Multifocal pneumocyte hyperplasia in tuberous sclerosis

Abstract

A 29-year-old woman, a known case of tuberous sclerosis, presented to our hospital with a 1-day history of multiple episodes of convulsions. This was associated with vague chest and abdominal pain. CT scans of the brain showed multiple calcific foci in the gray-white matter junctions and in the periventricular region. CT of the abdomen revealed liver hamartomas and renal angiomyolipoma.CT scans of the thorax showed multiple, tiny, randomly distributed, nodular densities of 3-10 mm in size and numerous miliary nodules 1-3 mm in both lung fields compatible with multifocal micronodular pneumocyte hyperplasia. They were more predominant in the lung periphery and the upper lobes. Few tiny simple cysts were also noted. Plain chest radiographs revealed bilateral diffuse fine nodular opacities (figs 1 and 2).Multifocal micronodular pneumocyte hyperplasia is a recently described pulmonary manifestation of tuberous sclerosis. First described by Popper et al in 1991, there are only slightly more than 30 cases reported in the literature since. These lesions are benign hamartomatous proliferations of type II pneumocytes along alveolar septa that exhibit fibrous thickening, increased elastic fibres, and aggregated alveolar macrophages. 1 2 It is usually with limited clinical significance and no known malignant potential.2

Yet another pulmonary manifestation of tuberous sclerosis

“…They show random distribution with peripheral and upper lobe predominance. [5] The radiological differentials include military granulomatous infections, langerhans cell histiocytosis and hematogenous metastasis.…”