Intravascular inflammatory myofibroblastic tumors in infancy

Inflammatory myofibroblastic tumor (IMT), previously described as inflammatory pseudotumor, can occur at any age but is a recognized soft tissue tumor of childhood. Less than 10 previous cases have been described of IMT affecting the heart, in patients ranging from 5 months to 17 years of age. We pr...

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Bibliographic Details
Published in:Pediatric and developmental pathology 2002-07, Vol.5 (4), p.400-404
Main Authors: Sebire, Neil James, Ramsay, Alan, Sheppard, Mary, Malone, Marian, Harding, Brian, Risdon, Rupert Anthony
Format: Article
Language:English
Subjects:
Biomarkers, Tumor - analysis
Brain Neoplasms - secondary
Cerebral Hemorrhage - complications
Diagnosis, Differential
Female
Granuloma, Plasma Cell - complications
Granuloma, Plasma Cell - metabolism
Granuloma, Plasma Cell - pathology
Heart Neoplasms - metabolism
Heart Neoplasms - pathology
Humans
Immunohistochemistry
Infant, Newborn
Male
Vascular Neoplasms - complications
Vascular Neoplasms - metabolism
Vascular Neoplasms - pathology
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Summary:Inflammatory myofibroblastic tumor (IMT), previously described as inflammatory pseudotumor, can occur at any age but is a recognized soft tissue tumor of childhood. Less than 10 previous cases have been described of IMT affecting the heart, in patients ranging from 5 months to 17 years of age. We present three unusual, but similar, cases of IMT in infants, which were all predominantly intravascular in location, one of which was associated with death due to angiodestructive lesions of the coronary and cerebral arteries. These cases demonstrate an apparently distinct phenotype, with a predominant intravascular location of the tumor. Furthermore, this series highlights the difficulty in categorizing such lesions as benign versus malignant on histological grounds alone. IMT should be considered in the differential diagnosis of unusual pediatric intravascular spindle cell lesions.
ISSN:1093-5266
1615-5742
DOI:10.1007/s10024-001-0198-8