Langerhans cell histiocytosis is a rare clonal disease characterized by the proliferation of CD1a-positive immature dendritic cells. The purpose of this article was to present an updated review of recent advances in the pathogenesis, clinical features, imaging and treatment of this disease. The discovery of oncogenic BRAF mutations and the presence of proinflammatory cytokines and chemokines confirmed the unusual characteristics of this disease. Currently, children with organ involvement who do not have a good response to chemotherapy and have neurodegeneration or diabetes insipidus are the most problematic patients. Further research is needed to improve the results of treatment.

We report a case of a six-week-old male infant with a congenital tumour of the left suprarenal gland. The tumour was diagnosed by ultrasound examination (USG) as a neonatal suprarenal mass. By the third month of life, the patient had typical signs of Cushing's syndrome. Based on the results of hormonal tests and imaging studies (USG and MRI), an adrenocortical tumour was suspected. The patient could only be cured by surgery. Histopathological examination confirmed clinical diagnosis of an adrenocortical tumour. We concluded that Cushing's syndrome may be a rare clinical manifestation of adrenal tumour in neonates. Only careful observation allows for proper diagnosis and appropriate treatment. Correct diagnosis is essential for optimal postnatal treatment.

Langerhans cell histiocytosis (LCH) is a rare disease with an unclear aetiology and extremely heterogenous clinical symptomatology. In the following manuscript, we discuss a case of a 3.5-year-old boy diagnosed with the multisystem form of LCH (MS-LCH) with involvement of the digestive tract, liver, bones, and skin. An increased abdominal circumference and diarrhoea were the first symptoms of the disease presented by the patient. The final diagnosis was made approximately 12-18 months after the early manifestations of the disease were reported. This case study clearly shows that in spite of significant progress in the health sciences, LCH still poses serious diagnostic difficulties, which may result in a delay in treatment and thus negatively affect the patient's prognosis.