Clinical Research Directory

Inclusion Criteria: * Confirmed diagnosis of JNA. * Male gender. * Age 7-18 years. * Signed informed consent of the parents or the official representative for patients under 14 years of age, the patient and the patient's parents for persons from 14 to 18 years of age. * Adequate liver and kidney function. * Patients with primary JNA * Patients with tumor volume according to MRI \>35 ml. Group A1 (Extended phase:) - patients with decrease or stable tumor volume or an increase in tumor volume \< 20 %, after 3 months of sirolimus therapy. Group A2 (Extended phase:)- patients with an increase in tumor volume\> 20 % after 3 months of sirolimus therapy or the presence of other indications for surgical treatment, according to the investigator opinion. Historical control group - patients with diagnosed primary JNA, received treatment in Dmitry Rogachev's Center in the period from January 1, 2013 to April 15, 2022. * Group B RECCURRENT JNA, Patients with recurrent JNA after primary surgery, who have not previously received sirolimus therapy. * Historical control group - patients with diagnosed recurrent JNA, received treatment in Dmitry Rogachev' s Center in the period from January 1, 2013 to April 15, 2022. Exclusion Criteria: Hypersensitivity to sirolimus or its analogues. The presence of acute or chronic infections, including opportunistic infections. Hepatic and/or renal insufficiency. The need for concomitant use of inducers (e.g. rifampicin, rifabutin) or inhibitors (e.g. ketoconazole) of the cytochrome CYP3A4 system Previous therapy with sirolimus or other mTOR inhibitors. Indications to palliative therapy, according to investigator's opinion. Participation in other clinical trials.