ACTA ENDOCRINOLOGICA (BUC)

Non-functioning pituitary tumors (NFPA) are usually large at the time of diagnosis and present with symptoms caused by compression on the neighbouring structures. Transsphenoidal resection of the tumor is the firstline treatment but in many cases complete tumor resection cannot be attained. Close follow-up with repeated hormonal assessment and pituitary imaging is recommended after surgery. During follow-up of the tumor remnant sometimes radiotherapy is used to stop or prevent tumor progression. Medical treatment with dopamine agonists (DA) is the mainstay in the management of prolactinomas. For NFPA several small series or case reports suggested beneficial effects with tumor shrinkage in some cases. The aim of the current paper is to summarize the available evidence related to the potential efficacy of DA medical treatment in NFPA while underlining that in the absence of randomized, placebocontrolled studies, no final conclusion on the efficacy of these drugs in the treatment of NFPA can be drawn.

Context. Pheochromocytomas are very rare but important and potentially life-threatening sources of ectopic ACTH secretion (EAS), and the diagnosis of Cushing’s syndrome due to adrenocorticotropic hormone (ACTH)- producing pheochromocytoma needs a high index of suspicion. Case presentation. Herein, we present a rare case of catastrophic Cushing ‘s syndrome due ACTH-producing pheochromocytoma in a 59-year-old woman, which was characterized by severe hypercortisolism, markedly elevated ACTH levels and rapidly progressed and persisting metabolic derangements, and complete resolution of symptoms and signs after adrenalectomy, despite no biochemical evidence of pheochromocytoma and the coexisting adrenal cortical adenoma. The timely recognition of findings sufficient to raise the suspicion of an ACTH-producing pheochromocytoma is crucial to plan surgical resection of the adrenal mass which is the only curative option enabling quick recovery with complete amelioration of symptoms and signs and restoration of organ functions. Conclusions. In this regard, our case highlights the likelihood of severe hypercortisolism even in the absence of typical Cushingoid features, and the consideration of suspected diagnosis of ACTH-releasing pheochromocytoma even in the absence of biochemical evidence on catecholamine hypersecretion when workup is suggestive of an ectopic source along with an adrenal mass on imaging.

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Context. Recent studies have demonstrated a strong association between cardiorespiratory fitness (CRF) and mortality, but bias due to differences in the distribution of baseline variables has not been adequately considered. We studied a cohort of veterans with and without Type-2 diabetes using a propensity score matching method. Methods. Males with (n=592) and without (n= 6,167) Type-2 diabetes were studied. Propensity scores were used to balance covariate distributions between groups with and without Type-2 diabetes. All-cause mortality was the end point. Results. Predictors of mortality included hypertension, smoking, Type-2 diabetes, BMI and CRF. For each 1 MET increase in CRF in the unmatched group, the adjusted HR was 0.83 in those with diabetes (95% CI 0.77- 0.89; p<0.0001) compared to 0.87 in those without diabetes (95% CI 0.86-0.89; p<0.0001). Similar trends were observed for the matched dataset: the adjusted HRs were 0.83 (95% CI 0.77-0.90; p<0.0001) and 0.88 (95% CI 0.82-0.94; p<0.0001) for those with and without diabetes, respectively. Conclusions. CRF is a strong predictor of mortality in veterans with and without Type-2 diabetes. Although the trend in the association between CRF and all-cause-mortality was similar for matched and unmatched data, the mortality risks were relatively inflated when using unmatched data.

Familial hypercholesterolemia (FH) is a genetic disease with autosomal dominant transmission, characterised by high blood cholesterol levels. The evolution of this disease leads to primary atherosclerosis and cardiovascular disease. Patients with HF develop atherosclerosis by the age of 20 and usually do not survive past the age of 30. We present the case and oro-dental aspects of a preschooler that was diagnosed at the age of 4 with FH, compound heterozygote (mutation/genotype1 LDLR: C20IX, exon 4; mutation/ genotype2 LDLR: G571E, exon 12) and the experience of our clinic in the management of this patient that received offlabel treatment with statins. When diagnosed, his cholesterol level was 932 mg/dL and his LDL-cholesterol level was 792 mg/dL. Treatment with rosuvastatin and ezetimibe was prescribed. Both substances (rosuvastatin and ezetimibe) are not approved for children under the age of 6 in Europe. Taking into considerations the diagnosis and prognosis for unfavorable evolution, treatment with statins was started at the age of 5 years.

Context. Immunoassays are vulnerable to occasional analytical errors despite their sensitivity and specificity. Adrenocorticotropic hormone (ACTH) is among hormones which are vulnerable to assay interference. Objective. In this case report, we aimed to present a case of ACTH interference in a patient with subclinical hypercortisolism. Case report. We present a case of subclinical hypercortisolism with unexpectedly elevated levels of ACTH in whom interference was shown by different biochemical methods. It is important for the clinician to keep in mind the possibility of interference when clinical and laboratory results are discordant. The communication between the clinician and laboratory staff is important while interpreting results. This case report shows that ACTH interference should be considered in patients with subclinical hypercortisolism who have unexpectedly high levels of adrenocorticotropic hormone.

Background. An adrenal collision tumor is a rare entity. We present a rare combination of giant adrenal ganglioneuroma (GN) and myelolipoma. GN is a rare benign tumor of the adrenal medulla that originates from primitive neural crest cells, while myelolipoma is a benign tumor of the adrenal cortex comprising of mature adipose tissue and blood components. Case Report. We present a case of a 52-year-old male who presented with generalized body swelling with episodes of vomiting and diarrhea. There was no history of abdominal pain or any significant history. Routine laboratory investigations and endocrine workup were within normal limits. MRI was performed for unexplained symptoms, and which revealed a solid homogeneous mass measuring 9x7x4.5cm arising from the adrenal gland. A diagnosis of myxoid adrenocortical neoplasm was suggested, and laparoscopic left adrenalectomy was performed based on imaging findings. The final diagnosis of coexisting giant adrenal GN with myelolipoma was made on histopathological examination, which was further confirmed by immunohistochemistry. Conclusion. Ganglioneuroma coexistence with myelolipoma is a rare finding in the adrenal gland. Therefore, histopathology is imperative in such cases for a definitive diagnosis.