Christopher P. Coppola, Alfred P. Kennedy, Jr. and Ronald J. Scorpio (eds.)Pediatric Surgery2014Diagnosis and Treatment10.1007/978-3-319-04340-1_63
© Springer International Publishing Switzerland 2014
Adrenal Tumors
(1)
Department of Pediatric Surgery, Janet Weis Children’s Hospital, 100 N. Academy Av. MC 21-70, Danville, PA 17822, USA
Abstract
In children, adrenocortical tumors of 1 cm or larger are rare but can be incidentally found during evaluation for a non-related issue. For this reason, they have earned the nickname “adrenal incidentalomas”.
In children, adrenocortical tumors of 1 cm or larger are rare but can be incidentally found during evaluation for a non-related issue. For this reason, they have earned the nickname “adrenal incidentalomas”.
1.
Pathophysiology:
(a)
Anatomy and physiology: The adrenal glands are small golden endocrine organs that reside along the superior medial aspect of the kidneys. They are composed of an outer cortex and an inner medulla.
(i)
The adrenal cortex is comprised of three separate zones that each function independently
1.
Zona glomerulosa: Secretes aldosterone
2.
Zona fasciculata: Secretes cortisol
3.
Zona reticularis: Secretes androgens
(ii)
The adrenal medulla secretes catecholamines: Epinephrine, norepinephrine.
(b)
Causes of adrenal masses include: Neuroblastoma, adrenal adenoma, adrenocortical carcinoma, pheochromocytoma, adrenal metastases, adrenal hyperplasia, adrenal cysts, and adrenal hemorrhage.
(i)
Adrenal masses other than neuroblastoma are very rare in the pediatric population.
(c)
The majority of adrenal incidentalomas in children are not hormonally active. The adrenal mass that is hormonally active likely produces symptoms which leads to its discovery on CT scan. These patients may present, to varying degrees, with signs of virilization, hypercortisolism, or subclinical Cushing’s syndrome identified through lab work-up.
(d)
Adrenal tumors have been associated with the following syndromes:
(i)
Beckwith-Weidemann, Li-Fraumeni, familial adenomatous polyposis.
2.
Diagnosis:
(a)
History and physical examination:
(i)
History: Palpitations, sweating, headaches.
(ii)
Physical: Hirsutism, penile or clitoral enlargement, Cushingoid features, abdominal pain, hypertension, hypokalemia.
(b)
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Labs:
(i)
24 hour urine cortisol, midnight cortisol level, baseline corticotropin, high dose dexamethasone suppression.
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