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Sexual precocity in a female infant due to feminizing adrenal carcinoma
Insights
This case study reports on a young child with adrenal adenocarcinoma causing sexual precocity. Surgical treatment was successful, with the patient remaining healthy long-term despite initial tumor invasion.
Area of Science:
- Pediatric Endocrinology
- Pediatric Oncology
- Reproductive Endocrinology
Background:
- Adrenal adenocarcinoma is a rare malignancy in children.
- Sexual precocity can manifest as virilizing or feminizing features due to hormone-secreting tumors.
- This case highlights the complex hormonal effects of adrenal tumors in early childhood.
Purpose of the Study:
- To report a rare case of adrenal adenocarcinoma presenting with both virilizing and feminizing sexual precocity in a pediatric patient.
- To detail the hormonal profile and diagnostic findings associated with this condition.
- To discuss the long-term outcome following surgical management.
Main Methods:
- Case report of a 21-month-old female with sexual precocity.
- Biochemical analysis including urinary neutral 17-ketosteroids and plasma dehydroepiandrosterone sulfate (DHEA-S) and estradiol levels.
- Surgical intervention and long-term follow-up.
Main Results:
- The patient presented with virilizing and subsequent feminizing sexual precocity.
- Elevated urinary 17-ketosteroids and high plasma DHEA-S levels were observed.
- Adult-level plasma and urinary estradiol correlated with significant breast development, clitoral enlargement, and an estrogenized vagina.
- Plasma cortisol levels were within the normal range.
Conclusions:
- Adrenal adenocarcinoma can cause profound and mixed hormonal disturbances leading to sexual precocity in young children.
- Surgical resection is a viable treatment option, even with evidence of capsular invasion.
- Long-term health outcomes can be favorable following successful surgical management of pediatric adrenal adenocarcinoma.
Abstract:
A 21-month-old female with virilizing and then feminizing sexual precocity due to an adrenal adenocarcinoma treated surgically at 21 months of age is reported. Urinary neutral 17-ketosteroids were 116 and 106 mg/24 h and correlated with high plasma levels (1408 microgram/dl) of dehyroepiandrosterone sulfate. Plasma estradiol (17 ng/dl) and urinary estradiol levels (6.3 and 7.1 mu/24 h) were elevated to adult levels and associated with breast development, clitoral enlargement, and an estrogenized vagina. Plasma cortisol was normal. The patient remains healthy over 10 yr later, although capsular invasion was present.