NuPetit be sporadic Familial forms
NuPetit be
sporadic. Familial forms of IM have been reported with autosomal dominant and
recessive inheritance patterns [13, 14]. Mutations in the PDGFRB
(platelet-derived growth factor receptor beta) and NOTCH3 gene have been
identified in the autosomal dominant forms of the disease [15, 16]. Genetic
counseling should be considered in familial cases, as future offspring may be
affected. Histopathology can provide a definitive diagnosis. IM have a characteristic
histological pattern with an outer zone of spindle-shaped myofibroblasts
arranged in fascicles and an inner zone of round cells with enlarged
hyperchromatic nuclei surrounding thin walled hemangiopericytoma-like blood
vessels.
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