Infantil myofibromatosis presenting with a pathological fracture: Case report Patolojik kirikla seyreden bir infantil miyofibromatozis olgusu


CELAYİR A., Özkan F., Daldal F., Özkan A.

Pediatrik Cerrahi Dergisi, cilt.14, sa.3, ss.134-136, 2000 (Scopus)

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 14 Sayı: 3
  • Basım Tarihi: 2000
  • Dergi Adı: Pediatrik Cerrahi Dergisi
  • Derginin Tarandığı İndeksler: Scopus
  • Sayfa Sayıları: ss.134-136
  • Anahtar Kelimeler: Child, Connective tissue neoplasms, Fracture, Myofibromatosis, Newborn, Skin
  • Sağlık Bilimleri Üniversitesi Adresli: Evet

Özet

Infantile myofibromatosis is a rare condition, commonly presenting the newborn period. This tumor can be seen as solitary or multi-centric. Sites involved include skin and subcutaneous tissues, muscles, bones and viscera. If the lesions are limited mainly to the skin and soft tissues, the prognosis becomes more favorable, but this tumor becomes potentially life threatening in case of multi-organ and/or multi-system involvement. When the histopathological examination confirmes infantile myofibromatosis, a full radiological examination is essential even in patients, who clinically present with a solitary disease. Lesions spontaneously regress in a 2-3 year period. We present a case with multi-centric infantile myofibromatosis that demonstrates a solitary lesion in the skin and a later pathological fracture in the right tibia. The patient remained well, there being only some mild modeling deformity of the right tibia on the X-Ray, with normal function of the leg. There has been no further treatment. No evidence of recurrence was noted. We stress the importance of clinical awareness in cases with infantile myofibromatosis.