MSC Wong, EYW Kwan

Abstract

Congenital infantile myofibromatosis is a rare disorder with multiple fibromatous tumours in skin, bone, muscle, viscera and subcutaneous tissue presenting in early infancy. Multiple lytic bone lesions and vertebra involvement are also common, mimicking the clinical picture of metastatic tumours. However, it is a disease with variable prognosis depending on the type and extent of involvement. Spontaneous resolution occurs in most of the cases without visceral involvement. We report good outcome in a case of multicentric type of infantile myofibromatosis with no visceral involvement. Invasive investigations and aggressive intervention should be avoided in these cases.

先天性嬰兒肌纖維瘤病是一種罕見的疾病，在嬰兒早期即出現皮膚、骨胳、肌肉、內臟器官以及皮下組織的多發纖維瘤，多發的骨質細胞溶解變性和椎體受累也非常常見，和轉移瘤的臨床表現相似。但本病因其腫瘤型別不同和受累的程度不同有各不相同的預後。在沒有內臟器官受累的病例，多數病人其腫瘤可以自行緩解。我們在這裏報導了一例嬰兒肌纖維瘤病的病例，這個病兒是不伴內臟器官受累的多竈性型肌纖維瘤病，其預後良好。我們建議在這類病例，應該避免創傷性的實驗室檢查和治療干預。