ACTA ENDOCRINOLOGICA (BUC)

Background. Langerhans cell histiocytosis (LCH) is a rare group of neoplastic diseases resulting from Langerhans dendritic cells. The most common site (80%) is bones. Thyroid gland involvement is exceedingly rare and usually expected to be seen as a part of multisystemic disease. Case Report. We present a 45 year old male patient operated due to multinodular goiter and neck pain, and diagnosed with LCH in his postoperative pathologic examination. As a result of the systemic screening performed after the pathological diagnosis, the disease was interestingly localized to the thyroid gland. Systemic involvement did not develop in the two-year follow-up of the patient who did not receive additional chemotherapy treatment. Conclusion. It is difficult to diagnose LCH in the thyroid gland before surgery. Although surgical treatment with or without chemotherapy is recommended, surgery is not recommended alone since it is generally systemic involvement. However, in primary thyroid LCH cases limited to the thyroid gland, we recommend that only total thyroidectomy treatment should be kept in mind.

Context. Hyponatremia is a common electrolyte abnormality. Objective. We report a patient who presented with hyponatremia and diagnosed as small cell lung cancer metastatic to hypothalamus and pituitary. Case report. A 68 year old male patient was admitted with fever and cough and pneumonia was considered. Serum sodium level was 113 mmol/L. Syndrome of in appropriate ADH (SIADH) is considered. Thyroid function tests and cortisol levels pointed out a central deficiency in both axes. Pituitary MRI was performed and a hypothalamic and pituitary mass were observed. Prednisolone therapy was started followed by L thyroxine replacement. A chest computer tomography (CT) was taken 2 weeks later revealed a mass lesion. Bronchoscopic biopsy was performed and histopathological diagnosis of the tumor was reported as small cell lung cancer. Result. Many mechanisms were considered as the cause of hyponatremia in our patient. SIADH, secondary adrenal insufficiency and secondary hypothyroidism due to pituitary metastasis are possible causes. Conclusion. The reason of hyponatremia is sometimes complex. When the underlying causes of hyponatremia are not evaluated in detail, many diagnoses can be missed.

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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