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患者32岁,社会性别男。因腹部包块进行性增大1年余就诊。查体:身高173cm,胡须明显,喉结突出,阴毛呈男性分布,阴茎发育正常,双侧睾丸位于腹股沟管内。下腹部可扪及20cm的包块,活动、质韧、结节状、压痛。于1990年3月12日行剖腹探查术。术中见腹腔有子宫及附件样器官,子宫10×10×5cm,左输卵管连接一19×17×5cm肿块,右输卵管接5×5×4cm肿块,行子宫及肿瘤切除术。病理检查报告:所送标本为子宫及输卵管,左卵巢无性细胞瘤。真二性畸形。术后随访1年,腹腔广泛转移,1991年5月死亡。讨论真二性畸形临床罕见,以患者体内有二性生殖腺为诊断标准。追问家族史,其父母系近亲婚配。本人婚后未生育,2次精液检查均无精子。本例一
Patient 32 years old, male gender. Progressive abdominal mass due to more than 1 year treatment. Examination: Height 173cm, beard obvious, prominent Adam’s apple, pubic hair was male distribution, normal penile development, bilateral testis located in the inguinal canal. Lower abdomen palpable mass 20cm, activity, quality and tough, nodular, tenderness. On March 12, 1990, a laparotomy was performed. Intraoperative see the abdominal cavity with uterine and attachment-like organs, the uterus 10 × 10 × 5cm, the left fallopian tube connected to a 19 × 17 × 5cm mass, right tubal then 5 × 5 × 4cm mass, line uterine and tumor resection. Pathological examination report: sent specimens for the uterus and fallopian tubes, left ovarian dysgerminoma. True gender deformity. Follow-up 1 year after surgery, extensive abdominal metastasis, died in May 1991. Discussion of true bisexual deformity clinically rare, in patients with gender gonads diagnostic criteria. Family history questions, their parents are close relatives of marriage. I have not been born after marriage, no sperm were detected in 2 semen. This example one