混合性性腺发育不全的不典型表现和治疗困境。
Atypical presentation and management dilemma of mixed gonadal dysgenesis.
机构信息
Division of Reproductive Endocrinology and Infertility, Department of Obstetrics and Gynecology, University of Alabama at Birmingham, Birmingham, Alabama 35249, USA.
出版信息
Fertil Steril. 2011 Jan;95(1):291.e7-9. doi: 10.1016/j.fertnstert.2010.05.058. Epub 2010 Jul 15.
OBJECTIVE
To describe a case of 45,X/46,XY mixed gonadal dysgenesis complicated by malignancy with possible metastasis.
DESIGN
Case report.
SETTING
University hospital.
PATIENT(S): A 15-year-old female with primary amenorrhea, short stature, and a vaginal septum.
INTERVENTION(S): Resection of transverse vaginal septum and laparoscopic bilateral gonadectomy.
RESULT(S): The patient had dysgerminoma arising from gonadoblastoma in the left gonad and gonadoblastoma in the right gonad. No normal gonadal tissue could be identified. Postoperative computed tomography scan results were suspicious for lung metastases, but the patient opted for conservative management without chemotherapy.
CONCLUSION(S): Mixed gonadal dysgenesis involves inherent malignancy risk and complex psychosocial issues, which necessitate a multidisciplinary approach to diagnosis and treatment.
目的
描述一例 45,X/46,XY 混合性腺发育不全伴恶性肿瘤及可能转移的病例。
设计
病例报告。
地点
大学医院。
患者
一名 15 岁女性，原发性闭经，身材矮小，阴道横隔。
干预
横隔切开术和腹腔镜双侧性腺切除术。
结果
患者左侧性腺为性腺母细胞瘤恶变的生殖细胞瘤，右侧性腺为性腺母细胞瘤。未发现正常性腺组织。术后计算机断层扫描结果提示肺部转移可疑，但患者选择不化疗的保守治疗。
结论
混合性腺发育不全存在内在的恶性肿瘤风险和复杂的心理社会问题，需要多学科方法进行诊断和治疗。
Zotero 插件