Vol 10, No 4 (2016)

Review of literature
Radioiodine therapy of thyrotoxicosis in children and adolescents. Indications, efficacy and safety. Literature review
Rumiantsev P.O., Kiyaev A.V., Sehemeta M.S., Chikulaeva O.A.

Graves’ disease (GD) is the most prevalent cause of thyrotoxicosis in children. Comprehensive clinical practice contains three methods of choice in the treatment of GD: antithyroid drugs, radioactive iodine and surgery. All these treatment methods are using for a long time resulting in great amount clinical experience accumulated. Virtually in all cases the treatment starting with antithyroid drugs and then inefficiency or complications leads to choose the alternative treatment method. The conscious choice of treatment method is based on potential efficacy and safety parameters understanding as well as affecting factors. The purpose of this review – a critical evidence-based analysis of efficacy/safety of radioiodine treatment of GD in children and adolescents compared with other treatment methods as well as the place and indications for this type of treatment in contemporary clinical practice of endocrinologists, radiologists and endocrine surgeons. The choice of treatment strategy at any step is adjusting with patient and his parents/guardians by informing with objective merits and demerits of methods upon existing evidence basis. Main recommendations of effective and safe therapy of GD with radioiodine in children and adolescents are also disclosed.

Endocrine Surgery. 2016;10(4):6-12
Clinical practice
Indolent form of adrenocortical carcinoma: clinico-morphological features of 7 patients.
Bokhian V.U., Pavlovskaya A.I., Kolomeytseva A.A., Bugaev V.E., Stilidi I.S.

Adrenocortical carcinomas (ACC) usually tends to be highly aggressive tumor with very poor prognosis, but some patients may have more indolent form of disease with much longer progression-free period despite of presence of distant metastases or repeated recurrence of disease. Stable tumor size (no growth of recurrent tumor or distant metastases for more than 12 months) was considered as indolent form of disease. We have analyzed clinical and histological characteristics (Weiss criteria, Ki-67 index, expression of p53, b-catenin) of recurrent or metastatic ACCs in 7 patients with indolent disease, and have compared it with control group consisting of 157 patients with ACC, who have undergone surgical treatment. Seven patients underwent repeated surgical treatment (up to 5 times) because of indolent form of recurrent or metastatic ACC. Longest time to progression was 37 months. There were no significant differences in clinical characteristics, Weiss score, Ki-67 index and expression level of p53 between two groups of patients. Indolent form of ACC was characterized by the absence of both nuclear and cytoplasmic expression of b-catenin expression; differences between two groups were statistically significant. Biological and molecular features of primary tumor, which usually does not correlate with clinical features, determine indolent form of ACC. Probably, inactivation of Wnt/b-catenin signaling pathway is one of the main mechanism leading to less aggressive disease progression. The phenomenon of unusually low aggressive natural history of ACC and its  biological basis needs to be further investigated.

Endocrine Surgery. 2016;10(4):13-19
Factors predicting the outcomes of removal of corticotropinom in Cushing's disease
Marova E.I., Kolesnikova G.S., Arapova S.D., Grigorjev A.U., Lapshina A.M., Melnichenko G.A.

Background. Cushing’s disease (CD) is the heavy disease of the hypothalamic-pituitary-adrenal axis. The cause of the disease is pituitary adenoma (corticotropinoma). Hypersecretion of ACTH by a pituitary adenoma leads to increased secretion of cortisol by the adrenal cortex and the development of total endogenous hypercortisolism. The gold standard treatment for this disease is surgical removal of corticotropinoma. However, adenomectomy is not effective in all cases and approximately 20% of cases after the radical treatment fails to achieve remission of the disease and then comes the relapse.

Aims. The aim of our work was to evaluate the factors influencing the result of adenomectomy in patients with the Cushing’s disease and identification of recurrence predictors.

Materials and methods. The study included 84 patients (80 women and 4 men), aged 18 to 58 years with a Cushing’s disease (CD) before and after the transnasal adenomectomy. The observation period from 3 to 15 years, starting in 2001 (average 9.0 years). There was a retrospective evaluation of clinical and hormonal (ACTH and cortisol) factors before surgery and 1–3 days and 8–13 days after surgery.

Results. Remission of the disease developed in 54 (64.3%) of the 84 patients with CD after transnasal adenomectomy. Surgical treatment was not effective in 30 patients (35.7%), and they had a repeat adenomectomy. According MRI of the brain among patients with remission of the disease was more frequently found pituitary microadenomas (54%), compared with patients in whom operation was not effective, and MRI were more frequent in macroadenomas (63%). All patients with CD regardless of the outcome of neurosurgical operations was observed a significant decrease of cortisol and ACTH in the early postoperative period.

Conclusions. Factors of efficiency of adenomectomy and long-term remission in patients with CD is the levels of morning cortisol less than 100 nmol/l and ACTH of less than 10,0 pg/ml in the early postoperative period (1–13 days). The presence of adrenal insufficiency after adenomectomy is a predictor of the effectiveness of the operation, but does not exclude the likelihood of relapse.

Endocrine Surgery. 2016;10(4):20-30
National clinical recomendations
Russian Association of Endocrinologists clinical practice guideline for adrenal incidentalomas differential diagnosis
Beltsevich D.G., Melnichenko G.A., Kuznetsov N.S., Troshina E.A., Platonova N.M., Vanushko V.E., Yukina M.Y., Molashenko N.V.

This article discusses the management guidelines for serendipitously diagnosed adrenal masses cases, assessment of their hormonal activity and malignancy potential, pro- and contra indications for surgical treatment and follow-up algorithm for hormonally inactive tumors.

Hypercathecholaminemya, endogenous hypercortisolism, primary hyperaldosteronism should be considered as variants of specific hormonal activity of tumor. The midnight suppression test with dexametasone 1 mg is recommended in all cases. Evaluation of basal ACTH in case of negative result of the test with dexametasone 1 mg (absence of morning cortisol level suppression) should be considered as confirmation test. For primary diagnosis of pheohromocytoma/paraganglioma (PPGL) a free plasma or fractionated urine methanephrines concentrations evaluation should be recommended. If test is positive, comprehensive examination to exclude or confirm PPGL is necessity. The aldosterone/rennin ratio exposure should be considered for patients with arterial hypertension to exclude primary hyperaldosteronism. To evaluate malignant pattern of a tumor in all unclear cases should be provide assessment of computed tomography quantitative indices. Adrenal incidentalomas treatment guidelines isn’t considered in the field of this recommendations and reported in relevant guidelines.

Endocrine Surgery. 2016;10(4):31-42

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