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Steroid receptors in a granulosa cell tumor (GCT) in an infant with precocious puberty
Insights
A rare granulosa theca cell tumor caused precocious puberty in a young girl. Tumor receptors indicated hormonal dependency, resolving after surgical removal.
Area of Science:
- Endocrinology
- Gynecologic Oncology
- Pediatric Endocrinology
Background:
- Precocious puberty in prepubertal girls can be caused by estrogen-producing ovarian tumors.
- Granulosa theca cell tumors (GTCTs) are the most common type of ovarian sex cord-stromal tumors.
Observation:
- A 10-month-old girl presented with precocious puberty due to a GTCT.
- Steroid hormone receptor analysis was performed on tumor cell fractions.
- Estrogen and progesterone receptors were quantified in cytosol and nuclear fractions.
Findings:
- High-affinity progesterone receptors were detected in the tumor cytosol.
- Cytosolic estrogen receptors were undetectable, but nuclear estrogen receptors were present.
- The tumor produced significant levels of estradiol, testosterone, and progesterone.
- Post-surgical hormone levels normalized, and pubertal signs resolved.
Implications:
- Cytosolic progesterone receptor may serve as an estrogenic marker in GTCTs.
- Nuclear estrogen receptors might indicate hormonal dependency in these tumors.
- Early diagnosis and surgical intervention are crucial for remission.
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.