DYSGENESIS OF TESTICULAR AND STREAK GONADS IN THE SYNDROME OF MIXED GONADAL-DYSGENESIS - PERSPECTIVE DERIVED FROM A CLINICOPATHOLOGIC ANALYSIS OF 21 CASES
DYSGENESIS OF TESTICULAR AND STREAK GONADS IN THE SYNDROME OF MIXED GONADAL-DYSGENESIS - PERSPECTIVE DERIVED FROM A CLINICOPATHOLOGIC ANALYSIS OF 21 CASES
复制标题
DOI：
10.1016/s0046-8177(82)80292-x
复制
发表时间：
1982-01-01
期刊：
影响因子：
3.3
通讯作者：
CRAWFORD, JD
中科院分区：
文献类型：
--
作者：
ROBBOY, SJ;MILLER, T;CRAWFORD, JD
本文对21例混合性性腺发育不全（MGD）的临床和病理进行了研究。15例患者的性腺由肉眼可见的睾丸和条纹状性腺组成; 6例患者有变异，包括2例双侧睾丸和4例双侧条纹状性腺或肿瘤。在功能上，性腺是无能的。睾丸不能完全抑制苗勒管的发育;它们不能支持中肾管结构的完全分化，不能使外生殖器的发育充分男性化，或者经常不能调解自己的血统，导致内外生殖器的不对称。没有一个条纹性腺介导正常的女性青春期生育能力的发展。显微镜检查显示，每个性腺，无论其外观，形态异常。虽然具有生精小管的性腺通常发育到中等程度的晚期状态，宏观上类似于睾丸，但门区仍然结构紊乱;皮质总是缺乏超过一个基本的图尼卡白蛋白层或表现出部分卵巢分化，有时甚至有一个罕见的原始卵泡。随着时间的推移，曲细精管萎缩和玻璃样变。在显微镜下观察到与条纹性腺大体相似的性腺由与正常卵巢皮质相似的基质组成。在几岁以上的患者中，条纹性腺中的生殖细胞的全部补体消失。建议将MGD患者按女性抚养。早期切除性腺可预防性腺母细胞瘤和无性细胞瘤的发生。如果保留子宫并随后给予患者外源性雌激素，则应注意早期发现子宫内膜癌或其前体发展的任何迹象，这些患者可能易于发生。
The clinical and pathologic aspects of 21 cases of mixed gonadal dysgenesis (MGD) were studied. The gonads in 15 patients consisted of a macroscopic testis and a streak gonad; 6 patients had variants, including 2 with bilateral testes and 4 with bilateral streak gonads or tumors. Functionally, the gonads were incompetent. Testes failed to completely inhibit Mullerian development; they failed to support full differentiation of mesonephric duct structures, failed to adequately masculinize development of the external genitalia or often failed to mediate their own descent, resulting in asymmetry of the internal and external genitalia. None of the streak gonads mediated normal female adolescent development of fertility. Microscopic examination revealed that every gonad, regardless of its gross appearance, was morphologically abnormal. Although gonads with seminiferous tubules usually developed to a moderately advanced state, macroscopically resembling testes, the hilar zone remained architecturally disorganized; the cortex invariably lacked more than a rudimentary tunica albuginea or exhibited partial ovarian differentiation, sometimes even with a rare primordial follicle. Over time, the seminiferous tubules atrophied and hyalinized. Gonads that grossly resembled streak gonads were observed microscopically to be composed of a stroma resemblng that of normal ovarian cortex. In patients more than several years of age, the entire complement of germ cells in streak gonads disappeared. It is suggested that patients with MGD be raised as females. Early removal of gonads will prevent the development of gonadoblastoma and dysgerminoma. If the uterus is retained and the patient is subsequently given exogenous estrogen, care should be taken to detect early any signs of the development of endometrial carcinoma or its precursor, to which these patients may be prone.