RESUMEN
A clinical case of a man 66 y.o. who was diagnosed with hormone-inactive pituitary macroadenoma complicated by corneal erosion and partial atrophy of the optic nerve of the left eye due to exophthalmos. The increase in prolactin level was regarded due to a «stalk-effect¼. The patient underwent a transnasal pituitary adenomectomy with subsequent regression of symptoms. After 4 years, against the background of a new coronavirus infection, increasing general weakness, headaches, a crisis increase in blood pressure and tachycardia attacks appeared. Computed tomography (CT) accidentally revealed an adrenal incidentaloma, in laboratory tests - hypercortisolism, elevated ACTH levels, hypokalemia, hyperglycemia, increased levels of metanephrine and normetanephrine. The patient developed acute steroid psychosis, after which an adrenalectomy with a tumor was performed, a pheochromocytoma was histologically confirmed. After surgery, there was a regression of symptoms, the development of adrenal insufficiency with reduced levels of ACTH and cortisol. Upon further examination, a polynodose euthyroid goiter was established, the biopsy of the nodes - Hashimoto's thyroiditis (Bethesda II). Meanwhile, primary hyperparathyroidism was detected. According to ultrasound, scintigraphy with Ts99m-Technetril and CT revealed an increase of left parathyroid gland. A bilateral revision of the neck, removal of the right upper and left upper parathyroid adenomas were performed. In the postoperative period, the levels of calcium and parathyroid hormone were normalized. Given the presence of a combination of multiple tumors of the endocrine system (primary hyperparathyroidism, corticotropin-producing pheochromocytoma, hormone-inactive pituitary macroadenoma, polynodose euthyroid goiter), the MEN1 syndrome was clinically established. The study of 2 and 10 exons of the MEN1 gene revealed no mutations, which does not exclude the presence of a hereditary syndrome. The patient continues observation. In the available literature in Russian and English languages the case of ACTH pheochromocytoma as part of the MEN type 1 syndrome have not been found. Therefore, we consider the presented case to be the first one.
Asunto(s)
Neoplasias de las Glándulas Suprarrenales , Hiperparatiroidismo Primario , Neoplasia Endocrina Múltiple Tipo 1 , Feocromocitoma , Neoplasias Hipofisarias , Masculino , Humanos , Feocromocitoma/complicaciones , Feocromocitoma/cirugía , Hormona Adrenocorticotrópica , Neoplasias de las Glándulas Suprarrenales/complicaciones , Neoplasias de las Glándulas Suprarrenales/cirugía , Neoplasias de las Glándulas Suprarrenales/diagnósticoRESUMEN
BACKGROUND: Primary hyperparathyroidism (PHPT) is a common endocrine disorder caused by a parathyroid tumor or hyperplasia, which is often accompanied with quality of life (QoL) impairment. A parathyroidectomy (PTX) is the preferred standard treatment for PHPT patients. In this single center study we aimed to evaluate the impact of PHPT on patient's QoL and identify QoL changes at early and long-term follow-up after surgery. METHODS: All the patients underwent routine PTX with the removal of the suspected hyperparathyroid gland(s). Patients filled out generic QoL questionnaire RAND SF-36, specific questionnaire PHPQoL and specific symptom assessment questionnaire PAS upon admission to the hospital before surgery, at 3 months, 12 months and 24 months after surgery. RESULTS: A total of 92 patients with PHPT (median age was 56 years, 95.7% females) were included in the study. Before PTX patient's QoL by SF-36 scores was significantly lower as compared to healthy controls (p < 0.01). Almost 40% of patients had poor or very poor QoL. The most frequent symptoms by PAS before surgery were as follows: tiredness (97.8% of patients), weakness (94.6%), forgetfulness (94.6%), mood changes (90%), feeling "blue"/depression (88%), joint pains (83.3%), headaches (80.2%), constant irritability (77.2%), bone pains (75%), thirst (70.7%) and trouble getting out of a chair (67.4%). The half of the patients had moderate-to-severe (≥ 40 scores) tiredness, weakness, joint pains, forgetfulness, as well as mood changes. Post-operative QoL changes were analysed in the group of 72 patients. After surgery there was significant improvement in QoL by all scales of SF-36 questionnaire, excluding bodily pain, and the PHPQoL total score (GEE, p < 0.01) as compared with their values before surgery. Also severity of tiredness, mood changes, weakness and forgetfulness significantly decreased after surgery as compared to their baseline values (GEE, p < 0.05). Decreased mental component of QoL by PHPQoL (OR = 0.927, 95%CI = 0.874-0.984, p = 0.013) predicted improved QoL after surgery. CONCLUSIONS: Patients with PHPT demonstrated significantly impaired QoL in physical, psychological and social functioning as well experienced a wide profile of common PHPT symptoms. Successful PTX was accompanied with remarkable QoL improvement and decrease in subjective symptoms for at least 24 months after surgery.
Asunto(s)
Hiperparatiroidismo Primario , Neoplasias de las Paratiroides , Femenino , Humanos , Persona de Mediana Edad , Masculino , Calidad de Vida , Hiperparatiroidismo Primario/complicaciones , Hiperparatiroidismo Primario/cirugía , Paratiroidectomía , Neoplasias de las Paratiroides/cirugía , EmocionesRESUMEN
Currently, all pheochromocytoma/paraganglioma (PPGLs) are considered malignant due to metastatic potential. Consequently, PPGLs are divided into «metastatic¼ and «non-metastatic¼. Metastatic PPGLs can be with synchronous metastasis (metastases appear simultaneously with the identified primary tumor) or metachronous (metastases develop after removal of the primary tumor). The term metastatic PPGLs is not used in the presence of tumor invasion into surrounding organs and tissues, without the presence of distant metastases of lymphogenic or hematogenic origin. It is generally believed that about 10% of pheochromocytomas and about 40% of sympathetic paragangliomas have metastatic potential. On average, the prevalence of PPGLs with the presence of metastases is 15-20%. Risk factors for metastatic PPGLs are widely discussed in the literature, the most significant of which are groups of clinical, morphological and genetic characteristics. The review presents a discussion of such risk factors for metastatic PPGLs as age, localization and type of hormonal secretion of the tumor, the size and growth pattern of the adrenal lesion, the presence of necrosis and invasion into the vessels, the tumor capsule surrounding adipose tissue, high cellular and mitotic activity, Ki-67 index, expression of chromogranin B and S100 protein, the presence of genetic mutations of three main clusters (pseudohypoxia, kinase signaling and Wnt signaling).Over the past two decades, a number of authors have proposed various predictor factors and scales for assessing a probability of metastatic PPGLs. The review contains detailed description and comparison of sensitivity and specificity of such predictor scales as PASS, GAPP, M-GAPP, ASES and COPPS.
Asunto(s)
Neoplasias de las Glándulas Suprarrenales , Paraganglioma , Feocromocitoma , Humanos , Feocromocitoma/patología , Feocromocitoma/epidemiología , Neoplasias de las Glándulas Suprarrenales/patología , Neoplasias de las Glándulas Suprarrenales/epidemiología , Factores de Riesgo , Paraganglioma/patología , Paraganglioma/genética , Paraganglioma/epidemiología , Metástasis de la NeoplasiaRESUMEN
BACKGROUND: In most cases adrenal tumours are detected by accident while performing medical imaging tests for other diseases. These findings are treated as adrenal incidentaloma. Prevalence of incidentalomas detected on CT scans is up to 4%. According to different authors, 4-12% of all adrenal tumours are adrenocortical carcinomas. As for today, the most significant medical imaging technique is CT scan with bolus IV injection of contrast agent and assessment of tumour's density. The analysis of the results of CT imaging in 67 patients with ACC was carried out according to a single protocol. The main signs characteristic of this disease are described. It is very important to evaluate typical signs of ACC on CT scans for risk assessment of ACC before surgical treatment. If malignant tumour is suspected during preoperative examination, it is extremely important to choose the right surgical treatment strategy. AIM: To evaluate the significance of CT as the main method of preoperative diagnosis in patients with malignant tumors of the adrenal cortex. Studying CT semiotics of adrenocortical cancer in a large group of patients using a single standard imaging protocol. Find the main radiological symptoms characteristic of adrenocortical cancerMATERIALS AND METHODS: Here are the results of retrospective study of CT scans performed on 67 patients with adrenocortical carcinoma who received treatment in the Department of Endocrine Surgery of Saint-Petersburg State University N.I. Pirogov Clinic of High Medical Technologies during 2012-2020. The diagnostic significance of CT in patients with ACC was assessed. RESULTS: The most common features of ACC: tumour heterogeneity (84.3%), tumour's size 3-9 cm (75%), signs of invasion into surrounding structures (10%), pre-contrast density above +30 HU (75%), absolute contrast washout less than 60% (68.8%), relative contrast washout less than 40% (64.6%)CONCLUSION: CT scan with IV contrast was not able to show any definitive pathognomonic signs of ACC. Nevertheless, CT scan should be performed in all patients with suspected (or confirmed using other medical imaging technique) adrenal tumour according to standard protocol. Bolus injection of contrast agent should be performed in all patients with tumour's pre-contrast density above +5 HU.
Asunto(s)
Neoplasias de la Corteza Suprarrenal , Neoplasias de las Glándulas Suprarrenales , Carcinoma Corticosuprarrenal , Neoplasias de las Glándulas Suprarrenales/diagnóstico por imagen , Carcinoma Corticosuprarrenal/diagnóstico por imagen , Carcinoma Corticosuprarrenal/terapia , Medios de Contraste , Humanos , Estudios Retrospectivos , Tomografía Computarizada por Rayos X/métodosRESUMEN
BACKGROUND: Precise localization of abnormal parathyroid glands is important for a successful surgery for primary hyperparathyroidism (PHPT). While a large number of patients can be successfully treated with the focused parathyroidectomy, there is a considerable rate of the persistent PHPT mostly because of undetected multiglandular disease (MGD). AIM: The aim of the study was to evaluate the meaning of preoperative visualization data for planning the surgery for patients with PHPT. MATERIALS AND METHODS: The study was conducted at SPBU Hospital in 2017-2018. 810 patients who underwent a primary surgery for PHPT were included in the study. Preoperative imaging results were investigated and multivariative logistic regressions were calculated to assess the predictive values of preoperative data. The rate of cases with persistent disease and cases with MGD were compared between patients with different results of preoperative data. RESULTS: Age, sex, body mass index, negative results of preoperative US, MIBI and 4D CT were not independently associated with the higher risk of multiglandular disease. The larger number of performed preoperative visualization studies were associated with the higher risk of persistence. 37% cases of MGD were not identified preoperatively. There were 7 cases with previously unsuspected second adenomas found only due to bilateral neck exploration. CONCLUSION: Any combination of preoperative visualization modalities was not able to rule out the MGD reliably. Efficacy of surgical treatment was not associated with the higher number of preoperative studies. Bilateral neck exploration may decrease the rate of the persistent hyperparathyroidism improving the identification of multiglandular disease.
Asunto(s)
Hiperparatiroidismo Primario , Humanos , Tecnecio Tc 99m Sestamibi , Glándulas Paratiroides , Paratiroidectomía/métodos , Tomografía Computarizada Cuatridimensional/métodosRESUMEN
The widespread introduction of genetic testing in recent years has made it possible to determine that more than a third of cases of pheochromocytomas and paragangliomas (PPPGs) are caused by germline mutations. Despite the variety of catecholamine-producing tumors manifestations, there is a sufficient number of clinical and laboratory landmarks that suggest a hereditary genesis of the disease and even a specific syndrome. These include a family history, age of patient, presence of concomitant conditions, and symptoms of the disease. Considering that each of the mutations is associated with certain diseases that often determine tactics of treatment and examination of a patient, e.g. high risk of various malignancies. Awareness of the practitioner on the peculiarities of the course of family forms of PPPGs will allow improving the tactics of managing these patients.The article provides up-to-date information on the prevalence of hereditary PPPGs. The modern views on the pathogenesis of the disease induced by different mutations are presented. The main hereditary syndromes associated with PPPGs are described, including multiple endocrine neoplasia syndrome type 2A and 2B, type 1 neurofibromatosis, von Hippel-Lindau syndrome, hereditary paraganglioma syndrome, as well as clinical and laboratory features of the tumor in these conditions. The main positions on the necessity of genetic screening in patients with PPPGs are given.
Asunto(s)
Neoplasias de las Glándulas Suprarrenales , Neoplasia Endocrina Múltiple Tipo 2a , Paraganglioma , Feocromocitoma , Enfermedad de von Hippel-Lindau , Neoplasias de las Glándulas Suprarrenales/diagnóstico , Neoplasias de las Glándulas Suprarrenales/genética , Humanos , Neoplasia Endocrina Múltiple Tipo 2a/diagnóstico , Neoplasia Endocrina Múltiple Tipo 2a/genética , Paraganglioma/diagnóstico , Paraganglioma/genética , Feocromocitoma/genética , Enfermedad de von Hippel-Lindau/diagnóstico , Enfermedad de von Hippel-Lindau/genéticaRESUMEN
Radioprotective therapeutic effect of lyophilized extract ofAmaranthus retroflexus on the resistance of the albino laboratory male mice exposed to X-ray radiation at a one-time dose of LD(100/30) is studied. It was established that a 30-day use of a lyophilized extract of the vegetative parts of A. retroflexus at a dose of 50 mg/kg after X-ray exposure increases the survival rate of mice by 80%, enhances the recovery in the radiation period of the number of leukocytes, neutrophils, lymphocytes in the peripheral blood of the animals and the normalization of the intensity of peroxide oxidation of lipids in liver cells. Dose modification factor on the 5th day after irradiation, when using a lyophilized extract, was 1.5 according to the criterion of survival of mice.