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.

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.

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.