ACTA ENDOCRINOLOGICA (BUC)

Neuroendocrine carcinoma (NEC) of the larynx is the most frequent neuroendocrine neoplasm of the head and neck and the most common nonsquamous carcinoma of the larynx. It usually occurs in the supraglottic area, in smoking men. We report a case of a 58-year-old woman with no history of smoking who presented with an atypical carcinoid, arising in the right piriform sinus of the larynx. During the 5-year follow-up, the patient developed metastases in the lymph nodes, palatine tonsils, parotid glands, breasts and skin. For this reason the patient underwent several surgical procedures, radiotherapy and eventually was qualified for chemotherapy. Our case shows that NEC of the larynx can have an atypical presentation. The diagnosis requires careful pathological evaluation with immunohistochemistry and a wide spectrum of imaging. The serum concentration of chromogranin A seems to be not useful in the diagnosis and follow-up of laryngeal NEC.

Resistance to thyroid hormone (RTH) is an inherited disease transmitted in an autosomal dominant manner. The diagnosis is suspected when peripheral thyroid hormones are increased contrasting with normal or increased levels of thyroid stimulating hormone. Usually, people harboring the rare syndrome have few or no symptoms. However, in some patients signs of hyperthyroidism may be the revealing anomalies as in the following case: A 75 year-old woman was referred to our department for a benign adrenal incidentaloma. In her medical history she was treated for systemic hypertension and diabetes mellitus for 15 years. Clinical examination did not show any sign of adrenal secretion, but discovered rapid irregular cardiac rhythm with some hyperthyroidism features such as increased sweating and upper limbs and jaw tremor. Electrocardiogram showed atrial fibrillation. Hormonal assessment confirmed hyperthyroidism as FT4 levels were high (mean value: 30.2pmol/L (n= 9–23)), contrasting with non-suppressed TSH levels (13.8μU/mL (n = 0.2 – 4)). Cerebral magnetic resonance imaging was normal. Genetic testing revealed a new heterozygous mutation on exon 10 in the THRβ gene (c.1366T>G) compatible with RTH syndrome. Screening of her children showed the same hormonal profile in five out of ten. These results confirmed RTH and the familial character.

Background. McCune-Albright syndrome (MAS) is a sporadic disease characterized by polyostotic fibrous dysplasia (FD), café au lait skin lesions and a variety of endocrine or non-endocrine dysfunctions. Case presentation. We present the case of a 33 years old woman diagnosed with MAS in 2011. She presented precocious puberty at the age of five, multiple long bone fractures, but no café-au-lait spots were identified. The thyroid investigation tests revealed a toxic multinodular goiter. The thyroid scintigraphy showed an increased uptake in the superior third of the right lobe. The X-rays and bone scintigraphy revealed polyostotic FD. The patient was previously diagnosed with transmission hypoacusia, explicable in the setting of bony auditory canal deformity. The assessment of phospho-calcium balance showed a hyperphosphaturic hypophosphatemia. The liver enzymes levels were also increased, especially GGT. Conclusion. The sporadic mosaic nature of the disease means that it is an uncommon disease with a variable expression. Each case is unique, and the approach that was appropriate in one of the cases is likely not to be the best in another.

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As men are aging, their testosterone levels decline, while the morbidity of atherosclerosis (AS) rises in accordance\r\nwith their age. Recent studies indicate that inflammation contributes to atherosclerosis. Whether hypogonadism in old men is associated with inflammation deserves to be\r\ninvestigated.\r\nBackground. To examine the relationship between free testosterone (FT) and inflammatory cytokines such as\r\npregnancy-associated plasma protein A (PAPP-A), interleukin-6 (IL-6), vascular cell adhesion molecule-1 (VCAM-1), intracellular adhesion factor-1 (ICAM-1) in old men with\r\nacute coronary syndrome (ACS).\r\nMethods. Serum was collected from 107 men aged 60-86 years. Among them, 52 were patients with ACS. The relationships\r\nbetween FT and PAPP-A, IL-6, VCAM-1 and ICAM-1 were examined respectively.\r\nResults. Levels of weight, body mass index, total cholesterol, low density lipoprotein cholesterol, triglyceride, PAPPA, IL-6, VCAM-1 and ICAM-1 of old male\r\nACS patients were higher than those of controls; FT and high density lipoprotein cholesterol levels were lower than those of controls. FT level was inversely associated with PAPP-A, IL-6, VCAM-1 and ICAM-1.\r\nConclusions. FT level is inversely associated with inflammatory cytokines such as PAPP-A, IL-6, VCAM-1 and\r\nICAM-1 in old men with ACS. This might imply that low testosterone is associated with inflammation in old men with ACS.

Increase in obesity pandemic all over the world consequently leads to the investigation of possible causes. In addition to the traditional explanation using the so-called caloric model, the field of endocrine disruptors (EDs), especially subgroup called obesogens, offered more light on the pathogenetic mechanisms involved. After the Second World War a correlation between an increased production of exogenous pollutants and actual obesity epidemic was suggested. “Obesogen hypothesis” implies that molecules called obesogens inadequately stimulate the development of adipose cells and lipid accumulation in existing adipose cells, as well as change metabolic balance or hormonal control of appetite and satiety, leading to an increase in body fat mass. The list of obesogens includes some industrial chemicals, biocides, pharmaceuticals, pollutants, and smoke. EDs from the group of obesogens may exert their effects by the impairment in the programming development of adipocytes, by an increase in energetic depot in the adipose tissue, and by influencing neuroendocrine control of appetite and satiety. Increased scientific evidence on obesogens and their mechanisms of action may help to prevent obesity and mitigate deleterious effects of the environment on human life and development. New translational studies are needed to explain the possible mechanism proposed.

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Hormonal therapy: estrogen therapy (ET) and estrogen-progestogen therapy (EPT), in postmenopausal women has to be individualized, taking into account benefits and potential risks, as part of a global strategy of health management. The randomized controlled clinical trials of the past decade have changed the general thinking in the field, leading to the mostly accepted theory of initiating hormone therapy in symptomatic women, near the menopause, in the so-called “window of opportunity”, when estrogens may be cardioprotective because the vascular endothelium is still intact, and may even be beneficial for some cognitive domains. The second point suggested is the use of lower doses of hormone therapy for the shortest period of time, necessarily to solve the problem. The main indications for initiating hormone therapy are: moderate to severe vasomotor symptoms, genitourinary complaints, and prevention of postmenopausal osteoporosis. In all women with an intact uterus who are using estrogen therapy, an adequate progestogen has to be added in a continuous or sequential regimen, in order to prevent endometrium carcinoma. The use of hormonal menopausal therapy for about five years does not seem to be associated with significant risks, such as breast cancer, cardiovascular risk, if therapy is initiated in the late menopausal transition and early postmenopausal, symptomatic women. Venous thromboembolism and stroke remain the main serious risks of taking hormone therapy. There are no data at present to support the indication of hormone therapy in older women in order to prevent chronic medical conditions, like coronary heart disease or dementia, as secondary prevention.

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