Empty sella developing during thyroxine therapy in a patient with primary hypothyroidism and hyperprolactinaemia.

A 35 year old woman presented with severe primary hypothyroidism and galactorrhea. A very high prolactin level was also detected and computerized tomography scan of the sellar region demonstrated an enlarged pituitary gland associated with contrast enhancement. Replacement therapy with thyroxine corrected both biochemical and clinical abnormalities but empty sella developed during this therapy. It is concluded that empty sella may be related to thyroxine-induced shrinkage of lactotroph and/or thyrotroph cell hyperplasia.     

The association of cystic nephroma with pulmonary sequestration: is it a coincidence or not?

Cystic nephroma (CN) is a rare, presumably benign, multilocular cystic renal tumor. Pulmonary sequestration (PS) also presents as cystic masses of non-functioning primitive lung tissue. We describe a 15-month-old girl with CN and PS. Although some rare associations of renal and pulmonary lesions have been reported, this is the first case report in the English literature that shows the association of CN with PS.     

A new autogenous graft choice in pelvic reconstructions: free vascularized rib (a case report)

Primary or secondary bone tumours are not uncommon in pelvic girdle. In some cases, after radical resection, there is a big bony defect where the prosthesis is not applicable; arthrodesis is the only choice for good functional results. In this instance, the major problem is to achieve the fusion. In this case report, we focused on easy harvesting and minimal time consumption with free vascularized rib graft to achieve the fusion between the resected segments. Two year follow up showed fusion with good functional result.     

Evaluation of the Cardiovascular Risk in Patients with Subclinical Cushing Syndrome Before and After Surgery

Background The widespread use of ultrasound, computerized tomography, and magnetic resonance imaging has led to an increase in the number of incidental adrenal masses identified. Asymptomatic incidentally discovered adrenal masses may indicate that subclinical Cushing syndrome (SCS) is not uncommon. We aimed to evaluate the cardiovascular risk of patients with SCS before and after surgery. Methods An autonomous cortisol-producing tumor was detected in 11 of 94 patients with adrenal incidentaloma between 1995 and 2005. Twenty-eight patients suffering from classical Cushing syndrome (CS) associated with unilateral adrenocortical adenoma, who were treated at our department in the same period, served as a control group. Cardiovascular risk factors such as blood pressure, body mass index, and lipid profile were evaluated before and 1 year after surgery. Results The frequency of hypertension (61% versus 63%), obesity (46% versus 55%), diabetes mellitus (50% versus 36%), hypercholesterolemia (39% versus 36%), and low HDL cholesterol (28% versus 36%) were not significantly different between CS and SCS patients, respectively. Adverse cardiovascular risk profile improved 1 year after adrenalectomy in both groups, although the changes were not significant with respect to body mass index, frequency of diabetes, and hyperlipidemia in SCS patients. But frequency of systolic/diastolic hypertension decreased significantly in this group. Conclusions These findings indicate that the increased incidence of cardiovascular risk factors commonly observed in classical CS, is also present in SCS. Unilateral adrenalectomy does not always lead to significant improvements in cardiovascular risk profile in SCS.     

Metaphyseal enchondrodysplasia with 2-hydroxy-glutaric aciduria: observation of a third case and further delineation

In the course of evaluating a 17 months old boy with waddling gait and swollen joints, we found generalized, severe ossification defects in the metaphyses of his long bones. The differential diagnosis included nutritional or genetic rickets, metaphyseal dysplasia, and enchondrodysplasia. Calcium, phosphate and alkaline phosphatase were normal, while targeted analysis of urinary organic acids repeatedly revealed excretion of 2-hydroxy-glutaric acid. Thus, this child appears to have an unusual combination of findings described in just two other patients so far, a girl and a boy, and called 'spondyloenchondrodysplasia with D-2-hydroxy-glutaric aciduria'. These three cases are similar in terms of severe metaphyseal lesions, mild vertebral involvement, and presence of 2-hydroxy-glutaric acid in the urine. We consider this a radiographically and biochemically distinct entity, for which we suggest the name of 'metaphyseal enchondrodysplasia with 2-hydroxy-glutaric aciduria'.     

Thyroid metastasis of malignant melanoma

Clinically significant metastases to the thyroid gland are very rare; however, they can present as the initial malignancy. The authors report a 53-year-old man who underwent surgery for malignant melanoma 5 years earlier, and recently presented with a thyroid nodule that turned out to be a metastasis of the primary malignancy. Fine-needle aspiration showed malignant cells, but was unable to indicate that their origin was malignant melanoma. Total thyroidectomy was the therapy chosen for the patient.