Proliferation and hyperactivation of B-lymphocytes in the salivary glands is a feature of primary Sj ц gren's syndrome (pSS). Detection in saliva of proteins synthesized by B-lymphocytes may be important in the diagnosis of this disease. Objective: to evaluate the diagnostic value of measuring the concentration of immunoglobulin free light chains (FLC) in saliva in patients with pSS. Material and methods. The cross-sectional study included 24 patients with pSS over the age of 18 years. PSS was diagnosed according to the 2016 ACR/EULAR classification criteria. The control group consisted of 11 healthy volunteers. Blood-salivary glands histohematic barrier permeability ratio for albumin, FLC was measured. Quantitative determination of FLC and in blood and saliva was performed by enzyme immunoassay. An immunohistochemical study of biopsies of minor salivary glands (MSG) was carried out with a quantitative assessment of CD3+, CD4+, CD8+, CD20+, CD21+, CD68+, CD138+ cells. The Mann–Whitney U-test was used to compare quantitative traits. Identification of diagnostic thresholds for the concentration of FLC in saliva for the diagnosis of pSS was carried out using the ROC analysis method. An operating characteristic curve was plotted, the area under the curve, indicators of diagnostic specificity, diagnostic sensitivity, and diagnostic accuracy were calculated. Results and discussion. The obtained values corresponded to the low permeability of the histohematic barrier of the salivary glands for albumin and FLC in patients with pSS and healthy individuals. The median concentrations of FLC ê and ë in the saliva of patients with pSS and healthy volunteers were 1.08 [0.58; 1.91], 1.038 [0.55; 2.03] mg/l and 0.36 [0.32; 0.54], 0.35 [0.21; 0.52] mg/l, respectively. The concentration of FLC in the saliva of patients with pSS was statistically significantly higher than in the control group (p<0.01). The amount of FLC ê and ë in saliva correlated with the rate of unstimulated saliva flow: r s =-0.483 (p=0.02), r s =-0.491 (p=0.017), respectively.A relationship was found between the concentration of ê -chains in saliva and the specific number of CD138+ cells: r s =0.733 (p=0.025). Statistically significant correlations between the concentration of ë -chains and the number of mononuclear cells in the MSG have not been established.Based on the results of ROC analysis, diagnostic thresholds for FLC concentrations in the saliva of patients with pSS were determined. Concentrations of ê - and ë -type FLC in saliva of 0.56 and 0.68 mg/l correspond to area under the curve values of 0.84 (95% confidence interval, CI 0.69–0.98) and 0.83 (95% CI 0.71–0.97), sensitivity 79.2% (95% CI 59.5–90.8) and 75% (95% CI 55.1–88), specificity 81.8% (95% CI 52.3–96.8) and 90.9% (95% CI 62.3–99.5), respectively.Salivary FLC concentrations were compared in patients with pSS receiving and not receiving glucocorticoids (GC). The groups did not differ in a statistically significant way in terms of clinical and laboratory parameters. The median daily dose of GC was 10 [5; 10] mg in prednisolone equivalent. There were no significant differences between the concentrations of saliva FLC in patients of these groups. Conclusion. Salivary-fixed FLCs are most likely produced by cells localized in the stroma of the salivary glands. Determination of the concentration of FLC in saliva can be proposed as a diagnostic test for the pSS. The concentration of free ê -chains in saliva can be considered as a surrogate marker of benign B-cell proliferation in the MSG. Therapy with low and medium doses of GC in pSS does not affect the concentration of FLC in saliva.

Synovial sarcoma of the retroperitoneum is a rare and aggressive tumor. In some cases, differential diagnosis between retroperitoneal sarcoma and adrenocortical cancer is difficult due to the similar clinical and intrascopic manifestations of these neoplasms. The final diagnosis is established based on the results of histological, immunohistochemical and genetic studies. The most important step in the treatment of synovial sarcoma is complete removal of the tumor. However, despite the radical nature of the intervention, the incidence of local relapse and metastasis remains high. AIM: to conduct a retrospective analysis of the parameters of operated patients with synovial sarcoma of the retroperitoneum. This study is aimed at studying the results of examination and surgical treatment of patients with synovial sarcoma of the retroperitoneal space who were hospitalized in the E.E. Eichwald Clinic surgical department of the North-Western State Medical University named after. I.I. Mechnikov. The patients were assessed for hormonal status using an immunoassay to determine the levels of adrenocorticotropic hormone, cortisol, aldosterone, renin, normetanephrine and metanephrine in the blood, and a dexamethasone suppression test has been performed at a dose of 1 mg. High-performance liquid chromatography has determined 6 glucocorticoids in the blood serum: cortisol, cortisone, corticosterone, 11-deoxycorticosterone, 11-dehydrocorticosterone, 11-deoxycortisol. The urine steroid profile was studied in all the patients using gas chromatography-mass spectrometry. All the patients underwent computed tomography of the abdominal organs and retroperitoneal space with contrast, morphological and immunohistochemical examination of the removed material. The material was fixed in 10% neutral buffered formalin. The pieces were then subjected to standard wiring followed by embedding in paraffin. Sections 2–3 microns thick were prepared from the resulting blocks and stained with hematoxylin and eosin. Immunohistochemical studies were performed on paraffin sections. A panel of monoclonal antibodies was used: protein S-100 (poly), pancytokeratin (AE1/AE3), EMA, cytokeratin-8, -18, chromogranin A, synaptophysin, α-inhibin, SOX-10, CD99, TLE-1, bcl- 2, GATA-3. Postoperative outcomes and follow-up were assessed according to the information in the medical records and data obtained from a telephone conversation with each patient. Statistical processing of the results has not been out due to rare cases of the disease.

Primary Sjogren’s syndrome (pSS) is one of the most frequent among the connective tissue diseases. Histological examination of the minor salivary gland (MSG) is important diagnostic method. The currently established histological criteria for pSS do not have absolute sensitivity and specificity, which makes the search for additional morphological hallmark relevant.Aim – to study of the qualitative and quantitative composition of cellular populations inflammatory infiltrates in MSG pSS patient with the assessment of additional diagnostic criteria for disease based on the data obtained.Subjects and methods. The study included 55 patients with a diagnosis of pSS according to the criteria of ACR/ EULAR 2016. The control group consisted of 18 healthy volunteers. A MSG biopsy was performed all subjects for histological and immunohistochemical studies with a quantitative assessment of CD3+, CD4+, CD8+, CD20+, CD21+, CD68+, CD138+ cells. Statistical data analysis was performed using the Statistica 10.0 for Windows (StatSoft Inc., USA). For comparison of quantitative traits, the Mann – Whitney U-test was used. To determine the diagnostic threshold of the number of a ROC analysis was performed. An operating characteristic curve was plotted. The area under the curve (AUC), diagnostic specificity, diagnostic sensitivity, diagnostic accuracy, likelihood ratio of the positive and negative results of the test were calculated. The construction of classification models, including the number of different cell types, was carried out using linear discriminant analysis.Results and discussion. The number of CD3+, CD4+, CD8+, CD20+, CD138+ cells in 4 mm2 (area of section) was significantly higher in the pSS group. The largest AUC were observed for the quantitative evaluation of CD3+ cells – 0.88 [95% confidence interval (CI): 0.80–0.96] and CD8+ cells – 0.87 [95% CI: 0.79–0.95], which at the specified diagnostic thresholds corresponded to the sensitivity of 70.9% [95% CI: 57.86–81.23] and 65.45% [95% CI: 52.25–76.64], specificity of 94.4% [95% CI: 74.24–99.72] and 100% [95% CI: 82.41–100], respectively. The CD21+ follicular dendritic cells were detected only in MSG of pSS group. AUC for quantitative assessment of these cells was 0.65 [95% CI: 0.52–0.78], sensitivity 29.1% [95% CI: 18.77–42.14] and specificity 100% [95% CI: 82.41–100]. Using the method of discriminant analysis, we designed classification models that included various combinations of the studied markers. The highest AUC among all possible combinations was observed for the decimal logarithms of the number of CD3+ and CD68+ cells – 0.92 [95% CI: 0.85–0.98], which for a given diagnostic threshold corresponded sensitivity – 81.82% [95% CI: 69.67–89.81], specificity – 94.4% [95% CI: 74.24–99.72].

Two cases of postoperative diagnosis of chronic pulmonary aspergillosis are presented, which were previously regarded as malignant neoplasms. A decisive role in the detection of chronic pulmonary aspergillosis is played by computed tomography, but the diagnosis should be confirmed by laboratory tests. The importance of early diagnosis of chronic pulmonary aspergillosis is associated with high risk of complications during surgery without the use of antifungal drugs.

Цель – провести клинико-морфологический и гормональный анализ оперированных больных с изолированными миелолипомами и миксоидными аденомами с миелолипомой надпочечника. Материал и методы. В основу работы легли полученные результаты обследования оперированных больных с миелолипомами надпочечника за период с 2000 по 2015 г. Был проведен анализ гормональной активности изолированных и миксоидных миелолипом надпочечника. Выполнено патоморфологическое и иммуногистохимическое исследование удаленных образований надпочечника. В работе представлены редкие клинические наблюдения больных, проходивших лечение в клинике Э.Э. Эйхвальда СЗГМУ им. И.И. Мечникова со случайно выявленными образованиями, представленными комбинацией аденомы и миелолипомы надпочечника. Результаты. Частота встречаемости изолированных миелолипом и миксоидных аденом с миелолипомой среди инциденталом составила 6,3% и 4,9% соответственно. Средний возраст больных с миелолипомой надпочечника равен 54,5 года. Соотношение между женщинами и мужчинами составило 2:1. На основании полученных данных выявлено четыре типа гормональной активности миксоидных образований надпочечника. 1 тип ассоциирован с синдромом Кушинга, 2 тип – с синдромом Конна, 3 тип – с автономной секрецией кортизола, 4 тип – без гормональной активности. На основании результатов, полученных методом высокоэффективной жидкостной хроматографии (ВЭЖХ), отмечена 11â-гидроксилазная недостаточность, а по данным исследования стероидного профиля мочи методом газовой хромато-масс-спектрометрии (ГХМС) получены признаки недостаточности 11â-гидроксистероиддегидрогеназы 2 типа и повышения активности 5a-редуктазы у больных с изолированной миелолипомой надпочечника. Заключение. Ассоциации аденомы с миелолипомой, а также аденомы, миелолипомы и кавернозной гемангиомы являются редкой патологией, диагностика которой основана на патоморфологическом исследовании удаленной опухоли. Миелолипома в составе аденомы надпочечника является инциденталомой, которая может быть разного размера и сопровождаться гормональной дисфункцией клеток коркового слоя надпочечника. Неопределенный интраскопический фенотип и гормональная активность миксоидных образований являются решающими факторами в пользу хирургического вмешательства.

Objectives to reveal the clinical, morphological and hormonal characteristics of patients operated on for isolated adrenal myelolipoma and myxoid adenomas with adrenal myelolipoma. Material and methods.The study was based on the results of examination of patients operated on for adrenal myelolipoma in the period from 2000 to 2015. During the study, the hormonal activity in patients with isolated and myxoid adrenal myelolipomas was analyzed. A pathomorphological and immunohistochemical study of the removed formations of the adrenal gland was performed. The article presents rare clinical observations of patients undergoing treatment in the clinic of E.E. Eichwald, North-Western State Medical University named after I.I. Mechnikov, with accidentally detected neoplasms, which were a combination of adenoma and myelolipoma of the adrenal gland. Results.The occurrence of isolated myelolipomas and myxoid adenomas with myelolipoma among incidentalomas was 6.3% and 4.9%, respectively. The average age of patients with adrenal myelolipoma is 54.5 years. The ratio of women to men was 2:1. According to our data, four types of hormonal activity have been identified among myxoid formations of the adrenal gland. Type 1 is associated with Cushing's syndrome, type 2 is associated with Conn's syndrome, type 3 is associated with autonomic cortisol secretion, type 4 has no hormonal activity. Based on the results obtained by high-performance liquid chromatography (HPLC), 11-hydroxylase deficiency was noted, and according to the study of the urine steroid profile by gas chromatography-mass spectrometry (GCMS), the signs of type 2 11-hydroxysteroid dehydrogenase deficiency and an increase in 5-reductase activity in patients with isolated adrenal myelolipoma were obtained. Conclusion.The combination of adenoma with myelolipoma, as well as of adenoma, myelolipoma and cavernous hemangioma is a rare pathology, the diagnosis of which is based on the pathomorphological examination of the removed tumor. Myelolipoma as a part of adrenal adenoma is an incidentaloma, which can be of different sizes and is accompanied by hormonal dysfunction of the cells of the adrenal cortex. An uncertain intrascopic phenotype and hormonal activity of myxoid formations are the decisive factors in favor of surgical intervention.