OBJECTIVE:To evaluate the immediate results of enucleation of pancreatic neuroendocrine tumors (pNETs).MATERIAL AND METHODS:The results of enucleation of pancreatic neuroendocrine tumors (pNETs) were analyzed in 95 patients between 2016 and 2021. Functioning tumors (mean size 16.8 mm) were found in 70 patients, non-functioning (mean size 25 mm) - in 25 patients. Intraparenchymal tumors were found in 48 people, extraorganic lesion - in 47 patients.RESULTS:There were 262 patients with pNETs who underwent various surgeries between 2016 and 2021. Various resections were performed in 167 (63.8%) cases, enucleations - in 95 (36.2%) patients. Traditional surgical approach was used in 65 patients. Pancreatic fistula occurred in 21 patients (type B - 17, type C - 4), while arrosive bleeding occurred in 6 patients with unfavorable outcomes in 2 cases. Minimally invasive surgeries were performed in 30 patients. Eight patients with intraparenchymal tumors required conversion to open surgery. Type B pancreatic fistula occurred in 5 patients that led to arrosive bleeding in 2 cases (hemostasis was provided by endovascular method). Comparison of intraparenchymal and extraorgan tumors regarding the incidence of pancreatic fistula revealed odds ratio 5.26 (95% CI 1.5355; 18.0323, p=0.0041). Postoperative mortality was 2.1%.CONCLUSION:Enucleation is advisable for highly differentiated pancreatic neuroendocrine tumors up to 2 cm. Minimally invasive enucleation is indicated for extraorgan tumors. Intraparenchymal tumors significantly increase the risk of postoperative complications.

The study of the genetic aspects of endocrine diseases is based on the aspiration to develop the methods of early diagnosis, treatment and observation of patients. Von Hippel-Lindau syndrome is genetically determined disease characterized by damage of various organs and systems. The article presents a clinical case of treatment of a patient with retinal detachment who was first admitted to the surgical department of the Federal State Budgetary Institution «NMIC of Endocrinology» of the Ministry of Health of Russia with complaints of dry mouth, general weakness. Further examination, revealed pathological changes in the adrenal glands, kidneys, brain, pancreas, spleen, spinal cord. The presented clinical case demonstrates the need for a multidisciplinary approach to the management of patients with von Hippel-Lindau syndrome.

Cushing’s syndrome accounts for approximately 20–30% of endogenous hypercortisolism cases, and adrenal involvement can be either unilateral or bilateral. Cushing’s syndrome due to bilateral adrenal tumors is extremely rare. Adrenal oncocytomas are another rare cause of endogenous hypercortisolism: about 13 cases are described in the literature. Oncocytomas are rare epithelial neoplasms, characterized by abnormally excessive accumulation of defective mitochondria in the cytoplasm of cells, and make up 1.8% of all adrenal neoplasms. We describe a 58-year old patient with Cushing’s syndrome and bilateral adrenal tumors. Multispiral computed tomography of the adrenals showed signs suspicious of lipid-poor atypical adenomas or malignant tumors. Surgical treatment was the method of choice, and the larger tumor was excised first. Due to the absence of remission of endogenous hypercortisolism the excision of the second tumor was performed. Morphological and immunohistochemical examination confirmed the diagnosis of bilateral oncocytic adrenocortical tumors with uncertain malignant potential. Cases of bilateral hormone-producing adrenal oncocytomas have not been described in the literature.

The significance and mechanisms of action of different antithyroid antibodies in diffuse toxic goiter (Graves' disease) and chronic lymphocytic thyroiditis (Hashimoto’s disease) arc analyzed. Antibodies immediately decreasing the level of cAMP in isolated thyrocytes were revealed in the sera of adolescents with juvenile struma, often resulting from lymphocytic thyroiditis. Complement-fixing cytotoxic antibodies are heterogeneous in patients with Graves' diseases and Hashimoto's thyroiditis. Thyrocytes from the tissue of diffuse toxic goiter are resistant to the cytolytic effect of such antibodies from patients with Graves' diseases but not from patients with Hashimoto's thyroiditis. The causes and mechanisms of development of resistance of thyrocytes from diffuse toxic goiter to antibody-dependent complement-mediated cytotoxicity of sera from patients with Graves' disease and the possibility of using this phenomenon as a differential diagnostic test are discussed.

The topical diagnosis of insulinoma continues to be an actual problem of modern medicine due to low detection rate of the tumor (about 75%) and inconsistent data on its localization when using various methods of visualization (more than in 50% of cases), as well as the lack of a single diagnostic algorithm. In the clinical practice, many different imaging assessments are conducted, including those with the administration of contrast agents and associated with radiation load, as well as invasive studies, associated with risk of various complications, high costs and duration of the examination. Thus, the search for highly sensitive and safe methods of topical diagnosis of the insulin-producing pancreatic tumor seems relevant. In the presented clinical case, the diagnosis of insulinoma is verified by diffusion-weighted magnetic resonance imaging (DW-MRI). It is an accurate and non-invasive method that does not involve exposure to ionizing radiation and does not require any administration of contrast agents. This technology, based on microstructural pathological changes, allows to identify small tumors and to make a differential diagnosis of benign and malignant neoplasms. However, at present the experience of DW-MRI use for the diagnosis of an insulin-producing neuroendocrine tumor is limited. Evaluation of its effectiveness in a large patient cohort would be necessary to assess the prospects for its introduction into clinical practice.

Primary hyperparathyroidism is common clinical endocrine disorder with a prevalence between 1–2%. Solitary parathyroid adenomas account from 80 to 85% of cases of PHPT, hyperplasia and multiple adenomas is up to 15%, parathyroid carcinoma is a very rare cause of PHPT, accounting for about a 1% of cases. Clinically aggressive and atypical adenomas should be separately noted because of severe clinical course and life-threatening hypercalcemia, high morbidity and mortality, unknown malignant potential. No definite criteria are considered to be present to distinguish preoperatively atypical adenoma from parathyroid typical adenoma or carcinoma. The clinical course of the disease remains the only tool that allows to suspect an aggressive tumor on the preoperative stage. We present the clinical case of a 61-year-old female patient with a clinically “aggressive” course of PHPT and severe metabolic disturbances of bone tissue due to the atypical adenoma of the parathyroid gland.

We report the case of a 61 year old woman with a typical clinical picture of ACTH dependent Cushing’s syndrome. Diagnostic tests confirmed ACTH dependent Cushing’s syndrome, which is caused by corticomedullary mixed tumor. ACTH-secreting pheochromocytoma are rarely described in accessible literature, there is no one case report about ACTH-secreting corticomedullary mixed tumor. Due to the rarity of the disease, we believe it appropriate to share our experience and consider all variants of interaction in hypothalamic-pituitary-adrenal axis, which could lead to morphology and clinical changes uncovered in this case.

One of the most difficult in diagnostic and treatment options for endogenous Cushing is the ectopic ACTH syndrome, which causes the development of tumors of different histogenesis localization producing adrenocorticotropic hormone (ACTH), and much less - corticotropin hormone (CRH). ACTH-secreting tumors varied in location, morphological structure and the degree of malignancy. Most of these tumors are characterized by an aggressive course with a propensity to metastasize and relapse. The article presents data of the prevalence, pathogenesis of ectopic ACTH tumors localized in the thymus, analyzis of clinical, morphological features, the methods of diagnosis and treatment. Based on the current literature, the world and our own experience on the diagnosis and treatment of patients with ectopic ACTH syndrome with localization of hormone production in the thymus, we want to highlight the current state of the problem in order to create the most efficient algorithm for diagnostic search and treatment of this difficult group of patients.

The ultimate rarity of ACTH-ectopic syndrome caused by a caecal tumour, its diversified clinical picture, and difficulty of topical diagnostics are the main causes of many diagnostic errors and the resulting unsuccessful treatment of this condition. The present paper was designed to report 3 our experience with the management of the patients presenting with this pathology.

Postoperative hypocalcemia is a most frequent complication following thyroidectomy in the patients presenting with non-toxic multinodular goiter. It may require long-term hospitalization, additional treatment, a large number of analyses, and frequent visits to outpatient facilities. The objective of the present study was to identify the predictors of postoperative hypoparathyroidism for the prevention of the development of hypocalcemia and its medicamental correction. The clinical data were collected prospectively during the period from October 2011 till May 2013. A total of 140 patients were available for the observation after thyroidectomy performed for the management of non-toxic multinodular goiter. Indications for the surgical intervention included cervical compression syndrome and a cosmetic defect. The patients were divided into two groups depending on the serum calcium level in the postoperative period. Group 1 (n=45) was comprised of the patients having the serum calcium level 2.00 mmol/l or lower, group 2 (n=95) included the patients showing the serum calcium level above 2.00 mmol/l. In addition, serum 25(OH) vitamin D and parathyroid hormone (PTH) levels were measured in all the patients pre- and postoperatively. The preoperative 25(OH)D level and postoperative calcium and PTH levels in the patients of group 1 were significantly lower than in group 2 (p=0.001). It was shown that the main prognostic factors of postoperative hypocalcemia are the preoperative 25(OH)D level below 15 ng/ml (p<0.001), postoperative PTH level below 10 pg/ml (p=0.01), and the age above 50 years (p=0.01). It is concluded that in the majority of the patients the age, the low serum 25(OH)D level in the preoperative period, and the low postoperative PTH level are significantly related to the development of postoperative hypocalcemia.

Beckwith-Wiedemann syndrome (BWS) is a genetic overgrowth disorder involving a predisposition to tumor development. The common features of Beckwith-Wiedemann syndrome include omphalocele, macroglos- sia and macrosomia. The increased risk for neoplasia is concentrated in the first eight years of life. However, this case presents a late onset of adrenocortical cancer assosiated with Beckwith-Wiedemann syndrome.

Синдром Беквита-Видемана это генетически обусловленное нарушение роста и развития с высоким риском канцерогенеза в детском возрасте. При синдроме Беквита-Видемана часто отмечают макроглоссию, грыжи передней брюшной стенки и макросомию. Первые восемь лет жизни особенно опасны в отношении развития неоплазии. Тем не менее данное клиническое наблюдение демонстрирует возможность поздней манифестации адренокортикального рака у пациентки с синдромом Беквита-Видемана.

Neuroendocrine tumors have the ability to produce the hormones and vasoactive peptides. Excess of these hormones leads to different symptoms and syndromes because of organs' injuries. Detection of ACTH origin by using of modern diagnostic methods is not always possible. Lungs and bronchi are one of the most frequent localization of ACTH-producing tumors. It is considered that carcinoids with bronchopulmonary localization like a benign tumors in the clinical course. But at the same time carcinoid tends to metastasize, so timely diagnostics and treatment improve quality of life significant and increase the life expectancy of patients. The modern state of diagnostics and surgical treatment problem of ACTH-producing tumors with bronchopulmonary localization is presented in the article. It was described the brief historical background, clinical symptoms, instrumental and biochemical methods of diagnosis. The principles of surgical treatment are presented in the article.

The aim of this prospective study was to determine the main factors influencing the development of postoperative hypocalcemia in patients with Gravesdisease after thyroidectomy. Assess whether patients with BG likely to develop postoperative hypocalcemia than those who performed thyroidectomy about nontoxiс multinodular goiter. In this study were prospectively included 54 patients with Gravesdisease, which from October 2011 to May 2013 was performed thyroidectomy. Additionally, as a control group from the database included 48 patients with nontoxiс multinodular goiter, who underwent thyroidectomy in the same time. All patients attended deficit/insufficiency of vitamin D (25(OH)D were 20/30 ng/ml). Patients were divided into 2 groups according to the postoperative calcium concentrations corrected for albumin in the blood: Group 1 - patients with postoperative levels of calcium in the blood of 2.0 mmol/l or less; Group 2 - Patients with blood calcium levels above 2.0 mmol/l. Thus, when patients Gravesdisease Group 1 disease duration was significantly higher concentration of 25(OH)D and postoperative parathyroid hormone was significantly lower compared to the 2nd group. According to logistic regression analysis, postoperative PTH level below 10 pg/ml was the main predictors of postoperative hypocalcemia ( p < 0,001). Patients with Gravesdisease increasingly requiring the appointment of calcium after the operation, they are demonstrating a significant clinical signs of hypocalcemia than patients with nontoxiс multinodular goiter after thyroidectomy.