Adrenal cortical tumors in childhood--clinicopathological features of six cases
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L Moore1, A P Barker, R W Byard
1Department of Histopathology, Adelaide Children's Hospital, South Australia.
Insights
Pediatric adrenal cortical tumors can present with hormonal imbalances or as masses. Surgical resection is effective, even with unusual histology, highlighting challenges in diagnosing malignancy in these childhood tumors.
Area of Science:
- Pediatric Oncology
- Endocrinology
- Surgical Pathology
Background:
- Adrenal cortical tumors are rare in children.
- These tumors can cause significant hormonal disturbances.
Purpose of the Study:
- To present clinical and histological findings of pediatric adrenal cortical tumors.
- To evaluate the efficacy of surgical resection for these tumors.
Main Methods:
- Case series of six pediatric patients with adrenal cortical tumors.
- Surgical resection as primary treatment.
- Perioperative hydrocortisone for functional tumors.
Main Results:
- Five of six children presented with hyperadrenalism (virilization or Cushingoid features).
- One child had an asymptomatic abdominal mass.
- Histological features like bizarre morphology and high mitotic counts were observed in benign cases.
- Five patients survived with no evidence of recurrence after surgery.
Conclusions:
- Surgical resection is an effective treatment for pediatric adrenal cortical tumors.
- Histological criteria for malignancy can be challenging in pediatric adrenal tumors.
- Complete resection is crucial for favorable outcomes.
Abstract:
Six cases of adrenal cortical tumors are presented with a discussion of the clinical features and histological findings. Five of the 6 children, aged between 6 mths and 6 yrs, presented with symptoms of hyperadrenalism, 4 with virilization and 1 with Cushingoid features. The remaining infant presented with an asymptomatic abdominal mass. In each case there was a unilateral tumor separated from the residual adrenal gland by a thin fibrous capsule. Surgical resection was the treatment employed and, in the 5 cases with functional tumors, perioperative hydrocortisone was given. None of the children received post-operative chemotherapy or radiotherapy. Bizarre cellular morphology, a high mitotic count and extensive necrosis were all seen in clinically benign disease, demonstrating not only the efficacy of local resection but also the difficulty in applying the usual histological criteria of malignancy to these pediatric adrenal tumors. At follow-up, 5 of the 6 patients are alive with no evidence of recurrent disease. The only death resulted from measles pneumonitis in the 1 child who presented with Cushing's syndrome.