺母细胞瘤。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b0b5/11327227/86a8f453ad02/12672_2024_1222_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b0b5/11327227/c5aaa53af0fb/12672_2024_1222_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b0b5/11327227/9bb74185fd11/12672_2024_1222_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b0b5/11327227/86a8f453ad02/12672_2024_1222_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b0b5/11327227/c5aaa53af0fb/12672_2024_1222_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b0b5/11327227/9bb74185fd11/12672_2024_1222_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b0b5/11327227/86a8f453ad02/12672_2024_1222_Fig3_HTML.jpg

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本文引用的文献

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Sex-limited experimental evolution drives transcriptomic divergence in a hermaphrodite.性限制实验进化导致雌雄同体的转录组差异。
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Dysgerminoma and gonadoblastoma in the course of Swyer syndrome.XY单纯性腺发育不全过程中的无性细胞瘤和性腺母细胞瘤。
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[Clinicopathologic analysis of gonadoblastoma].[性腺母细胞瘤的临床病理分析]
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Gonadoblastoma in patients with 45,X/46,XY mosaicism: A 16-year experience.45,X/46,XY嵌合体患者的性腺母细胞瘤:16年经验
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