Are Adrenal Tumors in Children Common?Adrenal tumors in children and adolescents are relatively rare. Adrenal tumors in children tend to be neuroblastomas, adrenocortical adenomas, pheochromocytomas, ganglioneuromas, ganglioneuroblastomas, and adrenocortical carcinoma. We discuss each of these below or on other sections of this large website (you can use the search function to find specific topics).
Hormone Producing Benign Adrenal Tumors in Children.
Children may suffer from tumors that produce too much aldosterone giving them a diagnosis of primary hyperaldosteronism. Children can also develop a tumor producing the adrenal hormone cortisol. These can be inherited and genetically based, and can be within one or both adrenal glands. We have more about cortosol producing tumors in the Cushing's Syndrome section of this large website.
Adrenal Tumors in Children are not Like Those in AdultsGenetic Syndromes in children with adrenal tumors.
In children and adolescents, there is a disproportionately higher rate of syndromic (i.e. genetic; see the molecular genetic section of each subset of tumor) adrenal tumors including pheochromocytomas, paragangliomas, and adrenocortical carcinoma. As in adults, operations in children should be performed by the highest-volume adrenal surgeon, whether it is performed by a minimally invasive or open approach (Dr. Carling’s personal opinion).
Pheochromocytoma and paraganglioma are rare tumors in children and adolescents. Their incidence in the pediatric population is around 0.2–0.3 cases per million. The majority of these tumors are clinically symptomatic due to the hypersecretion of catecholamines and their metabolites. They typically cause hypertension, palpitations, sweating, and headaches. Nevertheless, only about 1% of pediatric cases with arterial hypertension suffer from a pheochromocytoma or paraganglioma.
The majority of the patients with tumors arising during childhood are carriers of germ line mutations of the susceptibility genes (in brackets) which predispose to von Hippel–Lindau syndrome (VHL), Multiple Endocrine Neoplasia type 2 (RET), Neurofibromatosis type 1 (NF1), Paraganglioma Syndromes type 1–5 (SDHD, SDHAF2, SDHC, SDHB, SDHA), MYC-associated factor X (MAX), hypoxia-inducible factor 2A (HIF2A), fumarate hydratase (FH), and transmembrane protein 127 (TMEM127). To learn more about the molecular genetics of pheochromocytoma and paraganglioma, please view this page.
Most of pheochromocytoma and paraganglioma tumors are benign. However, the risk of malignancy is related to the underlying mutation and occurs mainly in patients carrying mutations of the SDHB gene. Surgery, either open or by endoscopic (scope adrenalectomy) approach, is the treatment of choice in pheochromocytoma and paraganglioma. Similarly, as in adults the posterior retroperitoneoscopic adrenalectomy (PRA) is the preferred technique in the vast majority of pediatric adrenal surgery cases.
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 is remarkable geographical variation with the incidence in parts of Brazil being 12–18 times higher than in the United States. This is due to an accumulation of p53 mutations in this geographical area. Similar as in adults, ACC commonly presents with virilization and Cushing syndrome and rarely with feminization and hyperaldosteronism. ACC can also be nonfunctional and diagnosed incidentally during the evaluation of abdominal pain, fatigue or other nonspecific symptoms. The majority of ACC are sporadic, but some are associated with a genetic defect. Individuals with the Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, multiple endocrine neoplasia type 1, and familial adenomatous polyposis (FAP) have an increased risk of ACC. Surgery is the mainstay of treatment. Even after complete resection, a high risk of recurrence of ACC remains. Nonetheless, the quality of surgery, age < 4 years, tumor size < 10 cm, tumor weight and stage, and absence of metastatic disease are positive and important prognostic markers.
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 in the ElderlyAdrenal tumors are common, especially in older individuals. As we age, the chances go up that we will develop an adrenal tumor. Numerous autopsy studies have examined the frequency of adrenal tumors, and adrenal lesions >1 cm are identified in 6 % of patients. Similarly, the prevalence of adrenal “incidentalomas“ is 4% when detected by abdominal CT or magnetic resonance imaging (MRI). The incidence of adrenal tumors increases with age. For instance, the likelihood of detecting an unsuspected adrenal lesion on an abdominal CT scan in individuals between 20 and 29 years of age is only 0.2% versus 7 % in individuals over 70 years of age.
The majority of adrenal tumors in patients of all ages are clinically non-functioning (making no hormone), benign adrenocortical adenomas and usually do not require surgical intervention. However, as we get older, the chances that the adrenal tumor will be functional (making hormone) increases. When dealing with the eldrly population we are faced with patients with functioning benign adrenal tumors much more commonly. The hormones these tumors can produce include: cortisol-, aldosterone- sex-steroids-, and catecholamine-producing pheo's, as well as malignant adrenal tumors (adrenocortical carcinoma; ACC, malignant pheochromocytoma, and metastasis to the adrenal).
Adrenal Surgery in the Elderly: Special ConsiderationsThe overall management principles of adrenal tumors in older patients are similar to their younger counterparts. However, the diagnosis as well as the management may provide unique challenges in the elderly:
1) the symptoms of hormone excess may be absent, subtle, atypical and/or masked by medications.
2) the biochemical diagnosis of endocrine hypersecretion may be cumbersome due to drug interference.
3) the index of suspicion (among doctors caring for older patients) may be too low for an alternative diagnosis to 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 due to previous open abdominal procedures. Again, the Mini-Posterior Retroperitoneal Adrenalectomy (Mini-PRSA) (the Mini-Back-Scope-Adrenalectomy) is the preferred technique in more than 95 % of adrenal surgery cases--even in the elderly population. This is especially true if you have had previous abdominal operations or you are in your golden years.
Table Comparison of indications for adrenalectomy in all versus elderly patients. Diagnosis % All patients % >65 years of age Pheochromocytoma 22–43 19 Adrenocortical carcinoma 18 8 Cushing’s syndrome 17–18 22 Nonsecreting adenoma 16–18 31 Hyperaldosteronism 11–19 20 Virilizing/feminizing tumor 6 <1 Myelolipoma 4 <1 Cyst 2–5 <1 Metastasis 1 <1