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Abstract:
A case of diffuse lymphangiomyomatosis with involvement of the large veins of the body is reported. The clinical, pathologoanatomic and especially the roentgenologic findings are described. Interstitial lung thickening, pleural effusions and spontaneous pneumothorax are findings which together with a stasis in the lymphatic system strongly suggest the diagnosis. The extensive involvement of the venous system supports the theory of a hamartomatous nature of the disease.