[Cushing's syndrome due to bilateral oncocytic adrenal tumor].

Cushing’s syndrome accounts for approximately 20–30% of endogenous hypercortisolism cases, and adrenal involvement can be either unilateral or bilateral. Cushing’s syndrome due to bilateral adrenal tumors is extremely rare. Adrenal oncocytomas are another rare cause of endogenous hypercortisolism: about 13 cases are described in the literature. Oncocytomas are rare epithelial neoplasms, characterized by abnormally excessive accumulation of defective mitochondria in the cytoplasm of cells, and make up 1.8% of all adrenal neoplasms. We describe a 58-year old patient with Cushing’s syndrome and bilateral adrenal tumors. Multispiral computed tomography of the adrenals showed signs suspicious of lipid-poor atypical adenomas or malignant tumors. Surgical treatment was the method of choice, and the larger tumor was excised first. Due to the absence of remission of endogenous hypercortisolism the excision of the second tumor was performed. Morphological and immunohistochemical examination confirmed the diagnosis of bilateral oncocytic adrenocortical tumors with uncertain malignant potential. Cases of bilateral hormone-producing adrenal oncocytomas have not been described in the literature.

A Case of Pregnancy Complicated by Primary Hyperparathyroidism Due to a Parathyroid Adenoma.

BACKGROUND Primary hyperparathyroidism is most common in women during the menopause and its occurrence in pregnant women is rare. However, because neonatal mortality is associated with maternal hyperparathyroidism, early diagnosis is essential. This report describes the case of a late diagnosis of primary hyperparathyroidism in a 28-year-old pregnant woman and describes the effects on the mother and neonate. CASE REPORT During her second pregnancy, a 28-year-old woman presented with symptoms of general weakness, bone and joint pain, multiple fractures with bone deformity, muscle weakness, and gait disturbance. Due to the high risk of perinatal pathology, a cesarean section was performed. Several weeks later, she underwent thoracoscopic removal of an ectopic parathyroid gland located at the aortic arch. Hypocalcemia in the newborn infant required treatment with calcium and magnesium supplements. CONCLUSIONS This case demonstrates that primary hyperparathyroidism during pregnancy requires timely diagnosis and treatment to reduce potential maternal and fetal complications. Screening for primary hyperparathyroidism should be undertaken in pregnant women with any symptoms associated with hypercalcemia. Treatment should be individualized and includes conservative management, parathyroidectomy in the second trimester, or parathyroidectomy performed in the early postpartum period.

Pheochromocytoma surgery: epidemiologic peculiarities in children.

Manifestations of pheochromocytoma have some specific features in children. The aim of this study was to explore epidemiologic differences of the disease course in children and adults, the principal causes of pheochromocytoma recurrence, and the optimal extent of an operative intervention in a group of patients with initial manifestation of their tumor during childhood. A total of 520 patients with pheochromocytoma underwent surgery from 1957 to 2001. The mean age of the patients was 39.3 +/- 9.2 years; 50 patients (9.6%) were 16 years or under (children's group). There were 213 males (41%) in the general group and 32 males (62%) in the children's group. Bilateral adrenal lesions were present in 68 patients (13.1%), including 16 of the 50 children (32%). The tumors were extraadrenal in 36 patients (6.9%), including 9 of the 50 children (18%). The combination of pheochromocytoma and a hereditary syndrome was present in 36 cases (6.9%). Follow-up was obtained in 260 patients, including 46 children. Length of follow-up varied from 4 to 25 years (average 8.4 +/- 1.9 years). The pheochromocytoma recurred in 49 patients (18.8% of surveyed patients), with a true recurrence (a tumor in the region of the primary operation or metastases) in 16 patients (6.15%). A true pheochromocytoma recurrence was noted in 6 of the 50 children (12.0%). Organ-sparing tactics for multicentric adrenal lesions was a principal cause of the true recurrence. Therefore we believe it is necessary to perform a radical operation, which includes adrenalectomy and removal of the tumor, in patients with a high risk of recurrence.