FUNCTIONING ADRENAL TUMORS IN CHILDREN: A REPORT OF 17 CASES
Nabhan MT Kaddah, Nabil M
Dessouky
Pediatric Surgical
Division, the New Children��s Hospital, Cairo University
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.
Follow up 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
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.