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Thyroid hemiagenesis is a rare congenital anomaly in which one thyroid lobe fails to develop. We recently observed a case of thyroid hemiagenesis accompanied by parathyroid adenoma and papillary thyroid microcarcinoma in a 51-years-old woman. The patient’s serum calcium level was 10.9 mg/dL and that of intact parathyroid hormone was 218 pg/mL. Although she had a history of thyroid hemiagenesis, the patient was in a euthyroid state without thyroxine replacement. Thyroid ultrasonography detected no right lobe and four nodules of varying sizes in the left thyroid lobe. Tc-99m scintigraphy also demonstrated the absence of the right thyroid lobe. In addition, MIBI-parathyroid scintigraphy showed a parathyroid adenoma at the lower pole of the right cervical region. Based on the diagnosis, parathyroid adenoma excision and thyroidectomy were performed. Postoperative pathological diagnosis revealed parathyroid adenoma and papillary thyroid microcarcinoma in the form of a 0.4 cm sized tumor in the left thyroid lobe. The coexistence of thyroid hemiagenesis, primary hyperparathyroidism, and papillary thyroid microcarcinoma is truly exceptional and has never been reported in the literature before. © 2018 by Turkish Journal of Endocrinology and Metabolism Association.

Triple-A syndrome is a rare, multi-systemic disease and is characterized by adrenal insufficiency, achalasia, or alacrima. The symptoms may also involve neurologic manifestations, including pyramidal findings and peripheral motor neuropathy. Other findings include autonomic dysfunction and cerebellar ataxia. In the present study, we present the case of two siblings with triple-A syndrome with neurologic manifestations. The neurologic abnormalities can lead to misdiagnosis owing to resemblance to the manifestations of other neurologic disorders. Therefore, the cases with tripleA syndrome should be carefully evaluated and examined for clinical and neurophysiologic signs. © 2018 by Turkish Journal of Endocrinology and Metabolism Association.

Ganglioneuromas are rarely occurring benign tumors characterized by hyperplasia of mature ganglia and satellite cells. They are well-differentiated, slow growing, and autonomous nervous system neoplasms, which are usually asymptomatic and do not release any hormones. A male patient aged 26 years was evaluated for secondary hypertension six months ago. Ultrasonography of the abdomen revealed a mass lesion around the right kidney. An analysis of the 24-hour urine sample of the patient revealed the following parameters: 5-HİAA=3.9 mg/day (2-7), metanephrine=56.3 µg/day (52-341), and normetanephrine=146.1 µg/day (88-444). The computed tomography scan of the abdomen showed a retroperitoneal mass of 10 cm in size, containing minute calcified foci in the right retroperitoneal region. The mass was excised through general surgery and was classified as ganglioneuroma. The blood pressure of the patient returned to normal level after surgery, and he needed no further antihypertensive treatment. Besides, the metanephrine and normetanephrine levels in the 24-hour urine were also observed to be normal as in the preoperative period. Retroperitoneal masses can actually be ganglioneuromas and an accurate diagnosis can be achieved only through postoperative histopathological evaluation. After the operation, blood pressure of the patient returned to normal. This suggests that retroperitoneal ganglioneuroma could possibly secrete dopamine, epinephrine, or norepinephrine. © 2018 by Turkish Journal of Endocrinology and Metabolism Association.