FUNCTIONING ADRENAL TUMORS IN CHILDREN: A REPORT OF 17 CASES | ||||
| The Egyptian Journal of Surgery | ||||
| Volume 20, Issue 2, April 2001, Page 485-498 PDF (1.23 MB) | ||||
| Document Type: Original Article | ||||
| DOI: 10.21608/ejsur.2001.376368 | ||||
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| Authors | ||||
| Nabhan MT Kaddah* ; Nabil M Dessouky | ||||
| From the Pediatric Surgical Division, the New Children’s Hospital, Cairo University | ||||
| Abstract | ||||
| Background / Purpose:Tumors of the adrenal gland are extremely rare in children, those of the adrenal cortex account only for 0.3-0.4 % of all childhood neoplasms. 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 over a period of 14 years, between 1986 and 2000 were clinically analyzed. Radiological studies primarily included bone age determination, IV urography, ultrasonography and scintigraphy scan .Magnetic resonance imaging was performed for only one patient. Hormonal evaluation included urinary levels of 17-ketosteroids and 17-hydroxycorticoids together with serum levels of testosterone and its precursors and cortisol. Dexamethasone suppression test was performed for all patients with suspected adrenocortical lesions. Estradiol, FSH and LH levels were done in a female presenting with isosexual precocious puberty. Estimation of catecholamines was performed for three patients with sustained hypertension. 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. Followup of the patients was achieved through clinical, radiological and hormonal assessments. Results: The study included seventeen patients 9 females and 8 males with their ages ranging between 1.8 and 9 years (mean = 4.9 years). Two sisters were affected with left-sided virilizing adrenocortical carcinoma. Ten patients presented with features of virilism, three cases had signs of Cushing’s syndrome, one girl with feminization and three cases with sustained hypertension. Palpable abdominal masses were present in four patients: three with signs of virilization and one with Cushing’s syndrome. The left adrenal gland was involved in ten cases, the right in seven with no bilateral lesions. Fourteen lesions were operated upon through an anterior transabdominal approach; in two cases the tumor was removed through a posterior incision and in one case a small lesion was excised through a lumbar approach. Seven lesions were less than 5 cm in diameter, one of them was malignant, while 10 tumors were less than 100 gm in weight, two of them were malignant. Medullary lesions included two cases of benign pheochromocytoma and a case of ganglioneuroblastoma. Among 14 patients with adrenocortical tumors, seven had four or less positive Weiss histological criteria ,all of them had favorable outcome while two of the other seven cases remained disease-free with a follow-up which ranged between 6 months and 7 years. One case developed local recurrence after surgery and four patients died postoperatively after partial response to chemotherapy, three of them had distant metastases. Conclusion: Functional adrenal tumors are extremely rare in children specially pheochromocytoma. 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 is 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 can not be confirmed. The anterior transabdominal approach provides the best exposure especially for large tumors, although very small lesions can be removed safely through a lumbar extra-abdominal approach. 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. Early diagnosis 486 Egyptian Journal of Surgery and management are important with special care for postoperative adequate steroid replacement. A more advanced study to analyze the familial occurrence of adrenal tumors in children is needed for the possible role of exposure to toxic substances in its occurrence. | ||||
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