Diffuse Leiomyomatosis of the Esophagus: Disorder of Cell-Matrix Interaction? |
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Authors: | Paul Thorner Laurence Heidet Fernando Moreno Merlo Vern Edwards Corinne Antignac Marie-Claire Gubler |
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Institution: | Department of Pediatric Laboratory Medicine, Division of Pathology, Hospital for Sick Children and University of Toronto, 555 University Avenue, Toronto, Ontario, Canada M5G 1X8, CA INSERM U423, H?pital Necker-Enfants Malades, Université René Descartes, 149 rue de Sèvres, 75743 Paris, France, FR
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Abstract: | Diffuse leiomyomatosis (DL) is rare condition characterized by proliferation of smooth muscle in the upper gastrointestinal
tract. Most cases are associated with X-linked Alport syndrome and have partial deletions in the genes encoding both the α5
and α6 chains of collagen type IV. We studied aspects of cell-matrix interaction of myocytes in an esophagogastrectomy specimen
from a 12-year-old patient with DL. Myocytes had central areas of cytoplasmic rarefaction, which were actin positive and desmin
poor, with the reverse pattern of staining at the cell periphery. Electron microscopy (EM) showed that the areas of rarefaction
consisted of disorganized aggregates of filaments. The basement membranes ranged from thickened to thinned or absent. Immunohistochemical
staining for the α1–α4 chains of collagen type IV, the α1, α2, β2, and γ1 chains of laminin, nidogen, type VI collagen, and
fibronectin was normal. There was loss of the α5 and α6 chains of collagen type IV and the β1 chain of laminin. Normal staining
for α1, α2, α3, α4, α6, α8, and β1 integrins was noted. Staining for α5 integrin varied from normal to reduced or negative
in different cells. In DL, a primary abnormality of basement membrane may be associated with disorganization of the contractile
apparatus and alterations of certain integrins. This may reflect a disturbance of cell-matrix interactions that play a role
in cell differentiation and internal organization.
Received October 10, 1997; accepted February 4, 1998. |
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Keywords: | : Alport syndrome leiomyomatosis collagen type IV basement membrane integrin |
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