ABSTRACT
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.
Subject(s)
Adrenal Cortex Neoplasms , Adrenal Gland Neoplasms , Adrenocortical Carcinoma , Adrenal Gland Neoplasms/diagnostic imaging , Adrenocortical Carcinoma/diagnostic imaging , Adrenocortical Carcinoma/therapy , Contrast Media , Humans , Retrospective Studies , Tomography, X-Ray Computed/methodsABSTRACT
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.
Subject(s)
Adrenal Gland Neoplasms , Multiple Endocrine Neoplasia Type 2a , Paraganglioma , Pheochromocytoma , von Hippel-Lindau Disease , Adrenal Gland Neoplasms/diagnosis , Adrenal Gland Neoplasms/genetics , Humans , Multiple Endocrine Neoplasia Type 2a/diagnosis , Multiple Endocrine Neoplasia Type 2a/genetics , Paraganglioma/diagnosis , Paraganglioma/genetics , Pheochromocytoma/genetics , von Hippel-Lindau Disease/diagnosis , von Hippel-Lindau Disease/geneticsABSTRACT
The article described a female patient with high-grade arterial hypertension diagnosed at her young age. Congenital abdominal aortic hyperplasia and conformity with criteria for Takayasu arteritis were diagnosed at 10 years following the clinical manifestation.
Subject(s)
Takayasu Arteritis , Female , Humans , HypertensionABSTRACT
Urinary steroid profiling (USP) was studied using high-performance liquid chromatography (HPLC) and gas chromatography-mass spectrometry (GC-MS) methods in 108 patients with adrenocortical adenoma (ACA) and in 31 patients with adrenocortical carcinoma (ACC). Thirteen ACC and Cushing's syndrome (ACC-CS) patients had two types of USP as well as 18 ACC patients without hypercortisolism. These four types differed by androgen and glucocorticoid secretion of the adrenal cortex. Fifteen main ACC features were observed by GC-MS. Urinary excretion of dehydroepiandrosterone (DHEA) was increased in 67.7 % of ACC patients and tetrahydro-11-deoxycortisol (THS) in 74.2 %. By combination of the following parameters: THS >900 µg/24 h and/or DHEA >1500 µg/24 h with ratios of 3α,16,20-pregnentriol/3ß,16,20-pregnentriol (3α,16,20dP3/3ß,16,20dP3) less than 6.0 and 3α,17,20dP3/3ß,17,20dP3 less than 9.0 and the detection of "non-classical" 5-en-pregnens, not found in ACA and healthy persons, 100 % sensitivity and specificity of ACC and ACA differential diagnosis were achieved. Features of 21-hydroxylase and 11ß-hydroxylase deficiency were observed by GC-MS in 32.2 and 61.3 % of the ACC patients, respectively. Additional features for ACC-CS diagnostic were increased urinary excretion of 6ß-hydroxycortisol, 18-hydroxycorticosterone, the sum (UFF + UFE) obtained by HPLC, tetrahydrocorticosterone, and the sum (THF + THE + allo-THF) obtained by GC-MS.
Subject(s)
Adrenal Cortex Neoplasms/urine , Adrenocortical Adenoma/urine , Adrenocortical Carcinoma/urine , Chromatography, High Pressure Liquid/methods , Gas Chromatography-Mass Spectrometry/methods , Steroids/urine , Adrenal Cortex Neoplasms/diagnosis , Adrenocortical Adenoma/diagnosis , Adrenocortical Carcinoma/diagnosis , Adult , Cortodoxone/analogs & derivatives , Cortodoxone/urine , Cushing Syndrome/urine , Dehydroepiandrosterone/urine , Diagnosis, Differential , Female , Humans , Male , Middle Aged , Steroid 11-beta-Hydroxylase/metabolism , Steroid 21-Hydroxylase/metabolism , Young AdultABSTRACT
J. Raven's (1992) classification was used in the treatment of patients with multiple fractures of the face associated with contusions, brain concussions, and injuries to the skull vault and base. According to this classification, all injuries of the cerebral and facial skull are classified as types I, II, and subtype Ia. Use of this classification became possible due to introduction of computer-aided tomography. A clinical example of a possible surgical correction of the orbit in a patient with type II fracture is presented.