[Solitary mediastinal fibrous tumor complicated by hypoglycemic crises (Doege-Potter syndrome)]. / Solitarnaya fibroznaya opukhol' sredosteniya, oslozhnennaya gipoglikemicheskimi krizami (sindrom Doge­Pottera).

Diagnostic and treatment algorithms for large mediastinal tumors are clear. However, long-term results are not always good. They largely depend on early diagnosis and morphological structure of tumor. Neoplasms may be asymptomatic for a long time, especially in case of slow growth. These tumors are usually diagnosed as soon as complications occur (for example, compression syndrome). Routine X-ray screening is rarer situation. Paraneoplastic syndromes are rare, and some ones are casuistic and unknown to surgical community. We describe the diagnosis and treatment of a patient with giant solitary mediastinal tumor complicated by hypoglycemic crises (Doege-Potter syndrome). This complication was life-threatening and required a multidisciplinary approach. Aggressive surgical approach cured the patient and returned her to normal lifestyle. The proposed algorithm for perioperative drug therapy was effective and deserves attention. This report will be useful for surgeons, oncologists, anesthesiologists, intensive care specialists and endocrinologists.

[Radioactive iodine in the treatment of Graves' disease: history and modern concept of radionuclide therapy].

Radioactive iodine 131I is a theranostic isotope used both for diagnosis and therapy of benign thyroid diseases and thyroid cancer for 85 years. The formation of nuclear medicine is closely linked with the use of 131I. The history of radioiodine therapy began in 1941, when endocrinologist Saul Hertz for the first time used 131I to treat patients with Graves' disease. Since 1946 radioactive iodine 131I became widely available, and its effectiveness became public knowledge after reports on thyrotoxicosis treatment published in the Journal of the American Medical Association by multidisciplinary groups of scientists - physicists and endocrinologists. In 1951, isotope 131I became the first Food and Drug Administration approved RP for the treatment of thyroid disorders. Around the same time on the basis of the First Moscow Medical Institute studies on the use of radioiodine isotopes in patients with thyrotoxicosis began. The head of the Soviet group on the studying of radioactive iodine was the physician-scientist Vera Georgievna Spesivtseva. The research works of medical physicists Edith Quimby and Leonidas Marinelli in optimizing therapeutic strategies using radioactive substances in the late 1940s and the wording of the ALARA (As Low As Reasonably Achievable) principle of minimizing exposure of ionizing radiation by the International Commission on Radiological Protection in 1954 contributed to the greater introduction of radionuclides into the medicine.

[Survival predictors in patients with ectopic acth syndrome].

AIM: To determine significant factors affecting the survival of patients with ectopic ACTH syndrome (EAS). MATERIALS AND METHODS: A multi-center, observational study with a retrospective analysis of patients with EAS. The end point of the study was the fatal outcome of patients from various causes. In order to identify predictors of survival or mortality, univariate and multifactorial Cox regression analyses were carried out. ROC-analysis was used to determine the prognostic threshold values of individual predictors. The survival analysis was carried out using the Kaplan-Mayer method. Statistical data processing was carried out by using IBM SPSS Statistics 23. RESULTS: The age of patients at the time of diagnosis ranged from 12 to 76 years (Me 40 years [28;54]). The age of the studied population was 55 years [38; 64] for women and 42 years [32; 54] for men. The median period of observation was 50 months [13;91], with a maximum follow-up of 382 months. 92 patients (60,9%) had bronchopulmonary NET, 17 (11,3%) - thymic carcinoid, 8 - pancreatic NET, 5 -pheochromocytoma, 1- cecum NET, 1- appendix carcinoid tumor, 1 - medullary thyroid cancer and 26 (17,2%) patients had an occult NET. The primary tumor was removed in 101 patients (66,9%). Bilateral adrenalectomy was performed in 42 (27,8%) cases. Metastases were revealed in 23,2% (n=35) of patients. Relapse of the disease was observed in 24,4%, long-term remission was preserved in 64 patients (74,4%). Death occurred in 42 patients (28%). The average age of survivors was 47,0±15,2 versus 53,5±15,6 years for the deceased (p=0,022). The average survival time from diagnosis for the deceased was 32 months, Me 16,5 months [7;54]. Multivariate analysis revealed that the following factors have a direct impact on survival: age of diagnosis ≥51 years (OR 4,493; 95% CI 2,056-9,818, p<0,001), bronchopulmonary neuroendocrine tumor (NET) (OR 0,281; 95% CI 0,119-0,665, p=0,004), the presence of distant metastases (OR 2,489; 95% CI 1,141-5,427, p=0,022), late-night salivary cortisol (LNSC) ≥122,2 nmol/L (OR 2,493; 95% CI 1,014-6,128, p=0,047). CONCLUSION: The prognosis of patients with EAS is influenced by the age of diagnosis, NET localization, distant metastases and level of LNSC. The most common cause of ectopic ACTH syndrome was bronchopulmonary NET which was associated with the best survival rate.

[Endocrine late-effects and bone mineral density after combined treatment of malignant brain tumors in childhood and adolescence].

BACKGROUND: The implementation of standardized protocols for combined treatment of cancer into clinical practice inevitably leads to a long-term consequence. AIMS: To study the prevalence of endocrine disorders, to assess the prevalence and degree of decline of bone mineral density (BMD) in individuals who have undergone combined treatment of malignant brain tumors in childhood and adolescence. MATERIALS AND METHODS: A retrospective study was conducted with 59 young adults (31 men; 28 women) who have undergone surgical treatment of malignant brain tumour followed by radiation treatment (craniospinal radiation in combination with or without polychemotherapy). Group I consisted of 37 patients, who were treated between the ages of 3 and 16 years. Group II included 22 patients who received treatment between the ages of 16 and 38 years. RESULTS: GH deficiency according to the results of the insulin hypoglycemia test was diagnosed in 48 patients (81%), 22 -patients had secondary adrenal insufficiency (37%). The majority of those examined (33 patients (56%)) did not achieve the target growth. Only 5 people from I group was treated with recombinant GH. Correlation analysis demonstrates that age of treatment is the main factor affecting final growth (r=0,619, p<0,001). Many cases of hypothyroidism (n=39 (66%)) and hypogonadism (19 women; 17 men) were detected. According to the DXA, a decrease of BMD ≤-2.0 SD (Z-score) in L1-L4 was found in 35 of 59 patients (59%). The BMD in the I group was significantly lower than in patients treated at an older age (p<0.001). A moderate correlation was discovered between BMD in L1-L4 at the time of examination and the level of estradiol in women (r=0.596, p<0.05) and testosterone in men (r=0.472, p<0.05). Direct correlation between BMD and age of diagnosis was revealed (r=0.781, p<0.01). CONCLUSIONS: The results show that patients need to be monitored annually and for life after the combined treatment of malignant brain tumors in order to detect the long-term effects of the treatment. The high incidence of osteopenic conditions determines the relevance and need for early diagnosis to prevent further bone loss, reduced bone strength and the risk of fractures.

[Clinical and laboratory characteristics and results of treatment of patients with ACTH-producing neuroendocrine tumors of various localization].

AIM: To study the clinical, biochemical characteristics, treatment results and follow-up of patients with ectopic ACTH syndrome EAS (ACTH adrenocorticotropic hormone ). MATERIALS AND METHODS: A retrospective, observational, single-center study of 130 patients with EAS. Demographic information of patients, medical history, results of laboratory and instrumental investigations at the pre- and postoperative stages and follow-up of EAS were analyzed. RESULTS: The mean age at the diagnosis ranged from 12 to 74 years (Me 40 years [28; 54]). The duration of the disease from the onset of symptoms to the verification of the diagnosis varied from 2 to 168 months (Me 17.5 months [7; 46]). Eighty-one (62,3%) patients had bronchopulmonary NET, 9 thymic carcinoid, 7 pancreatic NET, 5 pheochromocytoma, 1 cecum NET, 1 appendix carcinoid tumor, 1 medullary thyroid cancer and 25 (19.2%) had an occult source of ACTH. The median follow-up period of patients was 27 months [9.75; 61.0] with a maximum follow-up of 372 months. Currently, primary tumor was removed in 82 (63.1%) patients, bilateral adrenalectomy was performed in 23 (18%) patients, in 16 of them there was an occult source of ACTH-producing NET and in 7 patients in order to control hypercortisolism after non-successful surgical treatment. Regional and distant metastases were revealed in 25 (19.2%) patients. At the time of the last observation 59 (72%) patients were exhibited a full recovery, 12 (14.6%) had relapse of the disease and 26 (20%) died from multiple organ failure (n=18), pulmonary embolism (n=4), surgical complications (n=2), disseminated intravascular coagulation syndrome (n=1) or COVID-19 (n=1). CONCLUSION: In our cohort of patients bronchopulmonary NET are the most frequent cause of EAS (62.3%). Surgical treatment leads to remission of hypercortisolism in 72% cases; the proportion of relapse (14.6%) and fatal outcome (20%) remains frequent in EAS.

[Nosological structure of thyroid nodular lesions by autopsy data]. / Nozologicheskaia struktura uzlovykh obrazovanii shchitovidnoi zhelezy po dannym autopsii.

629 autopsies were studied to elucidate nosological structure of thyroid nodular lesions. The material was epidemiologically representative in relation to older Moscow population. Colloid stroma with proliferation, atrophic and scarred alteration (total 80.3% cases) prevailed among nodular lesions more than 1 cm in diameter followed by adenomas (11.6%), carcinoma (5.8%) and autoimmune thyroiditis (2.3%). It is shown that alcoholic disease is associated with thyroid atrophy and low risk of goiter alteration.