Fibrosing processes of connective tissue of unknown etiology affect different anatomical sites either in an isolated or a combined way, masking frequently as tumorous diseases. Clinical manifestations are nonspecific, determined by the site and spread of a process and appear in the form of the syndrome of compression of the adjacent organs. Organ site of a process determines complications and prognosis of disease. Fibromatosis can be diagnosed only in histological investigation. A physician should made aware of a history of traumas surgical interventions, intoxication, fever, increased ESR, dilatation of the aortic arch shadow during x-ray examination, GI tract bleeding, and outline the most effective therapeutic tactics for a patient with mediastinal fibromatosis.

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