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Introduction: Pancreatic tumors (PT) usually occur after age 60 and almost never in children and young patients (pts). Little is known concerning frequency, morphological and immunohistochemical (IHC) characteristics of PTs in children and young pts. Aim(s): To assess the incidence and prevalence of PT in children and young pts. Materials and methods: five-hundred and forty-eight PTs were verified by IHC, including 358 neuroendocrine neoplasms (pNEN), 53 solid-pseudopapillary (SPT), 129 ductal tumors, and eight other types of PT. 98 (17.8%) of these pts were at the age of 2 to 30 yrs. Results: pNEN were identified in 61.2% (60198) young pts: 32 female, 28 male, at mean age of 21.6 (2-30) yrs. pNENs in 10% pts (6/60) were malignant and sporadic with regional or distant metastases. Four of them at age of 11, 16, 16, 28 yrs with two insulinomas, gastrinoma and somatostatinoma died within 2-5 years because of disease progression. Fifty-one pts presented with hypoglycemia, four - Zollinger-Ellison syndromes (SM). The other cases had nonfunctioning pNENs. Sixteen (26.7%) of 60 pts with pNENs had hereditary SM: 13 (21.7%) - MEN-1, two - VHL, one - tuberous sclerosis. Nine (69.2%) of 13 MEN-1 pts had multiple (2 to 8) pNENs. SPT were identified in 35.7% (35198) young pts and mainly occurred in young females (F/M ratio=1:7.8), at mean age of 17.6 (9-30) years. No SPT had signs of malignancy. Conclusion: Predominant types of pancreatic tumors in children and young pts are pNEN and SPT. Young pts with MEN-1 syndrome often have multiple pNENs. [ABSTRACT FROM AUTHOR]

Introduction: Aim(s): To compare the clinical manifestations of the various causes of hypoglycemia and determine biochemical and instrumental predictors of the severity of hypoglycemia. Materials and methods: Thirty patients with complaints similar to hypoglycemia referred to our clinic over the past 10 years. Twenty-four of them - 18 women, six men, the average age - 43 years. A 72-hour fasting test and imaging methods were performed on all patients. Results: In only 15 patients was insulinoma confirmed (Group A). The remaining 15 patients (Group B) revealed other causes of hypoglycemia (gastrointestinal diseases - six patients, functional hypoglycemia - three liver failure, two anorexia nervosa, one affective disorders, one inadequate treatment of diabetes mellitus. There were no differences by gender and age between groups. Group A patients were more likely to have convulsions, coma, difficulty awaking in the morning (p<0.05). The duration of the fasting test was: in Group A - less than 24 hours (from 4 to 22 hours), in Group B -72 hours. In Group A, the test was stopped because of severe hypoglycemia symptoms (glucose-1.46 mmol / I), and in Group B - because there were no symptoms and no hypoglycemia for 72 hours. We did not find a reliable correlation between the test duration and the tumor size or C-peptide. Conclusion: In patients with insulinomas, symptoms of hypoglycemia were more severe. The lack of correlation between clinical manifestations and hypoglycemia and characteristics of the tumor were probably associated with a small number of patients. [ABSTRACT FROM AUTHOR]

Introduction: Defining localization and potential ACTH-producing extrapituitary neuroendocrine neoplasms (ACTH-epNEN) is important for clinical practice. Aim(s): To assess CK19 expression in ACTH-epNEN of different localization. Materials and methods: CK19 (clone RCK108) and Ki-67 (clone MIB-1) expression were evaluated immunohistochemically (IHC) in 37 ACTH-epNEN specimens: 19 lung (L-NEN) (51.3%); eight thymus (T-NEN) and two mediastinal (M-NEN) (27.2%); six gastrointestinal (GI): three pancreatic (Pn-NEN), one ampulla Vater's (AV-NEN), one appendical (A-NEN), one rectal (R-NEN) (16.2%); two medullary thyroid carcinomas (MTC) (5.4%); in 13 men and 25 women, at mean age 42.1 (16-67 yrs). Results: Clinical presentation of ACTH-ectopic syndrome was diagnosed in 30 (81.1%) patients (pts) at early stage, in three (8.1%) pts at late stage of the disease (Pn-NEN, AV-NEN, R-NEN). Four tumors (10.8%) were asymptomatic (2 L-NEN, Pn-NEN, M-NEN). Twenty of 37 (54.1%) ACTH-epNEN had CK19 expression: in 9/10 T-NEN and M-NEN (90%), 2/2 MTC (100%), 4/6 GI-NEN (66.7%) and 5/19 L-NEN (26.3%). All CK 19-positive ACTH-epNENs of GI and thyroid, mediastinum, thymus and three tumors of the lung were malignant (with metastases or recurrences). We got a strong correlation between CK19 expression in ACTH-epNE and an increased level of Ki-67 (Spearman r=0,75, p<0.001). Conclusion: CK19 expression in ACTH-epNEN is an important criteria for malignancy potential. Especially for L-NEN, this can be used to predict recurrence and metastases. [ABSTRACT FROM AUTHOR]

Introduction: A 39 y.o. man with renal insufficiency (GFR 34 ml/min) due to autosomal-dominant polycystic disease (in the liver and kidney) and ACTH-ectopy (AES). Aim(s): Materials and methods: Results: The first signs of hypercortisolism were only high blood pressure. Suspected kidney disease. Hypercortisolism symptoms gradually progressed and steroid diabetes mellitus and osteopenia diagnosed. AES suspected (HDDST - negative, 23.00 ACTH -20.2 pmol / 1 (N<5), pituitary MRI-normal, CT-scan -- thymic lesion found). The potassium level was on upper limit of normal probably because of kidney disease. BIPSS with desmopressin stimulation confirmed AES (Pit/Perif gradient of ACTH<3). CgA and tumormarkers were in normal ranges. Thymectomy performed. Histologically it was thymic cyst without ACTH-secreting cells. Ketokonazol was prescribed. Colonoscopy, pituitary MRI, CT chest and abdomen, bronchoscopy revealed no tumor six months thereafter. Because of hypercortisolism progression unilateral adrenalectomy was done. Immunohistochemically the adrenal medulla cells were positive for synaptophysin, CgA and ACTH. Annual examination failed to reveal any tumor. He is on ketokonazol (600 mg/d) now. No signs of hypercortisolism and no diabetes melitus. Conclusion: The source of AES is still unknown. But the hypercortisolism is well-controlled by ketokonazol. [ABSTRACT FROM AUTHOR]

The understanding of the nature of catecholamine-secreting tumors has changed significantly in recent years, affecting terminology and classification. Phaeochromocytoma/paraganglioma (PCC/PG) is a rare neuroendocrine tumor from chromaffin tissue that produces and secretes catecholamines. The incidence of PCC/PG is relatively low, with 2-8 cases per 1 million population per year; among patients with arterial hypertension, their prevalence is 0.2-0.6%. However, delayed diagnosis of PCC/PG is associated with a high risk of cardiovascular complications and a high mortality rate. The consensus presents the clinical manifestations of the disease with an emphasis on the course of arterial hypertension as the most common symptom in PCC/PG; modern ideas about the features of diagnosis, aspects of preoperative preparation, treatment, and follow-up of patients with PCC/PG are considered.

Introduction: The development of new highly accurate, inexpensive and accessible methods for the detection of lower-extremity peripheral artery disease (LE-PAD) in diabetic patients is required. The aim of this study was to evaluate the accuracy of a new incoherent optical fluctuation flowmetry (IOFF) method in detecting legs with hemodynamically significant stenoses compared to ankle brachial index (ABI) and transcutaneous oximetry (TcPO2) in patients with diabetes mellitus (DM)., Materials and Methods: Patients were recruited into 2 groups. Group 1 included patients with DM without LE-PAD and/or diabetic foot syndrome; Group 2 included patients with DM and LE-PAD. All patients underwent the following measurements: ultrasound (reference method), ABI, TcPO2, and the new IOFF method., Results: The new IOFF method showed a sensitivity of 79.5% and a specificity of 89.8% in detecting limbs with hemodynamically significant stenosis (AUC 0.890, CI 0.822-0.957). TcpO2 allows the diagnosis of LE-PAD with 69.2% sensitivity and 86.2% specificity (AUC 0.817, CI 0.723-0.911). Using a standard ABI cut-off of less than 0.9, the sensitivity and specificity for this parameter were 34.5% and 89.7%, respectively. Increasing the diagnostic cut-off of the ABI on the study group to 0.99 improved sensitivity to 84.6% and specificity to 78% (AUC,0.824 CI 0.732-0.915)., Conclusions: The new IOFF technique has demonstrated high sensitivity and specificity in the detection of LE-PAD in patients with DM. The high accuracy, rapid measurement, and potential availability suggest that the new IOFF method has a high potential for clinical application in the detection of PAD., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

(1) Background: To date, there are no studies evaluating the ability of the incoherent optical fluctuation flowmetry (IOFF) method to assess foot tissue perfusion. The aim of this study was to evaluate the correlation between perfusion values measured by IOFF and TcPO2 in patients with diabetes-related lower-extremity complications. (2) Methods: This was an observational, cross-sectional, two-center study. Diabetic patients with peripheral artery disease and/or diabetic foot ulcers were studied (n = 27, examinations were carried out on 54 legs). Perfusion in the foot tissues was assessed using TcPO2 (reference standard for this study) and the IOFF method. (3) Results: High correlation coefficients of all perfusion parameters measured by IOFF with TcPO2 (Rs 0.7 to 0.76) were shown. The study demonstrated that the IOFF method allows, with a sensitivity of 85.7% and a specificity of 90.0%, the identification of patients with a critical decrease in TcPO2 < 20 mmHg. (4) Conclusions: The high correlation of IOFF parameters with TcPO2 and the moderately high sensitivity and specificity in detecting patients with severe ischemia of foot tissues shows the promise of the method for assessing a tissue perfusion in patients with diabetes-related lower-extremity complications.

Objective: To analyze 60 cases of solid pseudopapillary tumors (SPTs) of the pancreas, to reveal their most characteristic clinical and morphological features, and to study their possible histogenesis., Material and Methods: Sixty cases of SPTs of the pancreas underwent clinical, morphological, and immunohistochemical (IHC) examinations; a comparison group consisted of 86 pancreatic tumors of other histogenesis., Results: It has been shown for the first time that SPTs are characterized by the nuclear expression of claudin 3 and the cytoplasmic expression of claudin 7. It has been also ascertained that the aberrant perinuclear (dot-like) expression of CD99 is a unique feature of these tumors., Conclusion: SPTs of the pancreas are distinguished by a diversity of clinical manifestations and morphological features, but have a unique immunophenotype, which can differentiate them from other types of pancreatic tumors.

Insulinoma in most cases is monohormonal orthoendocrine tumor. This fact limits its searching area by a pancreas. However the insulinoma's clinical picture can be caused by other pathology. Therefore the main task of visual the tumor but also to use all the possibilities of each method for defining type of surgery and avoiding such errors and complications, as pancreatorrhage, damage of bile or pancreatic duct, necrosis of duodenal wall, etc. On the basis of our own experience of endoscopic ultrasound for 61 patients with 66 insulinomas, we have allocated the basic variants of insulinoma ultrasound pictures: hypoechoic, heteroechoic, isoechoic tumor, and also foci without a trustworthy contour. On the basis of the analysis of our own errors we have essentially added the technique, having used multiplane pancreas scanning and biplane tumor scanning. Important points in EUS algorithm are also the following items: detecting tumor relationship to pancreatic surface, distance from the tumor to the ducts, gastrointestinal wall and vessels--for definition of possibility of the tumor enucleation; careful scanning of pancreas even after detecting one tumor--for an exception of multiple tumors, especially at MEN-I; careful scanning of a contour of the gland for an exception of parapancreatic insulinomas; estimation of vascularization not only of the detected tumor and suspicious foci, but also of all the gland--for detection of hypervascularized isoechoic tumors; revealing and the description of focal changes, especially more homoechogeneous in comparison with surrounding parenchyma-- for selection patients for examination by other methods; scanning of zones of possible metastasis. Examination of patients with organic hyperinsulinism demands combined application of two or more techniques. Use of all modern possibilities of endosonography can put this technique on one of the first places in the diagnosis of insulinomas.

The authors of the article analyze more than 30-year experience of surgical treatment of adrenal gland diseases, offering a detailed description of clinical and morphological features of the main nosological forms such as Itsenko-Cushing disease and syndrome, primary hyperaldosteronism, pheochromocytoma, incidentaloma, and malignant adrenal tumors. The paper contains basic diagnostic criteria, indications to operative treatment, and extents of surgery for each of the diseases considered, as well as comparative analysis of peculiarities of conventional ("open"), and endoscopic adrenal gland surgeries. The article demonstrates significance of various immunohistochemical markers in differentiation between malignant and benign adrenal tumors.