Infantile myofibromatosis.
Infantile myofibromatosis (IMF) is a rare tumour with a wide spectrum of disease activity ranging from a solitary cutaneous nodule through to a multicentric form with widespread visceral involvement. It is characterised by its unique ability to spontaneously regress and has a typical histological appearance of actin-positive fibroblasts arranged in whorls or fascicles and vessels in a pericytomatous pattern. A male infant with multiple lesions involving the subcutaneous tissue and bone from birth is described and followed-up for two years. Treatment of IMF is dependent on the location of the tumour/s with surgery or chemotherapy reserved for rapidly progressive or symptomatic disease. However, due to the low rate of recurrence and the possibility of spontaneous tumoral regression, therapeutic abstention, as practised in our patient, is justified.
Ariffin
H
H
Department of Paediatrics, University of Malaya Medical Centre, Kuala Lumpur.
Teh
K H
KH
Looi
L M
LM
Ariffin
W A
WA
Lin
H P
HP
D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D018224:Myofibromatosis / Q000151:congenital* / Q000473:pathology / Q000628:therapy