SUN-484 - A Rare Case of Androgen-Secreting Adrenal Tumor Causing Severe Virilization in A 10-Year-Old Girl.

A 10-year-old Hispanic girl was referred to the Pediatric Endocrinology clinic for evaluation of a newly discovered left adrenal mass. She reported progressive facial and body hair growth and voice deepening for over a year. Menarche began at 9 years of age; however, she had not had another menstrual cycle. The rest of her medical history was noncontributory, including denial of exposures to exogenous hormones or steroids, and no known family history of endocrine disorders or adrenal tumors. Her primary care provider began an initial evaluation due to significant hirsutism and voice deepening. Laboratory results showed markedly elevated serum testosterone at 362 ng/dL (reference range: 1 - 33 ng/dL) and a DHEA-S level exceeding 3000 ng/dL (reference range: 35.0 -192.6 ug/dL). An abdominal MRI revealed a well-circumscribed, approximately 8 cm mass arising from the left adrenal gland.
Review of her growth chart showed a steady rise in both height and weight, with her height at the 95th percentile and weight above the 99th percentile, corresponding to a BMI at the 98th percentile. Physical exam showed signs of virilization, including coarse dark hair over the chest and abdomen (figure 1), male-pattern scalp hair thinning, and a deep, hoarse voice. Her Blood pressure was at the 97th percentile for systolic and 89th percentile for diastolic for her age and height. There were no Cushingoid features, striae, acne, or signs of acanthosis nigricans.
Given these findings, a comprehensive hormonal workup was initiated to define the functional status of the adrenal mass and to rule out other hormone-secreting tumors. Ordered labs included: Overnight dexamethasone suppression test, ACTH and cortisol, 17-hydroxyprogesterone, Aldosterone and plasma renin activity, Plasma metanephrines (to exclude pheochromocytoma), β-HCG, AFP, and urine VMA/HVA (to rule out germ cell tumors and neuroblastoma); all results were within normal reference range. Bone age was 15 years, which is 5 years in advance of chronological age.
A multidisciplinary team (Endocrinology, Surgery, and Oncology) was assembled to plan management. The surgical team performed a laparoscopic left adrenalectomy, and the postoperative pathology confirmed a benign adrenocortical adenoma. Postoperatively, her hormone levels normalized, and she has had improvement in hirsutism and voice deepening.

Conclusion:
Severe virilization with markedly elevated DHEA-S in a child strongly suggests an adrenal source of androgen excess. Early identification of virilization symptoms is critical, as prompt intervention can prevent irreversible changes, such as voice deepening. We recommend that patients with early signs undergo evaluation to distinguish between other causes of virilization, like PCOS.
Early multidisciplinary assessment and surgical intervention in adrenal tumors are the key to ensure appropriate treatment and optimal patient outcome.

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