This study aimed to evaluate the various clinical syndromes of these lesions, the different methods for their diagnosis, their pathologic findings, the different surgical approaches for their excision, and their clinical outcomes in correlation to their pathological features.

Methods

         All patients with functioning adrenal tumors presenting to the Pediatric Surgical Division, Cairo University over a period of 24 years, between 1986 and 2010 were clinically analyzed. Full radiological studies and endocrinologic workups were performed.  All patients were treated by surgery using different surgical approaches. The tumor's size and weight were recorded and its histological grading was performed according to Weiss criteria. Follow- up of the patients was achieved through clinical, radiological and hormonal assessments.

Results

    The study included 29 patients - 19 females and 10 males - with their ages ranging between 1.8 and 16 years (mean = 6.2 years). Two sisters were affected with left-sided virilizing adrenocortical carcinoma. Twelve patients presented with features of virilism, 6 cases had signs of Cushing’s syndrome, one girl with feminization, 7 cases with sustained hypertension, 2 cases with diarrhea and one case with seizures. Palpable abdominal masses were present in 7 patients: four with signs of virilization and three with Cushing’s syndrome. Both sides of the adrenal gland were equally involved in 11 cases each, with bilateral lesions in one case of pheochromocytoma. Ten lesions were less than 5 cm in diameter, one of them was malignant, while 12 tumors were less than 100 gm in weight, and two of them were malignant. Medullary lesions included 6 cases of benign pheochromocytoma, 3 cases with neuroblastoma and a case of ganglioneuroblastoma. Among 19 patients with adrenocortical tumors, 7 had four or less positive Weiss histological criteria, all of them had favorable outcome while two of the other 10 cases remained disease-free with a follow-up which ranged between 3 months and 7 yearsThree cases developed local recurrence after surgery and 5 patients died postoperatively after partial response to chemotherapy, 4 of them had distant metastases.

Conclusion:

        Virilization is the commonest presentation, which is commonly due to carcinoma in females or adenoma in males. On the contrary, Cushing s syndrome is commonly due to carcinoma in males and adenoma in females. Palpable abdominal mass was rather uncommon and considered a late manifestation with poor prognosis. CT scan remains the standard accurate modality for their localization and during follow up. Complete removal of the tumor is the only effective treatment, the role of adjuvant chemotherapy or radiotherapy cannot be confirmed. Laparoscopic adrenalectomy needs to be tried and evaluated in small lesions. The determination of the neoplasm’s behavior by morphologic and histological criteria can often be unpredictable, even small lesions can be malignant and potentially lethal. Careful clinical follow-up may remain the final indicator for the diagnosis in some of these tumors. A more advanced study to analyze the familial occurrence of adrenal tumors in children is needed and the possible role of exposure to toxic substances in its occurrence.