Adrenal tumors in children

Posted by Dr Prashant Jain on November 2nd, 2021

Adrenal gland is an important gland responsible for secreting various hormones required for normal body functioning. In pediatric age group the tumors of adrenal gland are rare which arise from adrenal cortex and adrenal medulla.These tumors account for less than 0.2% of all pediatric neoplasms and 1.3% of all carcinomas.

The most common tumor of the adrenal gland in pediatric age group is neuroblastoma which arise from adrenal medulla , while  adrenal carcinoma and adenomas,  tumors of the adrenal cortex, are rare. Pheochromocytoma is a rare and very peculiar tumor of adrenal gland which causes hypertension. The advance adrenal malignant tumor has a poor prognosis.

The adrenal tumorswhich secrete various hormones (called as functioning adrenal tumors) causes various symptoms such as high blood pressure, sweating, palpitation, and early puberty.

The diagnostic work up includes imaging with computed tomography and magnetic resonance imaging, along with evaluation of hormone levels in functioning adrenal tumors.

Neuroblastoma:

Neuroblastoma is a malignant nerve cell tumor and is one of the commonest extracranial solid malignancy of infancy. The tumor requires multimodality approach involving chemotherapy, surgery, radiotherapy and bone marrow transplant with good outcome.

Ganglioneuroma and ganglioneuroblastoma  forms the benign  spectrum of neuroblastoma which can be cured only with surgical resection.

Adrenocortical Tumors

Adrenocortical tumors can be  functional or non-functional. Functional tumors present with features of precocious puberty, obesity, hypertension. Cushing syndrome is a result of high secretion of cortisol causing central obesity, thin arms and legs, moon facies and hypertension.

Adrenocortical tumors are rare malignant tumorbut prognosis depends on the stage of disease.

Pheochromocytoma

These are catecholamine secreting tumors presenting with hypertension, excess sweating, palpitation and weight loss. The diagnosis is confirmed by raised catecholamine levels in urine and blood. The surgical removal is curative but requires preoperative stabilisation to avoid hypertensive crisis during intraoperative period.

Surgical Management

After detailed initial evaluation and stabilisation the tumors are planned for surgical excision.Traditionally these lesions were managed by open surgical excision. With the advancement in minimal access  surgery these tumors can be surgically removed by laparoscopic or robotic surgery. This approach ensures faster recovery and reduces hospital stay because of small incion small incisions. The approach provides surgeon magnified view which ensures fine dissection and minimises blood loss. Although the approach may not be suitable for large and malignant lesions which will require open surgery.

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