Ectopic ACTH syndrome associated with ovarian steroid-cell tumor.

Several cases of ovarian neoplasms resulting in Cushing's syndrome due to ectopic secretion of ACTH or ectopic secretion of F have been reported. Tumors producing ACTH include adenocarcinoma, androblastoma, Sertoli cell carcinoma, carcinoid tumor and teratoma. Cortisol secretion has been reported in ovarian steroid cell tumor (unclassified steroid cell tumor). We present a case of a 19-year-old woman with Cushing's syndrome in course of an ovarian steroid cell tumor with ectopic ACTH production. To our knowledge, it is the first reported case of ACTH secreting ovarian steroid cell tumor causing Cushing's syndrome.

Clinical and histopathological evaluation of the adrenal incidentaloma.

Clinically silent adrenal masses (incidentaloma) are incidentally discovered lesions, when noninvasive imaging methods (USG, CT, MRI) are performed for reasons other than known or suspected adrenal disease. Most studies report on a prevalence of adrenal incidentaloma range between 1% and 10% in radiological series. Between 1994 and 1999 we observed in our Department 57 patients with incidentalomas of adrenal glands. After endocrinological evaluation silent Cushing's syndrome was found in 2 cases (3.5%). Fifty two patients were qualified for surgery. Adrenocortical adenoma was diagnosed in 73.1%; adrenocortical carcinoma in 7.7%; pheochromocytoma in 7.7% and less frequent adrenal lesions in 11.5%. All adrenal carcinomas and malignant pheochromocytomas (11.5%) were found in tumors with diameter over 4 cm.

[Diagnostic difficulties in primary hyperparathyroidism based on personal observations]. / Trudnosci diagnostyczne pierwotnej nadczynnosci przytarczyc na podstawie obserwacji wlasnych.

The primary hyperparathyroidism (PHP) is a complex of nosological symptoms associated with disturbances of calcium-phosphate equilibrium and bone metabolism, caused by excessive secretion of parathormone. In the past regarded as a rare entity, today it is arousing great interest due to ever more frequent diagnosis of it in the stage of subtle changes or in the asymptomatic period. Untreated PHP leads to the development of a number of organ complications, and even to a life-threatening state, that is hypercalcemic crisis. The authors discussed differential diagnostic and localisation difficulties in PHP on example of own observations.

[Complete heart block treated with electrotherapy in a 30-year-old pregnant women with Kearns-Sayre syndrome]. / Blok calkowity serca wymagajacy elektroterapii u 30-letniej ciezarnej z zespolem Kearns-Sayre.

Kearns-Sayre syndrome (KSS) is a form of mitochondrial myopathy in which specific clinical features, namely progressive external ophthalmoplegia, pigmentary retinal degeneration and onset before age 20 occur. It can also be associated with cardiac conduction defects, neurological and variety of endocrine and metabolic disorders. Recognition of mtDNA deletion as the genetic basis of KSS has confirmed the validity of clinical criteria. The purpose of the report is to describe a 30-year-old woman presenting typical clinical features of KSS. On muscle biopsy ragged red fibres and mitochondrial abnormalities on electron microscopy were seen. In spite of menstrual disturbances she became pregnant and delivered health child. In 30 week of pregnancy the VVI pacemaker was implanted because of syncope most probably related to paroxysmal complete heart block.