Medulloblastoma is the most common childhood malignant tumor of the posterior cranial fossa. A comprehensive treatment approach, including surgery, polychemotherapy and radiation therapy, inevitably leads to the development of various long-term consequences from the endocrine system, including a negative impact on bone tissue. Reduced linear growth rate, low adult height and low peak bone mass are well-known long-term effects of treatment. Based on data from Russian and foreign researchers, as well as our own experience, this review presents the endocrine consequences of treatment of malignant tumors of the central nervous system in childhood and adolescence, and summarizes the risk factors for various endocrine system disorders, including a decrease in bone mineral density (BMD). The review pays special attention to the assessment of BMD in a group of patients who have had cancer in childhood and adolescence and have undergone complex treatment, as well as the prevention and treatment of osteoporosis in children.
Laboratory diagnosis of endocrine diseases has undergone many important changes over the past decades, despite the progress of thyroid function immunoassays technologies interferences cannot be completely excluded. These interferences can affect measurement of analyte which leads to misinterpretation and subsequent wrong clinical decisions, the probability of which is about 1%. However, the scale of the problem may be greater due to the lack of awareness to the problem among doctors and the lack of laboratory screening for interfering factors. These factors can be both endogenous and exogenous, bind both to antibodies to the analyte and to the reagent in the test system. The specificity of the immunoassay depends not only on the binding properties of antibodies, the activity of reagent, but also on the composition of the test system and the format of the methodology (non-competitive two-site or “sandwich” and competitive assays). This review provides a description of the main interferences that can affect the measurement of thyroid hormones, in particular thyroid stimulating hormone, free thyroxine and triiodothyronine, calcitonin, and demonstrates clinical cases reported in the literature over the past few years.
Научно-практический центр специализированной медицинской помощи детям им. В.Ф. Войно-Ясенецкого, Москва, Россия ОБОСНОВАНИЕ. Внедрение в клиническую практику стандартизированных протоколов комбинированного лечения онкологических заболеваний неизбежно приводит к развитию отдаленных последствий. Поскольку у лиц, излеченных в детском и подростковом возрасте, ожидаемая продолжительность жизни велика, своевременная диагностика и коррекция отдаленных последствий противоопухолевого лечения имеют даже большее значение, чем острые осложнения химиолучевой терапии. ЦЕЛЬ. Изучить распространенность эндокринных нарушений, оценить распространенность и степень снижения минеральной плотности костной ткани (МПК) у лиц, перенесших комбинированное лечение злокачественных опухолей головного мозга в детском и молодом возрасте. МАТЕРИАЛЫ И МЕТОДЫ. Проведено ретроспективное исследование с участием 59 пациентов (31 мужчина; 28 женщин), перенесших в детском и молодом возрасте оперативное лечение злокачественной опухоли головного мозга с последующей лучевой терапией в объеме краниоспинального облучения в сочетании с полихимиотерапией или без нее. I группу составили 37 пациентов, которым комбинированное лечение проводилось в возрасте от 3 до 16 лет. Во II группу были включены 22 пациента, получившие лечение в возрасте от 16 до 38 лет. РЕЗУЛЬТАТЫ. Недостаточность соматотропного гормона по результатам пробы с инсулиновой гипогликемией выявлена у 48 пациентов (81%), вторичная надпочечниковая недостаточность-у 22 (37%). Большая часть обследованных (33 пациента (56%)) не достигли целевого роста. Лечение рекомбинантным гормоном роста (рГР) получили только 5 человек из I группы. Проведенный корреляционный анализ показал, что возраст на момент лечения-основной фактор, влияющий на конечный рост (r=0,619; p<0,001). Выявлена высокая частота развития гипотиреоза (n=39 (66%)), гипогонадизма (19 женщин; 17 мужчин). По результатам DXA снижение МПК ≤-2,0 SD по Z-критерию в поясничном отделе позвоночника выявлено у 35 из 59 обследованных (59%). МПК у пациентов I группы была значимо ниже по сравнению с пациентами, получившими лечение в более старшем возрасте (p<0,001). Обнаружена умеренная корреляция между МПК в поясничном отделе позвоночника на момент обследования и уровнем эстрадиола в крови у женщин (r=0,596; p<0,05) и тестостерона у мужчин (r=0,472; p<0,05). Выявлена прямая зависимость МПК от возраста на момент заболевания (r=0,781; p<0,01). ЗАКЛЮЧЕНИЕ. Полученные результаты свидетельствуют о необходимости ежегодного и пожизненного наблюдения пациентов после комбинированного лечения злокачественных опухолей головного мозга на предмет выявления отдаленных последствий лечения. Высокая распространенность остеопенических состояний определяет актуальность и необходимость проведения ранней диагностики для предотвращения дальнейшей потери костной массы, снижения прочности кости и риска переломов.
Parathyroid cancer (PTC) is usually sporadic; however, it could be presented as a component of hereditary syndromes. The prevalence of PTC among patients with primary hyperparathyroidism (PHPT) is about 1% cases. The lack of reliable preoperative predictors significantly complicates the diagnosis of PTC. The clinical course is non-specific and in most cases is determined by severe hypercalcemia. The final diagnosis can only be made on the basis of invasive histopathologic features, while an analysis immunohistochemical (IHC) one can be used only as an additional method. Given the rarity the diagnosis of MEN1-related PTC a challenge. We present two clinical cases of patients with PTC and a verified heterozygous mutation in the MEN1 gene. The described cases demonstrate the complexity of morphological diagnosis for PTC, the heterogeneity of clinical manifestations in patients with the MEN1 mutation, as well as the need for timely screening to identify other components of MEN1 syndrome and mutations of the MEN1 gene among first-line relatives.
Summary A 59-year-old male presented with an accidental thyroid mass in 2022. Ultrasound and CT scan showed a nodule 5.2 × 4.9 × 2.8 cm (EU-TIRADS 4) in the right lobe of the thyroid gland. Taking into account the results of the fine needle aspiration biopsy (Bethesda V), intrathyroid localization, and absence of clinical symptoms, a malignant tumor of the thyroid gland was suspected. The patient underwent total thyroidectomy using fluorescence angiography with indocyanine green, and two pairs of intact parathyroid glands were visualized in typical localization. Unexpected histological and immunohistochemistry examinations revealed parathyroid carcinoma. Due to the asymptomatic course of the disease and atypical localization of parathyroid tumor, primary hyperparathyroidism was not suspected before the surgery. The diagnosis of asymptomatic intrathyroid parathyroid cancer is a serious diagnostic challenge for a wide range of specialists. Learning points Parathyroid cancer is a rare disease that may be asymptomatic. Intrathyroidal localization of parathyroid carcinoma is casuistic and challenging for diagnosis, and the treatment strategy is not well defined. Preoperative parathyroid hormone and serum calcium testing are recommended for patients with solid thyroid nodules (Bethesda IV–V).
BACKGROUND: The most of the current studies include patients who are different by the etiology of secondary adrenal insufficiency (SAI), or investigate SAI among other late effects of the radiation therapy. AIMS: To describe the features of SAI and to select the best method of screening SAI in adult patients followed complex treatment of nonpituitary brain tumors in childhood. MATERIALS AND METHODS: It was the retrospective cross-sectional study. 31 patients after the complex treatment of nonpituitary brain tumors in childhood and 20 healthy volunteers were examined. Age and sex ratio were comparable between the groups. Biochemical and clinical blood tests, levels of cortisol, ACTH, DHEA-C were evaluated. The insulin tolerance test (ITT) was performed for all patients and 11 volunteers. RESULTS: The prevalence of SAI by ITT was 45.2%. The levels of basal cortisol (BC) were significantly higher in patients without SAI in comparison with the SAI group and volunteers (505 [340; 650] vs 323 [233; 382] and 372 [263; 489] nmol / l; pSAI- without_SAI=0.001; pwihtout_SAI-healthy = 0.04). The SAI group had DHEA-C significantly lower than in other groups one (3.1 [1.8; 3.4] vs 5.1 [2.5; 6.4] and 6.8 [4.1; 8.9]; рSAI- without_SAI = 0.036; pSAI-healthy = 0.001). ROC analysis showed that BC and DHEA-S can be used as high-quality screening tests for SAI (AUC = 89.3% and 88.3%). The maximum level of cortisol (656 [608-686] vs 634 [548-677]; p = 1) and the time of its increase (45 and 60 min) did not differ during ITT in patients without SAI and volunteers. Side effects: delayed hypoglycemia occurred in 4/14 patients of the SAI group 4090 minutes late of injection 60-80 ml of 40% glucose solution for stopping hypoglycemia in the test. CONCLUSIONS: 45.2% of patients followed craniospinal irradiation had SAI that is characterized by a decrease in DHEA-C levels. A highly normal level of basal cortisol was observed in 45% of patients without SAI. DHEA-C and blood cortisol can be used for SAI screening.
The obesity epidemic has led to the growing number of bariatric operations and the expansion of indications for this operation as the most effective method of treatment, that’s why endocrinologists are increasingly faced the challenge of late complications, including postbariatric hypoglycemia. Postbariatric hypoglycemia is a rare but severe metabolic disorder that occurs months or years after upper gastrointestinal surgery. Postbariatric hypoglycemia can be accompanied by severe clinical symptoms and lead to disability and decreasing of the life’s quality. It is difficult to assess the prevalence of hypoglycemia after bariatric surgery due to the lack of clear diagnostic criteria, often a hidden clinical picture and ignorance of doctors and patients about this complication. Hypoglycemia in this case has postprandial and hyperinsulinemic nature. The mechanisms of development of this complication have recently been actively discussed. The exchange of incretins and dysregulation of insulin secretion are the subject of constant research in this area. Understanding the mechanisms of development of this condition makes it possible to develop optimal methods of diagnosis and treatment. The issues of pathophysiology, basic principles of diagnosis and treatment of post-bariatric hypoglycemia will be considered in this review.
Cushings syndrome accounts for approximately 2030% of endogenous hypercortisolism cases, and adrenal involvement can be either unilateral or bilateral. Cushings 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 Cushings 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.
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