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  • • 2 types: virilizing and feminizing

    • Virilization is due to hypersecretion of adrenal androgens, mainly dehydroepiandrosterone (DHEA), its sulfate derivative (DHEAS), and androstenedione. All of these are converted peripherally to testosterone and 5α-dihydrotestosterone

    • Very rarely do virilizing adrenal tumors secrete only testosterone

    • Estrogens are not normally synthesized by the adrenal cortex

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Epidemiology

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  • • These tumors are rare

    • 70% of virilizing adrenal tumors exhibit malignant behavior

    • Virilizing adrenal tumors in children are less likely to be malignant

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Symptoms and Signs

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  • • Virilization includes hirsutism, male pattern baldness, acne, deep voice, male musculature, irregular menses or amenorrhea, clitoromegaly, and increased libido

    • Rapid linear growth with advanced bone age is common in children with virilizing tumors

    • Feminization in men (bilateral gynecomastia, accelerated growth rate, female hair distribution, advanced bone age), or precocious puberty in women

    • Feminizing adrenal tumors may present with vaginal bleeding in adult women

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Laboratory Findings

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  • Virilizing adrenal tumors: Elevated DHEA, DHEAS, androstenedione, and testosterone

    Feminizing adrenal tumors: Elevated plasma or urine estrogens

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Imaging Findings

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  • • Adrenal cortical mass on CT or MRI

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  • • Differentiating between benign and malignant virilizing adrenal tumors, even with histopathology, is very difficult

    • Feminizing adrenal tumors are almost always carcinomas

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Rule Out

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  • • Congenital adrenal hyperplasia

    • Ovarian tumors

    • Testicular feminization

    • Exogenous estrogen administration

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  • • Complete history and physical exam

    • Plasma androgen levels; urine and plasma estrogen levels

    • Localization with CT or MRI

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  • • Resection is the only successful treatment

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Surgery

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Indications

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  • • All virilizing adrenal tumors should be resected

    • All feminizing adrenal tumors should be excised

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Prognosis

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  • • Worse for large virilizing tumors (> 100 g)

    • Guarded for feminizing adrenal tumors

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References

Cordera F et al: Androgen-secreting adrenal tumors. Surgery 2003;134:874.  [PubMed: 14668717]
Latronico AC et al: Extensive personal experience: adrenocortical tumors. J Clin Endocrinol Metab 1997;82:1317.  [PubMed: 9141510]
Liou LS et al: Adrenocortical carcinoma in children. Review and recent innovations. Urol Clin North Am 2000;27:403.  [PubMed: 10985141]
Merk DP et al: NIH conference. Future directions in the study and management of congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Ann Intern Med 2002;136:320.
Moreno S et al: Profile and outcome of pure androgen-secreting adrenal tumors in women: experience of 21 cases. Surgery 2004;136:1192.  [PubMed: 15657575]

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