Are Adrenal Tumors in Children Common?
Adrenal tumors in children and adolescents are relatively rare. The most common is neuroblastoma, a childhood cancer. Others include adrenocortical adenomas, pheochromocytomas, ganglioneuromas, ganglioneuroblastomas, and adrenocortical carcinomas. We discuss these below or in other sections of this website.
Hormone-producing Benign Adrenal Tumors in Children.
Children may have too much aldosterone, giving them a diagnosis of primary aldosteronism (PA). In children, PA is more often inherited (familial) than caused by a single tumor. Children can also develop a tumor or nodules producing the adrenal hormone cortisol. These can be inherited (genetic predisposition), and can be within one or both adrenal glands. We have more about cortisol-producing tumors in the adrenal hypercortisolism (AHC) and Cushing syndrome section of this website.
Adrenal Tumors in Children Are Not Like Those in Adults
Genetic syndromes in children with adrenal tumors. In children and adolescents, a much higher share of adrenal tumors, including pheochromocytomas, paragangliomas, and adrenocortical carcinomas, is caused by an inherited genetic syndrome. Genetic testing and counseling should be offered to every child with one of these tumors. As in adults, we believe operations in children should be performed by a surgeon who does adrenal surgery regularly, whether by a minimally invasive or an open approach.Pheochromocytomas and paragangliomas are rare tumors in children and adolescents. Most of them cause symptoms because they release too much catecholamine (adrenaline-type) hormone: typically hypertension, palpitations, sweating, and headaches. About 1% of children with high blood pressure have a pheochromocytoma or paraganglioma. Read more about pheochromocytoma in children.
The majority of patients whose tumors arise during childhood carry an inherited (germline) pathogenic variant in one of the susceptibility genes (in parentheses): von Hippel–Lindau syndrome (VHL), multiple endocrine neoplasia type 2 (RET), neurofibromatosis type 1 (NF1), the paraganglioma syndromes type 1–5 (SDHD, SDHAF2, SDHC, SDHB, SDHA), and variants in MAX, HIF2A (EPAS1), FH and TMEM127. You can learn more on our page about pheochromocytomas and paragangliomas.
All pheochromocytomas and paragangliomas have some potential to spread (metastasize), although most never do. The risk is related to the underlying gene and is highest in patients carrying variants of the SDHB gene. Surgery, either open or with a scope, is the treatment of choice for pheochromocytomas and paragangliomas. For adrenal pheochromocytomas and adenomas in older children and adolescents, the Mini Back Scope Adrenalectomy (MBSA) is our preferred technique, as it is in adults. In children with hereditary disease, adrenal-sparing (partial) adrenalectomy is often the right choice, because they are at risk of tumors in both adrenal glands over a lifetime.
Adrenocortical carcinoma (ACC) in children and adolescents represents a unique challenge. ACC is a rare but aggressive childhood cancer with a reported incidence of 0.2–0.3 new cases per 1 million children per year. There are remarkable geographical variations, with the incidence in parts of southern Brazil being many times higher than in the United States. This is due to an inherited (founder) variant of the TP53 gene that is common in that region. In children, ACC most commonly presents with virilization (early puberty-like changes), with or without cortisol excess (Cushing syndrome), and rarely with feminization or aldosterone excess. ACC can also be nonfunctional and diagnosed incidentally during the evaluation of abdominal pain, fatigue or other nonspecific symptoms. Many childhood ACCs are associated with an inherited genetic change, so genetic testing should be offered to every child with ACC. Individuals with Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, multiple endocrine neoplasia type 1, and familial adenomatous polyposis (FAP) have an increased risk of ACC. Surgery, usually an open operation, is the mainstay of treatment. Even after complete resection, a high risk of recurrence of ACC remains. Nonetheless, complete removal of the tumor, younger age (under 4 years), a smaller and lighter tumor, a lower stage, and the absence of metastatic disease are favorable prognostic factors.
Neuroblastomas are pediatric tumors requiring a multidisciplinary pediatric oncology team that includes pediatric surgeons and oncologists. We do not treat neuroblastomas at the Carling Adrenal Center; they are best treated at a very high-volume children’s hospital.
Adrenal Tumors (Adrenal Nodules) in Older Adults
Adrenal tumors are common, especially in older individuals. As we age, the chances go up that we will develop an adrenal tumor. Most are found by accident on a CT or MRI scan done for another reason (an adrenal “incidentaloma”). An unsuspected adrenal tumor is found on well under 1% of abdominal CT scans in people in their twenties, compared with about 7% or more in people over 70 years of age.The majority of adrenal tumors in patients of all ages are benign adrenocortical adenomas. But they should never simply be assumed to be harmless. Every adrenal tumor, at any age, needs hormone testing: a 1-mg overnight dexamethasone suppression test (DST) with ACTH and DHEA-S, aldosterone and renin if you have high blood pressure or low potassium, and plasma or urine metanephrines. Read more on our adrenal incidentaloma page. The most common hormone problem by far is adrenal hypercortisolism (AHC; formerly called MACS, mild autonomous cortisol secretion, or subclinical Cushing syndrome), and it is commonly missed in older adults because its consequences (high blood pressure, diabetes, osteoporosis and fractures, weight gain) are put down to age. Adrenal tumors can also produce aldosterone (primary aldosteronism, PA), sex steroids or catecholamines (pheochromocytoma). A small number are cancers: adrenocortical carcinoma (ACC), metastatic pheochromocytoma, or metastases to the adrenal from a cancer elsewhere.
Adrenal Surgery in Older Adults: Special Considerations
The overall management principles of adrenal tumors in older patients are similar to those in younger patients. However, the diagnosis and the management may pose particular challenges in older adults:1) The symptoms of hormone excess may be absent, subtle, atypical and/or masked by medications.
2) Hormone testing may be more difficult to interpret because of interference from medications.
3) The index of suspicion (among doctors caring for older patients) may be too low for a diagnosis other than ordinary (essential) hypertension. This is important: do not let people assume you should have high blood pressure just because you are old.
4) Laparoscopic transabdominal surgery may be more difficult because of previous abdominal operations. The Mini Back Scope Adrenalectomy (MBSA) is our preferred technique for most adrenal operations, including in older adults, and especially if you have had previous abdominal operations. It is a short operation, about 20 minutes, and less time under anesthesia is generally better for older patients. The patient lies face down (prone) during the MBSA, so in frail patients, or those with significant heart, lung or spine problems, we consider carefully whether this position is suitable.
Last updated October 2026.