Adrenal Tumors in Children

There are two adrenal glands, one on top of each kidney toward the back. Tumors of the adrenal glands are rare. However, when present, they can cause many problems by excess secretion of certain adrenal-produced hormones. One type of tumor of the adrenal glands is called a pheochromocytoma.

Adrenocortical carcinoma is a rare malignant neoplasm of adrenal glands, which most often presents without any hormonal symptoms. The most common clinical presentation of patients with hormone-secreting adrenocortical carcinoma is that of CushingÕs syndrome. Other hormonal hypersecretion syndromes associated with adrenocortical carcinoma include virilization (from androgen-producing tumors), feminization (estrogen-producing tumors), and hyperaldosteronism. Multiple hormones may be produced by a single tumor, causing a mixed clinical picture.

Tumors of the adrenal glands are rare. However, when present, they can cause a multitude of disorders by excessively secreting certain adrenal-produced hormones. One type of tumor of the adrenal glands is called a pheochromocytoma.

The inner part of the adrenal gland is called the adrenal medulla. The adrenal medulla produces hormones called catecholamines such as adrenaline and noradrenaline. Catecholamines play a role in the response to acute or sudden severe stress, for example during life threatening event.
Catecholamines are responsible for the palpitations (racing heart), sweatiness, widening of eyes and shakiness of the hand when faced with sudden fear or other stressful situation.

Survival rates for children with kidney tumors approach 90% for even the most advanced stages of disease, but the surgical management of large lesions remains challenging. With the development of additional chemotherapeutic regimens and the use of radiation therapy, survival rates have improved dramatically. The National Wilms’ Tumor Study has conducted four long-term studies addressing how adjunctive therapy may be tailored optimally to maximize survival and minimize the exposure to chemotherapy and radiation therapy.

Morphologic features (abnormal mitoses, necrosis, vascular and capsular invasion, broad fibrous bands, cellular pleomorphism, size) previously suggested to be predictors of malignant behavior in adrenal cortical tumors were assessed individually in 23 (17 benign, 6 malignant) pediatric and 42 (29 benign, 13 malignant) adult tumors. Of these features, size was the only predictor of malignancy in pediatric tumors. All pediatric tumors weighing more than 500 g were malignant and all but one weighing less than 500 g were benign.

Three seemingly occult secretory tumors in children (pheochromocytoma, ganglioneuroblastoma and islet cell carcinoma) were localized within the abdomen by computed tomography after other diagnostic imaging procedures had failed. The superb density resolution and tomographic formating of CT images make CT uniquely suited for the demonstration of small abdominal lesions. CT is recommended as a primary imaging modality for secretory tumors in children.

For a hormone to affect the cell, it must first bind to a molecule, or receptor, on the cell’s surface, analogous to how a key fits into a lock. This action triggers the cell to produce substances known as cyclic nucleotides. These function as ‘second messengers,’ often stimulating the cell to begin an activity. In the case of adrenal cells, cyclic nucleotides, such as cyclic AMP and cyclic GMP, may stimulate cell growth or other activities. Once the activity has ended, phosphodiesterases degrade the cyclic nuke.

A three-year old female child presented with a 2 month history of bilateral breast development, bleeding per vaginum and pubic hair which had appeared 1 month previously. There were no complaints of deepening of voice, acne, or hirsutism. On physical examination, there was bilateral breast development and appearance of pubic hair, without clitoral enlargement or thickening of vaginal mucosa ( Fig 1).