Hollow
Visceral Myopathy
Epidemiology and Etiology:
Common sites:
Gross features:
- megaduodenum is
common radiologic finding
- thinning of
muscularis propria
- may see diverticuli
in affected areas
Histologic features:
- vacuolar
degeneration of muscularis propria smooth muscle cells (not a prominent
feature of progressive systemic sclerosis)
- vacuoles are empty
or filled with degenerated, poorly staining, fragmented muscle cells
- preferential
involvement of the external longitudinal muscle layer
- distinctive
honeycombed appearance to the muscle, apparent at low magnification
- muscularis mucosa
not involved
- separation of vacuolated
muscle cells with mild, lacy fibrosis in the involved muscularis propria
(in progressive systemic sclerosis the fibrosis is much more dense)
- subserosal
fibrosis may be seen
- normal myenteric
plexus
- absence of any
inflammatory infiltrate or vasculitis associated
- mucosal damage may
occur secondary to motility disorder
Immunophenotype:
Marker:
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Sensitivity:
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Specificity:
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Molecular features:
Other features:
- pseudo-obstruction
clinically
- not associated with
skeletal muscle abnormalities
References:
- The American
Journal of Surgical Pathology 1982;6;269.
- Gastroenterology
1979;77;664.