Guo L, Liu T, Tian Q
Departments of Pathology and Obstetrics and Gynecology, PUMC Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730.
Zhonghua Bing Li Xue Za Zhi. 1998 Jun;27(3):209-12.
To investigate the gonadal histopathology of true hermaphroditism and its correlation with the clinical features.
Clinico-pathological materials and chromosomal karyotypes from 9 true hermaphroditisms were reviewed.
Seven out of 9 patients aged 5-21 years had been raised as females, and the other two were raised as males. Ovotestis was the most common form of the abnormal gonads, 2 out of 9 patients had bilateral ovotestes, 7 had unilateral ovotestes (5 in right side, 2 in left side). In the 7 patients with unilateral ovotestis, 6 had a contralateral ovary and one had a contralateral testis. Microscopically, the ovarian tissue of 11 ovotestes, including 6 biopsies from contralateral ovaries were normal, with many primordial follicles and a few growing follicles. In two of the patients, aged over 15 years, evidence of ovulation was observed. In comparison, the testicular tissue of the ovotestis and one left inguinal testis was histologically abnormal. Two of the 9 patients conceived and delivered normal infants by cesarean section after surgical treatment.
Identification of the gonadal histopathology in these cases is important in order to make a correct scheme for the treatment.
探讨真两性畸形的性腺组织病理学及其与临床特征的相关性。
回顾9例真两性畸形患者的临床病理资料及染色体核型。
9例患者年龄5～21岁，其中7例自幼按女性抚养，另2例按男性抚养。卵睾是性腺异常的最常见形式，9例中有2例双侧卵睾，7例单侧卵睾（右侧5例，左侧2例）。7例单侧卵睾患者中，6例对侧为卵巢，1例对侧为睾丸。显微镜下，11个卵睾的卵巢组织（包括6例对侧卵巢活检组织）正常，有许多原始卵泡和少数生长卵泡。2例年龄超过15岁的患者有排卵迹象。相比之下，卵睾及1例左侧腹股沟睾丸的睾丸组织在组织学上异常。9例患者中有2例在手术治疗后通过剖宫产成功受孕并分娩出正常婴儿。
明确这些病例的性腺组织病理学对于制定正确的治疗方案很重要。
