Adrenocortical carcinoma in children and adults: Two decades experience in a single institution.

CONTEXT: Adrenocortical carcinoma (ACC) occurring in children and adults show distinct characteristics. However, due to rarity of the disease no large series addressing this issue has been published. AIMS: The aim of this study was to study clinico‑pathologic profile and outcome of ACC in children and adults. SETTINGS AND DESIGN: Tertiary referral center. Retrospective study (January 1990‑June 2011). SUBJECTS AND METHODS: Forty‑five patients with ACC were included; 16children (aged < 18 years) and 29 adults. Clinical details, hormonal profile, operation records, pathology reports and follow‑up findings were noted and compared. Survival analysis was performed using Kaplan‑Meier method. Log rank test and Cox regressionan alysis were performed. RESULTS: Mean age was 8 ± 5.7 (M: F = 1:2.1) in children and 44.4 ± 15 years (M: F = 1:1.1) in adult groups. Prevalence of functioning tumors was significantly high in children (87.5 vs. 31% P = 0.001), while prevalence of incidentalomas was high in adults (6.3 vs. 51.7% P = 0.05). Tumor stage distribution at presentation, mean diameter (10.9 vs. 13.7 cm), and weight (392.9 vs. 892.9 g) didn’t differ significantly in two groups. Adults had better albeit non‑significant 5 year overall survival (OS) than children (0 vs. 13%). On univariate analysis stage of disease (P = 0.008), surgical intervention (P = 0.004), Weiss score (P = 0.04) and hormonal secretion (P = 0.04) were significantly associated with OS in adults but not in children. No factor was found significant on multivariate analysis. CONCLUSIONS: Except for high prevalence of functioning tumors in children, clinico‑pathologic attributes and outcome of ACC in the two groups didn’t differ significantly.

Functional parathyroid cystic adenoma: A rare cause of hypercalcemic crisis with primary hyperparathyroidism.

We discuss a case of primary hyperparathyroidism caused by a giant cystic parathyroid adenoma presenting with neck swelling and hypercalcemic crisis. Fine‑needle aspiration cytology of presumed thyroid swelling from one of the two sites aspirated yielded clear fluid but was not attributed to parathyroid pathology. Elevated serum calcium and intact parathormone (iPTH) levels suggested preoperative parathyroid pathology. Ultrasound neck and sestamibi scan for parathyroid localization were not conclusive. Due to resistant hypercalcemia, the patient underwent emergency bilateral neck exploration and excision of the identified left superior parathyroid cyst along with total thyroidectomy. Monitoring of intra‑operative iPTH helped complete removal of hyperfunctioning parathyroid tissue. Histopathological examination confirmed the parathyroid cyst. Cystic parathyroid adenoma should be considered in the differential diagnosis of cystic neck lesions.