ACTA ENDOCRINOLOGICA (BUC)

Xanthomatosis is a rare disease; predominantly, it is a response to altered lipid levels in the form of a mucocutaneous granulomatous proliferative disorder of unclear origin. When blood lipid levels exceed the normal values, the macrophages around the blood vessels may result in xanthoma. The present case was observed in a 55-year-old woman who suffered from Cushing’s disease and had atypical xanthomas in her oral mucosa that were diagnosed by histopathological analysis and were associated with normal serum cholesterol levels.

A 40-year-old woman has been followed up for 19 years by the\r\nendocrinology clinic with the diagnoses of Addison disease and primary hypothyroidism. During the most recent\r\nvisit of the patient, she complained about fatigue and malaise with pretibial edema. In albumin analysis: 2.2 gr/dL and 5.8 g/day proteinuria were detected. In terms of\r\nnephrotic syndrome etiology, renal biopsy was performed and it was considered as minimal change disease. The dose of 7.5\r\nmg/day methyl prednisolone was potentiated to a dose of 1mg/kg/day. During her control, the proteinuria did not regress and 150 mg/day cyclophosphamide was added to the treatment. During the control, her proteinuria regressed from 5.8 r/day to 1.95 mg/day.

Background. Small-cell lung carcinoma is a neuroendocrine tumor that exhibits aggressive behavior, rapid growth, early spread to distant sites and frequent association with paraneoplastic syndromes, the most frequent being the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). Case report. We present the case of a 66 year-old woman who presented to the Emergency Department for a syncope preceded by vomiting. She had onemonth history of progressive confusion, disorientation, drowsiness, and generalized malaise. At presentation, the biochemical profile showed normal hemogram values, severe hyponatremia (seric sodium level 120 mg/dL), low plasma osmolality (<275 mOsm/ kg). The blood tests for kidney, thyroid and adrenal functions were in normal limits. There was no evidence of congestive heart failure or nephrotic syndrome. A diagnosis of SIADH was made. Because of a high suspicion for lung cancer, chest computed tomography (CT) was done, together with neck, abdominal and pelvic CT scan, all showing normal data, excepting necrotic lymph nodes in the lower right carotid space and right paratracheal. Right laterocervical lymph node biopsy was done. The histopathological and immunohistochemical result showed lymph node metastasis of a neuroendocrine small cell carcinoma, most probably with broncho-pulmonary origin. Conclusion. The case is interesting due to long term absence of any respiratory symptom, absence of identification of pulmonary tumor, despite repeated CT scans, the only clinical manifestations being related to SIADH. Also, it emphasizes the importance of early recognition of SIADH, which may be the only initial manifestation of a neuroendocrine small cell carcinoma.

Medullary thyroid carcinoma (MTC) arising from aberrant tissue is almost unknown. Our aim is to report our first case.\r\nObservation. A woman, aged 46, was operated on for multinodular goiter with lateral mass considered as a metastasis. Thyroid surgery was well supported. The surgeon discovered a median thyroid which seemed normal, and a lateral mass totally separated from the thyroid. The pathologist who did very thin slices of the whole thyroid excluded the malignancy, but in the lateral tissue, he discovered a typical MTC with positivity to thyrocalcitonin (TCT), chromogranine A, and thyroid transforming factor1 antibodies. That lateral tissue did not contain any thyroid or lymphoid cells. The tumoral tissue was sent to another\r\npathologist who confirmed the diagnosis. In post operative period TCT was normal (mean value=4.5pg/mL, N<13), but urinary metanephrines were slightly increased (1.32\r\nmg/24h, N=1). Radiological explorations (except MIBG scintigraphy not done for low socioeconomic conditions) failed to show any pheochromocytoma. One year later that one was found by CT scan, and our patient was classified as multiple endocrine neoplasm type 2A. Laparoscopic surgery\r\ndone under thiopental and curare was uneventful, histological exam confirmed the pheochromocytoma. Genetic screening for RET proto-oncogene was negative arguing\r\nfor a sporadic case. Conclusion. MTC located in\r\naccessory thyroid is a scientific curiosity which can be mistaken with a lymph node metastasis. In our case a meticulous exam of the median thyroid and the lack of thyroid and lymph cells in the lateral mass were\r\nagainst the metastasis.

Background. The modifications of blood pressure and lipid metabolism, the decrease of heart contractility and the increase of systemic vascular resistance that accompany hypothyroidism are caused by the decreased action of thyroid hormones. Objective. The purpose of this study is to evaluate, using echocardiography, the changes of the heart functional parameters in patients with untreated thyroid dysfunctions. Pacients and Methods. Study group included 348 patients for an evaluation of cardiovascular and thyroid status. The control of the thyroid condition was conducted using measurements of the FT3, FT4 and TSH levels, echocardiographic measurements defining the parameters of the function of the left ventricle. Results. The study group was divided into subclinical hypothyroidism (SH, 75 cases), overt hypothyroidism (OH, 123 patients) and normal thyroid function (C, 150 patients). TSH values decreased (from 5.8±15 mUI/L to 2.28±0.63 mUI/L in SH and from 20.23±17.33 mUI/L to 4.24±0.24 mUI/L in OH) after one year of treatment while fT4 and fT3 increased. The parameters obtained revealed significant decreases of the E/A ratio in both groups with hypothyroidism. The isovolumetric relaxation time was significantly higher in SH and OH as compared to the control group (C: 112.50±35.45 msec, SH: 118.75±16.52 msec p<0.05, OH: 130±41.83 msec p<0.001). In addition, the tissue Doppler revealed significant decreases of the E’/A’ ratio, measured septally and laterally in the group with overt and subclinical hypothyroidism. Conclusion. In hypothyroidism the systolic and predominantly diastolic function of the left ventricle are impaired. These dysfunctions are reversible under L-T4 substitutive treatment.

We describe a Turkish woman with the association of classical cutaneous features of EDS, extensive cerebral calcification and multiple emphysematous bullae. A 20-year-old woman with a 8-year history of EDS with mild chronic lung disease presented with headache. Physical examination revealed fragility and hyperelasticity of the skin and hypermobility of the joints. A chest computed tomography (CT) showed bilateral paraseptal emphysematous areas and bilateral bullous disease. A cranial CT revealed bilateral extensive calcifications in the cerebral and cerebellar hemispheres and basal ganglia with periventricular and subcortical white matter. Cerebral calcification should be carefully searched for using MRI in patients with EDS and neurological symptoms, such as headache.

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Background. Psychiatric patients with schizophrenia present a higher risk for dyslipidemia than other categories. Aim. To evaluate the values of glucose, triglycerides, cholesterol and total lipids in patients with schizophrenia, who received typical or atypical antipsychotics. Demographic characteristics were also collected. Subjects and methods. This observational, retrospective cohort study included 54 inpatients diagnosed with schizophrenia who were observed during a period of one year. Glucose, triglycerides, cholesterol and total lipids were monitored after 6 and 12 months. We selected patients who were not on hypolipemiant treatment, for various reasons, in order to better evaluate the impact of antipsychotic medication. Results. The majority of patients were male (93% ). The mean age of the patients was of 38, and 87% of them were living in urban areas. Analyzing the mean values of glycemia, cholesterol, triglycerides and total lipids under different types of antipsychotics, higher values of these constants were observed in patients who received olanzapine and risperidone treatment, but with no significant correlations. Conclusion. Patients with schizophrenia naturally shown an increased risk for dyslipidemia, partially due to poor diet and sedentary lifestyle, and these conditions may be exacerbated by antipsychotic medication.

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Objective. This study was undertaken to examine prognostic factors in patients with pancreatic neuroendocrine tumors (PNET) undergoing surgical treatment to evaluate the prognostic value of recently introduced immunohistochemical staining methods of CD10 and cytokeratin 19. Materials and Methods. Tumors were classified on the basis of 2004 WHO Classification Guidelines and European Neuroendocrine Tumor\r\nSociety (ENETS) grading system. Immunohistochemical staining with Ki- 67, CD10 and cytokeratin 19 was performed. Results. A total of 36 patients with a mean age of 53.7 ? 12.0 years were included. Overall, 33 patients had a long-term follow-up with 10 patients (30.3%) experiencing recurrence. Seven\r\npatients (21.1%) died. Clinical parameters that were associated with recurrence included liver metastasis at the time of surgery and extra-pancreatic invasion (p < 0.005). Positive surgical margins, extra-pancreatic invasion, and multi-focal disease were associated with reduced survival (p < 0.05). In addition, there was an association between\r\nsurvival and WHO 2004 classification (p < 0.05).\r\nConclusions. Although vascular and peripancreatic invasion showed increased risk of recurrence, they were unrelated to survival. Of the histopathological examinations, Ki-67\r\nand mitotic activity showed a correlation with both recurrence and survival, while immunohistochemical\r\nstaining with cytokeratin 19 and CD 10 did not provide adequate prognostic information.