Ten cases of benign primary pulmonary meningioma have been reported in the English literature. We describe herein an additional case of a benign meningioma arising in the lung showing comparable histologic features of sheets and whorls of epithelioid and meningothelial cells with numerous psammoma bodies. Immunohistochemistry showed that the tumor expressed vimentin and epithelial membrane antigen and was negative for keratin, CD34, glial fibrillary acidic protein, CAM 5.2 and S-100, in keeping with previously reported findings. Our review of the literature reveals similar clinical presentations and follow-up behavior and, where reported, similar electron microscopic and immunostaining features.
The clinical, electromyographic, and histologic characteristics of a 17-year-old girl with reducing body myopathy are described. She is, to our knowledge, the oldest reported case and the only patient described with severe mitral valve prolapse and scoliosis. Electromyography demonstrated spontaneous positive sharp waves and fibrillation potentials with many low-amplitude, short, polyphasic motor unit potentials. The right deltoid muscle was characterized by focal areas with large fibers associated with increased endomysial connective tissue and "split" fibers. Purple-gray sarcoplasmic masses stained with trichrome were PAS-negative, appeared as "empty" spaces with both ATPase and NADH-TR, and stained darkly with menadione NBT. The features described expand the clinical presentation of this myopathy, and may lead to a better understanding of its etiology.
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