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 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.
Sebire
Neil James
NJ
Department of Histopathology, Great Ormond Street Hospital for Children, Great Ormond Street, London WC1N 3JH, UK.
Ramsay
Alan
A
Sheppard
Mary
M
Malone
Marian
M
Harding
Brian
B
Risdon
Rupert Anthony
RA
D014408:Biomarkers, Tumor / Q000032:analysis; D001932:Brain Neoplasms / Q000556:secondary; D002543:Cerebral Hemorrhage / Q000150:complications; D003937:Diagnosis, Differential; D005260:Female; D006104:Granuloma, Plasma Cell / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D006338:Heart Neoplasms / Q000378:metabolism / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D007231:Infant, Newborn; D008297:Male; D019043:Vascular Neoplasms / Q000150:complications / Q000378:metabolism / Q000473:pathology*
D014408:Biomarkers, Tumor