Remission of infantile generalized myofibromatosis after interferon alpha therapy.
Infantile myofibromatosis is the most common fibrous tumor of infancy. Solitary or generalized myofibromas without visceral involvement usually regress within a few months. The multifocal disease infantile generalized myofibromatosis, with visceral involvement, is associated with a significant mortality due to the effect of tumors on vital organs. We report a case of infantile generalized myofibromatosis with visceral involvement, including 2 right atrium tumors. The infant expressed high circulating vascular endothelial growth factor and fibroblast growth factor-2 levels, and interferon alpha-2b was started as antiangiogenic treatment, aimed at triggering regression of the life-threatening cardiac lesions. The tumors regressed and vascular endothelial growth factor and fibroblast growth factor-2 levels were reduced after treatment discontinuation.
Auriti
Cinzia
C
Department of Neonatology, Bambino Gesù Children's Hospital, Rome, Italy. auriti@opbg.net
Kieran
Mark W
MW
Deb
Giovanni
G
Devito
Rita
R
Pasquini
Luciano
L
Danhaive
Olivier
O
D005260:Female; D016222:Fibroblast Growth Factor 2 / Q000097:blood / Q000652:urine; D006801:Humans; D007223:Infant; D000077190:Interferon alpha-2; D016898:Interferon-alpha / Q000627:therapeutic use*; D018224:Myofibromatosis / Q000188:drug therapy*; D011994:Recombinant Proteins; D042461:Vascular Endothelial Growth Factor A / Q000097:blood / Q000652:urine
D000077190:Interferon alpha-2; D016898:Interferon-alpha; D011994:Recombinant Proteins; D042461:Vascular Endothelial Growth Factor A; D016222:Fibroblast Growth Factor 2