ADRENAL SEX-STEROID PRODUCING TUMOURS (ADRENOGENITAL SYNDROME)
Adrenal sex steroid producing tumors are rare in childhood and may be virilizing (androgen-producing) or feminizing (estrogen-producing).
• Androgen-producing tumors are usually malignant, presenting at any age with virilization in girls and precocious puberty in boys, along with accelerated linear growth and skeletal maturation.
Cushing syndrome may be present in some cases.• Estrogen-producing tumors are extremely rare and usually benign, presenting with heterosexual precocious puberty or gynecomastia in boys and peripheral precocious puberty in girls.
Diagnosis rests on elevated 24-hour urinary 17-keto- steroids and serum androgen/estrogen levels, along with abdominal CT/MRI to localize the tumor. Adrenal calcifications may be present.
Treatment is mainly surgical to enucleate the tumor with/without chemotherapy and radiotherapy.
Clinically these tumors present with features of catecholamine excess, i.e. (a) hypertension of variable severity, including hypertensive encephalopathy, and (b) severe cachexia despite good appetite, due to hypercatabolic state.
Diagnosis rests on elevated plasma/urinary catecholamines and their derivatives with total urinary catecholamine excretion being gt;300 g/24 hour. Fractionated plasma and urinary metanephrines are more useful for diagnosis as catecholamines are secreted episodically whereas metanephrines are continuously produced by tumour cells.
Urinary VMA levels may be falsely elevated after consumption of vanila-containing foods before the test and hence no longer routinely used.
Tumor may be localized by USG, CT/ MRI or 131I-metaiodobenzylguanidine (MIBG) scans.
Treatment is surgical removal of the tumor with preoperative #945;-and #946;-adrenergic blocker therapy, to prevent sudden adrenergic crisis due to tumor spillover and post-operative monitoring for the same.
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