Multivisceral dysplastic lesions in a patient with tuberous sclerosis and Langerhans cell histiocytosis.
Collections of large cells and smaller satellite-like cells arranged in an autonomic ganglion-like pattern and resembling the white matter lesions of tuberous sclerosis were found in the thymus, lungs, liver, appendix, and heart of an 8-month-old infant who exhibited other pathologic findings of tuberous sclerosis (rhabdomyomas of the heart and multiple kidney cysts). Focal cytoplasmic staining for S-100 and GFAP was noted in some large cells, suggesting neural (probably schwannian) differentiation. Dysplastic neurogenic foci appearing in internal organs may represent another morphologic marker of tuberous sclerosis. The simultaneous presence of Langerhans cell histiocytosis produced an unusual combination not previously reported.
Servicio de Patología, Hospital de Niños, La Plata, República Argentina.
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Drut
D001065:Appendix / Q000473:pathology; D006338:Heart Neoplasms / Q000473:pathology; D015614:Histiocytosis / Q000150:complications* / Q000378:metabolism / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D007231:Infant, Newborn; D007801:Langerhans Cells / Q000473:pathology*; D008099:Liver / Q000473:pathology; D008168:Lung / Q000473:pathology; D008297:Male; D009206:Myocardium / Q000473:pathology; D012207:Rhabdomyoma / Q000473:pathology; D013950:Thymus Gland / Q000473:pathology; D014402:Tuberous Sclerosis / Q000150:complications / Q000378:metabolism / Q000473:pathology*