ACTA ENDOCRINOLOGICA (BUC)

Ectopic lingual thyroid is a rare developmental abnormality caused by aberrant embryogenesis during thyroid migration. Even though, most patients are asymptomatic, uncommonly the mass can be enlarged and cause dysphagia, dyspnea, upper airway obstruction, dysphonia, hypothyroidism. We report a very rare case of ectopic lingual thyroid presenting with massive hematemesis.

Isolated sarcoidosis of the hypothalamic-pituitary system is a very rare form of neurosarcoidosis. A high index of suspicion is required for diagnosis and the choice of therapy embodies another challenge due to lack of standardized protocols. Glucocorticoids are the mainstay of initial treatment, whereas the second and third-line therapy include immunomodulators and cytotoxic drugs, in addition to monoclonal antibodies. This report presents an unusual case of panhypopituitarism in a 32-year-old previously healthy male patient due to isolated hypothalamo-pituitary sarcoidosis confirmed histologically, refractory to pulsedose glucocorticoids and then successfully treated by methotrexate. Based on our report, in patients requiring additional therapy usage of the methotrexate as the second line agent should be considered, however the time frame and the dosing schedule of methotrexate are still unknown and deserve further investigation.

Background. Primary lymph nodes gastrinomas are rare and difficult to diagnose not only because of their small size, but also because modern imaging techniques have limitations in detecting duodenal and/or adjacent lymph nodes gastrinomas. Endoscopic ultrasonography is particularly useful in the identification of gastrinomas and can provide cytopathological evaluation for a gold standard diagnosis. Aim. To report a rare case of periduodenal lymph node gastrinoma and to emphasise the value of endoscopic ultrasound evaluation in patients with Zollinger Ellison syndrome. Methods. Presentation of clinical case and comments. Results. The current study reports a rare case of a patient with a small size gastrinoma located extrapancreatically and extraduodenal in a periduodenal lymph node, which exhibited the Zollinger-Ellison syndrome. Gastroduodenoscopy evidenced moderate esophagitis and multiple, superficial gastroduodenal ulcers. Basal gastrin levels were elevated. Enhanced abdominal computer tomography showed a small tumor located near the pancreas head with a very fast and fine enhancement in an early arterial phase. Endoscopic ultrasound-guided fineneedle aspiration of the periduodenal lesion was performed. Histopathology and immunohistochemistry evaluation were positive for a low risk neuroendocrine tumor. A double dose of proton pump inhibitors was recommended resulting in an improvement of symptoms. No symptoms, endoscopic lesions or additional tumors at up to 9 months of follow-up were detected. Conclusions. We report a rare case of low risk lymph node gastrinoma, small in size at diagnosis with favorable results after a double dose of proton pump inhibitors was recommended. Using endoscopic ultrasound, an early and complete diagnosis was established.

Xanthogranulomas are inflammatory lesions exceptionally rarely occurring in the sellar region. Sellar xanthogranulomas (SXG) result from secondary hemorrhage, infarction, inflammation or necrosis upon existing craniopharyngioma (CP), Rathke`s cleft cyst (RCC) or pituitary adenoma (PA), or represent a stage in xanthomatous hypophysitis evolution. “Pure SXG” are independent of a preexisting lesion. A 70 year old male patient, laryngeal cancer survivor, presented with central diabetes insipidus (CDI). MRI revealed an intra-suprasellar mass of uncertain origin. Transsphenoidal surgery resulted in an efficient lesion resection with maximal pituitary sparing. Pathological report has confirmed SXG without conclusive identification of preexisting sellar lesion. Age at presentation and gender were atypical for SXG. The most frequent presenting signs of SXG were absent. Most SXG are initially misdiagnosed as CP, RCC or PA. Preoperative clinical and radiological uncertainty may impact operative planning. Differentiating from CP is crucial, due to divergent operative target goals and prognosis. Intraoperative frozen section analysis could guide surgical extensiveness. Close collaboration must include endocrinologist, neuroradiologist, neurosurgeon and pathologist. Quantity and quality of provided tissue are essential for avoiding bias in pathohistological analysis of cystic or heterogenous lesions. Awareness is needed of new pathological entities in the sellar-parasellar region. SXG should be considered in differential diagnosis of CDIcausing sellar lesions.

A young Caucasian woman presents several episodes of severe fasting hypoglycemia. Fasting lab tests revealed: glycemia 28 mg/dL, insulinemia 143.3 μU/mL, insulin antibodies above 100 U/mL, leading to the diagnosis of insulin autoimmune syndrome. Due to lack of clinical improvement after 2 months, prednisone was started at 0.5 mg/kg/day, and then tapered by 5 mg every 5 days. Three weeks after discontinuing corticotherapy, the patient had no more severe fasting hypoglycemia, but occasionally postprandial mild hypoglycemia. Fasting lab tests showed: glycemia 83 mg/dL, insulinemia 58.6 μU/mL. At 5 hours during oral glucose tolerance test glycemia was 33 mg/dL, insulinemia 152.9 μU/mL.

Diabetes is one of the most prevalent chronic disorders, associating numerous somatic and behavioral modifications. Oxytocin has been widely studied for its involvement in social behavior and psychiatric disorders. This pilot study presents a series of 3 patients with type 1 diabetes and diabetic neuropathy in which the values of plasma oxytocin, neurotensin, β-endorphins, α-MSH, substance P and orexin A were measured in comparison to 3 healthy controls with matching ages. In the diabetic patients group, there was a strong negative correlation between the value of plasma glucose and oxytocin (r=-0.99, p=0.04), respectively neurotensin (r=- 0.99, p=0.03). These values did not correlate in the control group. The results suggest that oxytocin, in conjunction with neurotensin, could be investigated as a potential early detection marker of diabetic neuropathy and, to our knowledge, this is the first report focusing on plasma oxytocin levels in patients with diabetic neuropathy.

Context. Open varicocelectomy is generally performed without microscopic equipment in Iran. We report our experience with loupe-assisted inguinal varicocelectomy, and its impact on semen parameters and pregnancy rate. Subjects and Methods. We conducted a retrospective interventional study on secondary data obtained from the medical records of 303 men with varicoceles, who underwent an inguinal varicocelectomy between March 2003 and April 2012. The surgical technique involved the use of a 3.0 × loupe during spermatic cord dissection at the level of the internal inguinal ring under spinal anesthesia. Semen samples were analyzed for sperm concentration, motility, and sperm morphology before the varicocelectomy and after 3 months. All the infertile patients were followedup postoperatively for more than 1 year. Results. There were 9 (3.0%) varicocele recurrences and 3 (1.0%) hydroceles. After varicocelectomy, sperm concentration and motile spermatozoa increased. In addition, spermatozoa with normal morphology improved significantly postoperatively. Of the 303 subjects treated, 153 (83.2) had a 1-year preoperative history of infertility; the spontaneous pregnancy rate of the spouses during the follow-up period was reported to be 61.4%. Conclusions. The results of this research indicated that varicocelectomy using loupe-assisted inguinal technique could improve semen parameters and pregnancy rate with a low postoperative complication rate.

hormone (ACTH) mediated multisystemic state of adrenal hypercortisolism. An ACTH secreting benign pituitary tumor (adenoma) is the most common cause in the majority of patients with CD. Case report. This article describes a case of a 56-year-old woman presenting with clinical manifestations of hypercortisolism, with high plasma cortisol and ACTH levels that was suppressed with high-dose dexamethasone administration, suggestive of CD. Pituitary magnetic resonance imaging (MRI) of the sellar region was inconclusive. During the single photon emission computed tomography (SPECT) examination, an increased accumulation of technetium - 99m - hexakis - 2 - methoxy - isobutyl-isonitrile (99mTc-MIBI) in the pituitary gland area was noticed. Finally, response to corticotropinreleasing hormone (CRH) and ghrelin stimulatory testing, differentiated CD from ectopic ACTH and primary adrenocortical hypersecretion. Conclusion. We highlight the potential of 99mTc- MIBI SPECT as sensitive and specific method of pituitary gland adenoma detection in patients with Cushing’s disease, when MRI fails to directly detect an adenoma and stimulatory tests with CRH and ghrelin are not in routine diagnostics.

Context. Noninvasive encapsulated anaplastic thyroid carcinomas (NE-ATCs) have been described in few case reports, and consistently associated with favorable outcome compared to the classical ATCs. Objective. Our aim is to remark a rare histological finding in ATCs, encapsulation, which has been associated with a favorable outcome. Design. We have documented a rare case of an NE-ATC with its clinical, pathological, and molecular features. We also provided a thorough discussion of all the encapsulated ATCs reported in the literature. Subjects and Methods. A 50-year-old woman with an unremarkable medical history, who presented with a thyroid nodule, and diagnosed as “follicular lesion of undetermined significance” by fine needle aspiration biopsy. The patient was lost to follow-up for six years and revisited upon her neck disturbances and underwent total thyroidectomy. Results. Sections of the right lobe revealed a grossly encapsulated nodular lesion, measuring 75x55x55 mm. Histologically, the tumor consisted of both carcinomatous and sarcomatous components supported by immunohistochemical stains. Necrosis and atypical mitotic figures were evident. Capsular and/or vascular invasion was not identified. There were no BRAF codon 600, KRAS, NRAS mutations and RET/PTC rearrangement. During three-month follow-up, the patient was free of disease without adjuvant therapy. Conclusion. Encapsulated ATCs tend to follow a favorable clinical course and may deserve conservative treatment approaches.

Background and Purpose. Fibroblast growth factor 21 (FGF21) has recently been identified as a metabolic regulator, but its physiological role is still not completely known. The aim of this study was to evaluate serum FGF21 levels in an Iranian population with type 2 diabetes. Materials and Methods. This cross-sectional study was conducted in patients with type 2 diabetes. All patients were evaluated for fasting serum levels of glucose, glycated hemoglobin (HbA1c), lipids, urea and creatinine. Participants were divided into two groups with poorly-controlled and wellcontrolled diabetes based on their HbA1c levels. Healthy nondiabetic subjects (matched with patients in terms of age, sex and body mass index [BMI]) were also recruited as control group. Serum FGF21 concentrations were determined in all subjects using ELISA. Results. Of the evaluated 141 subjects, 49 (34.8%) were categorized as having well-controlled diabetes, 66 (46.8%) had poorly-controlled diabetes, and there were 26 subjects in the normal control group. Mean serum FGF-21 concentration was 337.89±283.67 ng/L in the diabetic group and 237.25±43.22 ng/mL in the non-diabetic group (p<0.001). Mean serum FGF21 level was 237.25 ± 43.22 ng/mL in the control group, 309.81 ± 301.68 ng/mL in the well-controlled diabetic group, and 358.73 ± 269.98 ng/mL in the poorly controlled diabetic group. Serum FGF21 level in the poorly controlled diabetic group was significantly higher than that in the well-controlled diabetic and the healthy control groups (p=0.02) but there was no significant difference between the well-controlled and healthy groups. There was no significant association between serum FGF21 levels with lipid levels, presence of diabetic complications and BMI (p > 0.05). Conclusions. The present results suggested an association between elevated serum levels of FGF21 and poor control of diabetes. Future studies are warranted to elucidate the prognostic role of these elevated levels of FGF21 in diabetic subjects.