Draft of the clinical practice guidelines “Adrenal incidentaloma”

The wider application and technical improvement of abdominal imaging procedures in recent years has led to an increasingly frequent detection of adrenal gland masses — adrenal incidentaloma, which have become a common clinical problem and need to be investigated for evidence of hormonal hypersecretion and/or malignancy. Clinical guidelines are the main working tool of a practicing physician. Laconic, structured information about a specific nosology, methods of its diagnosis and treatment, based on the principles of evidence-based medicine, make it possible to give answers to questions in a short time, to achieve maximum efficiency and personalization of treatment. These clinical guidelines include data on the prevalence, etiology, radiological features and assessment of hormonal status of adrenal incidentalomas. In addition, this clinical practice guideline provides information on indications for surgery, postoperative rehabilitation and follow-up.

Clinical and genetic heterogeneity of micronodular adrenal hyperplasia

Micronodular adrenal hyperplasia is a rare cause of ACTH-independent Cushing syndrome. It can be divided into two entities: primary pigmented nodular adrenocortical disease (PPNAD) and non-pigmented micronodular adrenocortical disease, among which familial and sporadic forms are distinguished. The most common is the genetically determined familial form PPNAD, as one of the components of Carney complex. The vast majority of patients have identifiable pathogenic variants in the PRKAR1A gene. In addition to the PRKAR1A gene mutations, inactivating mutations in the genes encoding phosphodiesterases (PDE11A4 and PDE8B), as well as PRKACA gene amplification, have been described in individuals with isolated forms. Despite the relative antiquity of the description of micronodular adrenal hyperplasia and the Carney comlex, a detailed study of pathophysiological mechanisms, genetic and clinical aspects of this pathology, nowadays, clinicians continue to face «atypical» cases. Thus, the nature of this disease is not well understood and requires further research. This review presents the accumulated data on micronodular adrenal hyperplasia, genetics aspects, and also describes 2 unique clinical cases of isolated PPNAD with unilateral adrenalectomy results.

Primary adrenal leiomyosarcoma: clinical case and literature review

Adrenal leiomyosarcoma — rare mesenchymal tumor, which is diagnosed most often after it reaches a large size. Primary adrenal leiomyosarcoma is extremely rare: in the English-language literature, only 45 cases were previously reported. We report the case of a 46-year-old man with the formation of the right adrenal gland measuring 12x8x7,5 cm. The patient underwent right-sided adrenalectomy, nephrectomy, and plastic of the inferior vena cava. The phenotype was confirmed by light microscopy and immunohistochemistry. Microscopic examination of the tumor is represented by fusiform and polymorphic cells that form multidirectional bundle structures with a high rate of mitosis. Immunohistochemically, tumor cells were positive for smooth muscle actin, CD-34, CD-117.

Surgical treatment of patient with combination of amiodaron-induced thyrotoxic and esophagesl cancer

The article presents the successful experience of surgical treatment of a patient with a malignant tumor of the esophagus, which was detected in a patient with amiodarone-induced thyrotoxicosis. Thyroidectomy made it possible to quickly eliminate thyrotoxicosis and timely perform radical surgery for esophageal cancer.

Papillary thyroid microcarcinoma: distinct form or cancer growth stage?

Background. Over the past decades an increase in the incidence of papillary thyroid microcarcinoma (PTMC) has been observed throughout the world owing to the improved diagnostics. There are many different opinions about the aggressiveness degree of this group of tumors, as well as about the tactics of managing patients with PTMC.Aim of the study is the identification of the prognostic factors responsible for the features of the clinical course, including the more aggressive one.Materials and methods. A study was carried out with a detailed analysis of a group of patients with papillary thyroid cancer ≤1 cm in size and the existing clinical data of regional and distant metastases. All patients underwent thyroidectomy with bilateral central cervical lymph node dissection. Factors such as gender, patient age, bilaterality, extrathyroid extension, the presence or absence of a capsule around the tumor node, the absence or presence of metastases in the central part were assessed. In 26.6% histological examination revealed metastatic lesions of the central group lymph nodes. Latent metastases were detected in 24.2% of women and 43% of men, in 36.7% of patients <55 and in 14.3% of patients ≥ 55 years, in 29.5% with the absence of the node capsule and in 19.3% with encapsulated tumors, in 48.1% with multicentric growth and in 19.5% with a solitary neoplasm, in 21.7% with a tumor size ≤0.5 cm and in 27.9% with a node of 0.6–1 cm, in 24% with the absence of invasion of the thyroid capsule and in 31% with the presence of extrathyroid invasion, in 21% of patients with typical, in 26% with follicular and 43% with mixed papillary cancer. 95 patients received radioiodine therapy. No additional metastases were found in them.Results. When conducting univariate analysis, the main signs influencing the development of metastases in the central zone were age up to 55 years (p = 0.009, χ2 = 6.919) and multicentric neoplasm (p = 0.004, χ2 = 8.530); in multivariate analysis, similarly, age younger 55 years (p = 0.000, Exp B = 0.011, CI 95.0% 0.001–0.106) and multifocality (p = 0.027, Exp B = 2.686, CI 95.0% 1.119–6.448).Conclusion. PTMC is not a separate group or tumor morphotype, and the determination of treatment tactics for this group of patients should be based not only on the size of the tumor, but on the clinical and biological parameters of the tumor.

Extralaryngeal branching of the recurrent laryngeal nerve. Autopsy case series

Background. The thesis «thyroid surgery is the surgery of the recurrent laryngeal nerve», which was defined in the XX ­century, remains relevant to this day. Thus, despite the use of modern scientific and technological achievements, vocal cord paresis is diagnosed on average in 9.8% patients after thyroid and parathyroid surgery.According to many authors, the main problem which a surgeon encounters is a difficult and sometimes individual anatomy of the recurrent laryngeal nerve. For example, in one study authors identified 28 variants of relationships between the ­recurrent laryngeal nerve and the inferior thyroid artery. In a recent meta-analysis, it was noted that the frequency of extralaryngeal branching remains underestimated and reaches 73% of cases. Anatomical variants of the recurrent laryngeal nerve or its thin branches, which have not been noticed in time, can lead to inevitable consequences — to nerve injury and a laryngeal dysfunctions, and if anatomical features are not detected on both sides — to a tragedy.Aim. The aim of this study is to determine anatomical variants of the extralaryngeal branches of the recurrent laryngeal nerve and their frequency in autopsy case series.Materials and methods. 46 (100%) recurrent laryngeal nerves were dissected in 23 cadavers. Their path was traced, their relationships with the inferior thyroid artery were determined, and the extralaryngeal branches of the recurrent laryngeal nerve were identified.Results. Few extralaryngeal branches of the recurrent laryngeal nerve were identified during dissection: tracheoesophageal branches, laryngeal branches, aberrant branches. Tracheoesophageal branches were determined in all cadavers. In 37 (80.4%) cases the recurrent laryngeal nerve had got two or more extralaryngeal laryngeal branches. In 6 (13.0%) cases aberrant branches were presented.The inferior thyroid artery was detected in 42 (91.3%) cases. The vessel was in close location with the recurrent laryngeal nerve, forming a neuro-arterial chiasm. In 39.1% of cases the recurrent laryngeal nerve was located between the branches of the inferior thyroid artery, in 39.1% of cases — deeper than the artery, and in 10.9% of cases — more superficial than the artery.Conclusion. The variable anatomical configuration of the recurrent laryngeal nerve inevitably causes difficulties during intraoperative nerve dissection. Tardy identification of these anatomical features can lead to the injury of nerves or its branches, which determines the amount of postoperative vocal cord paresis.

The choice of feminizing genitoplasty tactic in girls with congenital adrenal hyperplasya

The choice of tactics for feminizing plastic surgery (one- or two-stage) remains an actually issue. To operate early, in the first months of a child’s life, when endocrinological stabilization is achieved, or to postpone the intervention until puberty? Despite the large number of approaches to surgical treatment, still not all results can be recognized as positive. There are complications that affect the quality of life of patients, not only of a surgical but also of a psychological nature. The advantages of one or another approach are currently impossible to prove, since this will require a prospective multicenter multidisciplinary study of long-term results with each of the approaches. Therefore, pediatric surgeons for a long time in the choice of tactics will be based on accumulated experience and logical reasoning. Given the extensive experience of multidisciplinary rehabilitation, the positive long-term results of two-stage feminization remains the preferred tactic for surgical treatment of girls with CAH. The increased risk of complications from the genitourinary system in the form of infections of the urinary tract, hematocolpos and hematometra in girls with a narrow common urogenital canal, which is inherent in virilization of 3, 4 and 5 degrees according to Prader, can be considered as a reason for an early one-stage feminizing plasty.

Syndrome of inappropriate secretion of antidiuretic hormone in the practice of endocrinologist

Hyponatremia is the most common disorder of water and electrolyte balance encountered in clinical practice. Conditions associated with hyponatremia require hospitalization in 15–20% of cases. Hyponatremia is a predictor of poor outcome in a wide range of diseases and therefore requires an interdisciplinary approach. This problem leads to an increase in complications and the length of hospital stay and mortality. The review focuses on the syndrome of inappropriate secretion of antidiuretic hormone (SIADH), which accounts for approximately one third of all cases of hyponatremia and is more common in endocrinology than other fluid and electrolyte disorders along with central diabetes insipidus. The article presents modern approaches to the treatment of SIADH based on international clinical guidelines.

Experience of using vacuum fine-needle aspiration biopsy under ultrasound control in patients with focal thyroid pathology

Background: Due to its availability, high information content and harmlessness, ultrasound is an extremely effective method of differential diagnosis of thyroid pathology. Despite the sufficient arsenal of diagnostic methods, thyroid biopsy with cytological examination is the «gold» standard in diagnostic algorithms. Most often, fine — needle aspiration biopsy is carried out using disposable syringes of different capacities (5, 10, 20 ml3). The procedure is performed in most cases according to the «Free hand» method, this leads to several problems associated with the peculiarity of the study, primarily with the low vacuum created in the syringe and insufficient collection of cytological material, as well as insufficient practical experience for a novice specialist.Aim: Determination of the range of the possibility of using vacuum aspiration fine needle biopsy under the control of ultrasound navigation in patients with focal thyroid pathology.Materials and methods: On the basis of the Problem Research Laboratory «Diagnostic studies and minimally invasive technologies» of the Smolensk State Medical University, a device for vacuum fine — needle aspiration biopsy was developed. In the period 2019–2020, 780 vacuum fine — needle aspiration biopsy was performed under the control of ultrasound navigation in patients with thyroid nodules. The level of vacuum in the syringe during sampling of the material was — 0,5 bar (- 50 kPa).Results: A comparative analysis of the cytological results obtained with the help of vacuum fine — needle aspiration biopsy and the «Free hand» method shows a decrease in obtaining the category of Bethesda 1 by 3.3% and Bethesda 3 by 10.2%.Conclusion: This indicates the effectiveness and in formativeness of the use of vacuum fine — needle aspiration biopsy in patients with focal thyroid pathology in comparison with the standard «Free hand» method, as well as with vacuum fine — needle aspiration biopsy due to constant visual monitoring and the lack of movement of the syringe piston to create a discharge when taking cytological material, the probability of needle deviation from the biopsy zone is reduced, which increases the effectiveness of the study and facilitates the work of a novice specialist.

Laparoscopic removal of a large pheochromocytoma-paraganglioma of the Zuckerkandl organ

Paragangliomas represent 15 to 20% of all chromaffin tissue tumors. Most often, paragangliomas are located in the abdominal cavity along the large vessels — in the para-aortic region from the diaphragm to the aortic bifurcation. One of the most common extra-adrenal pheochromocytomas is the Zuckerkandl tumor, originating from the para-aortic accumulation of sympathetic tissue located in the area of the inferior mesenteric artery’s origine or in the zone of the aortic bifurcation itself. Due to the technical difficulty in performing laparoscopic removal of paragangliomas, conversion to laparotomy is frequent and reaches 80%. The article describes a clinical case of a patient suffering from this type of neoplasm, with diagnosis details and treatment by a successful radical laparoscopic intervention with a large chromaffinnoma located in a difficult anatomical zone removal.