Children should not have to deal with adrenal tumors, but unfortunately, sometimes they and their parents do. Although adrenal tumors in children are quite uncommon, they do occur. Fortunately, most of them are benign, and can be cured with adrenal surgery (adrenal gland removal), including using the best operation: Mini Back Scope Adrenalectomy (MBSA).

Adrenal tumors in children: What you need to know:

1. The most common adrenal tumors in children are pheochromocytomas and cortisol-producing adrenal tumors/hyperplasia (Cushing’s syndrome)
2. Adrenal tumors in children are often due to an inherited tumor susceptibility syndrome
3. Adrenal cancer can occasionally occur in children
4. The Mini Back Scope Adrenalectomy (MBSA) is the best operation for almost all adrenal tumors in children

#1 Thing to Know about Adrenal Tumors in Children:

The most common adrenal tumors in children are pheochromocytomas and cortisol-producing adrenal tumors/hyperplasia (Cushing’s syndrome)

Pheochromocytomas are tumors of the adrenal gland that produce excess adrenaline (also referred to as catecholamines; epinephrine, metanephrine, and dopamine). Pheochromocytomas arise from the central portion of the adrenal gland, which is called the adrenal medulla. Paragangliomas are similar to pheochromocytomas and arise from the same cell type (extra-adrenal chromaffin cells) but arise outside of the adrenal gland.  Both of these tumors occur in children, usually as part of an inherited tumor susceptibility syndrome. In today’s age of genetic testing and counselling, many of these children can be genetically diagnosed even before they develop a tumor or have problems with symptoms. If they do develop symptoms, they tend to be similar as in adults. Read more about the symptoms for paraganglioma and pheochromocytoma.

Adrenocortical adenomas (ACA) are very common in adults, but rare in children. However, if they do occur, they are more likely to be functional, meaning hormone overproducing. Cortisol hypersecretion leading to Cushing’s syndrome is the most common in children, although primary hyperaldosteronism also can occur.

Furthermore, children may develop adrenocortical hyperplasia (meaning it affects both glands) and they most commonly present with Cushing’s syndrome. The most common variants are isolated micronodular adrenal disease and its variant, primary pigmented adrenocortical disease, as well as primary bilateral macronodular adrenal hyperplasia (PBMAH) 

Adrenocortical carcinoma (adrenal cortex-derived cancer) is a very aggressive disease that affects children with an annual incidence of 0.2-0.38 pediatric cases/million. In the pediatric population it represents 0.2% of all cancers. It is more common in girls than boys. A total of 80-90% of cases have hormonal imbalances such as Cushing syndrome, virilization, and puberty anomalies.

Figure 1. Bilateral (both left and right) pheochromocytomas seen on a CT scan (arrows) in a young patient with inherited pheochromocytomas. The patient has Multiple Endocrine Neoplasia type 2 (MEN2), which is due to a RET gene mutation.

#2 Thing to Know about Adrenal Tumors in Children:

Adrenal tumors in children are often due to an inherited tumor susceptibility syndrome

Altogether, a germline genetic mutation (this is a DNA alteration you inherit form a parent) in a known tumor gene is identified in 35-40% of patients with pheochromocytoma and paraganglioma. In children, this percentage is even higher, above 70-80%. An inherited germline mutation is more common in paraganglioma than pheochromocytoma. Dr. Carling and others recommend that all patients with these tumors are offered genetic testing and counselling.

Broadly, the types of genetic alterations can be divided in 3 clusters:

• pseudohypoxia-related clusters 1A and 1B
• kinase signaling–related cluster 2
• Wnt signaling–related cluster 3.

The most commonly altered pheochromocytoma and paraganglioma susceptibility genes are: RET, VHL, NF1, SDHA, SDHAF2, SDHB, SDHC, SDCD, TMEM127 and MAX.

In children, Dr. Carling most frequently operates on pheochromocytoma and paraganglioma with Multiple Endocrine Neoplasia type 2 (RET gene), von Hippel Lindau (vHL) disease (vHL gene), Neurofibromatosis type 1 (NF1) and familial pheochromocytoma and paraganglioma syndrome (SDHx genes).

Hereditary syndromes associated to adrenocortical carcinoma include Li-Fraumeni, Beckwith-Wiedemann, Multiple Endocrine Neoplasia type 1 (MEN1), and Lynch syndrome. Recently, mutations in epidermal growth factor receptor have been reported in teenagers, suggesting the future use of tyrosine kinase inhibitors. Genetic testing and counselling are recommended for all children with adrenocortical carcinoma.

Figure 2. CT scan showing a 3 cm left pheochromocytoma (arrow) in a 12-year-old boy with von Hippel Lindau (vHL) disease due to an inherited mutation in the vHL gene.

#3 Thing to Know about Adrenal Tumors in Children:

Adrenal cancer can occasionally occur in children

Adrenocortical carcinoma (ACC) is a very aggressive disease both in children and adults. A total of 80-90% of cases have hormonal imbalances such as Cushing syndrome, virilization, and puberty anomalies. Precocious puberty (PP) is common and may lead to accelerated growth while Cushing’s syndrome by reduced growth velocity.

Learn more about the top symptoms of adrenal cancer.

Adrenalectomy is the first line therapy offering the best prognosis if complete tumor removal is achieved. Surgery is the only potentially curative treatment. Unfortunately, in too many children, the disease is already spread (stage 4) at the time of diagnosis. Genetic testing is recommended before surgery. Adjuvant therapies are less standardized in children (mitotane is a key adjuvant drug in addition with different regimes of chemotherapy such as etoposide, Adriamycin and cisplatin). The prognosis of ACC is unfortunately poor with an overall 5-year survival rate of 55%. At present, pediatric adrenocortical carcinoma still represents a severe condition that requires prompt intervention.

#4 Thing to Know about Adrenal Tumors in Children:

The Mini Back Scope Adrenalectomy (MBSA) is the best operation for almost all adrenal tumors in children

The best adrenal operation for about 95% of adrenal tumors in children is the posterior retroperitoneal adrenalectomy, or more simply put, the Mini-Back Scope Adrenalectomy (MBSA).

The Mini Back Scope Adrenalectomy (MBSA) is the best adrenal operation. It is much faster than any other technique, the scopes are placed directly into the space where the adrenal glands are, requiring less dissection. A swift operation means that your surgeon is skilled and efficient with an excellent team in-place to best care for you. A faster operation means less time under general anesthesia, less opportunity for blood pressure and heart rate fluctuations, which can be extreme during an adrenal operation especially for pheochromocytoma. Even though general anesthesia is very safe, the longer duration of time that you are under anesthesia, the greater the risk of anesthesia-related complications such as nausea, vomiting, blood clots, pulmonary embolism (blood clots to the lungs), headache, and in some cases, long-term effects on cognition and memory.

Figure 3. Intraoperative photo of a 3 cm left pheochromocytoma (arrow) in a 12-year-old boy with von Hippel Lindau (vHL) disease due to an inherited mutation in the vHL gene. Dr. Carling removed the tumor using the Mini Back Scope Adrenalectomy (MBSA) in 16 minutes

Learn why the Mini Back Scope Adrenalectomy (MBSA) is the best adrenal operation.

As mentioned, paragangliomas may be encountered anywhere from the base of the skull to the urinary bladder, and other common extra-adrenal locations include the chest and neck areas. Thus, the surgical approach is dependent on the location, involvement of major vessels, and the body habitus of the patient.

For Adrenocortical carcinoma (ACC), the surgical approach may be dependent on

• The size of the tumor (as measured on preoperative X-ray: oftentimes a CT scan, MRI, or specialized nuclear medicine scan)
• The type of adrenal tumor (Is it very large? Is it producing too much hormone? Does it look cancerous?)
• The appearance of the tumor on preoperative X-ray (Is the tumor involving surrounding structures, such as organs or vessels?)

Figure 4. Thumbs up! Next stop, Legoland!

Thyroid and parathyroid tumors can occur together with adrenal tumors in children, or individually. Learn more about thyroid tumors, thyroid cancer and parathyroid tumors and primary hyperparathyroidism.

Additional Resources: 

• Learn more about the Carling Adrenal Center
• Learn more about our sister surgeons at the Norman Parathyroid Center, Clayman Thyroid Center and Scarless Thyroid Surgery Center
• Learn more about the Hospital for Endocrine Surgery