Infantile myofibromatosis: a case with unusual features and review of the literature
Kotiloğlu, E.; Göğüş, S.; Ruacan, S.; Akyüz, C.; Büyükpamukçu, M.; Srialioğlu, F.
Turkish Journal of Pediatrics 38(4): 527-532
1996
ISSN/ISBN: 0041-4301 PMID: 8993185 Document Number: 461350
A three-month-old boy was admitted for a slowly progressing nodule in his right chin, first recognized at birth and recently accompanied by facial paralysis. It was found to be a soft tissue mass arising in subcutaneous tissue and extending deep Into the temporal muscle, causing temporal bone erosion without infiltrating the dura. Initially interpreted as mesenchymal chondrosarcoma, combined chemotherapy (PULSE-VAC) was given. Eighteen months later an additional nodule developed in the peraspinal skin. Evaluation of both lesions showed vimentin and alpha-smooth-muscle-action positivity. As the final diagnosis was multicentric infantile myofibromatosis, the child was followed up without therapy. Thirty months later, multiple osteolytic lesions appeared on the skull and long bones of the extremities. Some lesions remained stable and some regressed during two years of follow-up.