Adrenal Tumors, Sex Hormone-Producing

• • 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

Epidemiology

• • These tumors are rare

  • 70% of virilizing adrenal tumors exhibit malignant behavior

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

Symptoms and Signs

• • 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

Laboratory Findings

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

  • Feminizing adrenal tumors: Elevated plasma or urine estrogens

Imaging Findings

• • Adrenal cortical mass on CT or MRI

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

  • Feminizing adrenal tumors are almost always carcinomas

Rule Out

• • Congenital adrenal hyperplasia

  • Ovarian tumors

  • Testicular feminization

  • Exogenous estrogen administration

• • Complete history and physical exam

  • Plasma androgen levels; urine and plasma estrogen levels

  • Localization with CT or MRI

• • Resection is the only successful treatment

Surgery

Indications

• • All virilizing adrenal tumors should be resected

  • All feminizing adrenal tumors should be excised

Prognosis

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

  • Guarded for feminizing adrenal tumors

References