Infantile myofibromatosis: a case with unusual features and review of the literature

Kotiloǧlu E., Göǧüş S., Ruacan Ş., Akyüz C., Büyükpamukçu M., SARIALİOĞLU F.

Turkish Journal of Pediatrics, cilt.38, sa.4, ss.527-532, 1996 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 38 Sayı: 4
  • Basım Tarihi: 1996
  • Dergi Adı: Turkish Journal of Pediatrics
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.527-532
  • Anahtar Kelimeler: myofibromatosis, mesenchymal hamartomas, immunohistochemistry, childhood tumors, CONGENITAL FIBROMATOSIS, SOLITARY, BONE
  • Dokuz Eylül Üniversitesi Adresli: Evet

Özet

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 paraspinal skin. Evaluation of both lesions showed vimentin and α-smooth-muscle-action positivity. As the final diagnosis was multicenlric 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.