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一名性早熟婴儿的颗粒细胞瘤(GCT)中的类固醇受体。

Steroid receptors in a granulosa cell tumor (GCT) in an infant with precocious puberty.

作者信息

Leiberman E, Glezerman M, Karny N, Levy J

出版信息

Med Pediatr Oncol. 1985;13(6):370-4. doi: 10.1002/mpo.2950130614.

DOI:10.1002/mpo.2950130614
PMID:4046974
Abstract

A granulosa theca cell tumor was found in a 10-month-old girl with precocious puberty. Steroid hormone receptors were measured in fractionated tumor cells. Estrogen and progesterone receptors were measured in the cytosol, and estrogen receptors were measured in the nuclear fraction of the tumor. Although high affinity (Kd 0.4 nM) progesterone receptors were present in the cytosol of this tumor-54 fmole/mg protein-no cytosolic estradiol receptors could be detected; however, these receptors were found to be tightly bound to the nuclear fraction of the tumor. The nuclear protein had lower apparent Kd for the hormones-5.4 nM. This tumor produced and released various steroid hormones. The plasma steroid levels before removal of the tumor were 1,200 pg/ml for estradiol, 350 pg/ml for testosterone, and 5 ng/ml for progesterone. All these steroid hormones declined postoperatively to undetectable levels, with total remission of pubertal signs. Plasma-dehydroepiandrosterone, prolactin, and gonadotropin levels were in the normal range before and after surgery. These results suggest that cytosolic progesterone receptor as an estrogenic marker and/or nuclear estrogen receptors may predict the hormonal dependency of these estrogen-producing tumors.

摘要

在一名患有性早熟的10个月大女孩中发现了颗粒卵泡膜细胞瘤。对分离出的肿瘤细胞进行了类固醇激素受体检测。在肿瘤细胞的胞质溶胶中检测了雌激素和孕激素受体,在肿瘤的核部分检测了雌激素受体。尽管该肿瘤的胞质溶胶中存在高亲和力(解离常数Kd为0.4 nM)的孕激素受体——54飞摩尔/毫克蛋白,但未检测到胞质溶胶中的雌二醇受体;然而,发现这些受体紧密结合于肿瘤的核部分。核蛋白对激素的表观解离常数较低——5.4 nM。该肿瘤产生并释放多种类固醇激素。切除肿瘤前,血浆中雌二醇水平为1200 pg/ml,睾酮水平为350 pg/ml,孕酮水平为5 ng/ml。术后所有这些类固醇激素均降至无法检测的水平,青春期体征完全缓解。手术前后血浆脱氢表雄酮、催乳素和促性腺激素水平均在正常范围内。这些结果表明,胞质溶胶中的孕激素受体作为雌激素标记物和/或核雌激素受体可能预测这些产生雌激素的肿瘤的激素依赖性。

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