Abstract
Objective: Childhood adrenocortical tumors (ACTs) are rare neoplasms, about which etiopathogenesis and disease management are not yet clearly understood. We aimed to review the management of ACTs in our single medical center. Methods: We retrospectively reviewed findings in seven children, who were 15 years old or younger, and were diagnosed with ACTs in our institution over the past 10 years. Information recorded for each patient included age, sex, presenting symptoms, hormonal status, pathological findings, stage of disease, treatment and outcome. Results: Four girls and three boys were treated for ACTs. Five had adrenocortical carcinoma (ACC) and two had adrenocortical adenoma (ACA). All patients underwent laparotomy and complete excision. Two of ACCs had a stage I disease, one had a stage III, and the latter two ACCs had a stage IV disease. ACAs were treated successfully by total excision without any concomitant therapy. Adjuvant chemotherapy and mitotane were commenced postoperatively in ACC patients. Six patients are alive and doing well at a mean follow-up of 5 years and 8 months. Conclusion: The small number of ACTs and the short follow-up period limit the assessments of prognosis and management. As a result, patients with ACTs should be studied in multi-institutional trials to address the role of prognostic factors and cytotoxic drugs in the disease management.
Highlights
There were seven children treated for adrenocortical tumors (ACTs) in the study period
Clinical characteristics and laboratory findings of these patients are shown in Tables 1 and 2
It has been indicated in stage III and IV adrenocortical carcinoma (ACC) in association with cisplatin, etoposide, and doxorubicin (CED) as it was previously recommended for adult patients
Summary
The records of seven children treated for ACTs in our unit between 2003 and 2013 were reviewed retrospectively. Information recorded for each patient included age, sex, presenting symptoms, hormonal status, pathological findings, stage of disease, treatment and outcome. The extent of disease was defined as localized or advanced on the basis of a modified staging system proposed by Sandrini et al [7]. Assignment to the localized disease (stage I: tumor weight ≤ 200 gram or stage II: tumor weight > 200 gram) category required complete tumor resection and no evidence of disease elsewhere. Assignment to the advanced disease (stage III or IV) category required the presence of local residual or metastatic disease. When tumor weight was not provided, it was estimated from tumor measurements obtained from computed tomography scans, ultrasound examinations, or pathology reports by using the following formula: weight = 1.342x(volume)0.8563 [8]
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