婴儿型肌纤维瘤病的临床特点

目的 通过对婴幼儿肌纤维瘤病临床特点的分析，提高对本病的认识，掌握鉴别诊断的方法。方法 通过收治的3例患儿的诊治经过，3例在术前均经CT或钡餐检查，并经手术切除或活检和病理检查证实为本病，并结合文献讨论其临床特点。结果 3例患儿分别为2岁，1d和13岁，均以无痛性肿块为主要表现，病变部位分别位于盆腔、会阴部皮下及胃，均经手术治疗和病理证实。结论 本病少见，先天性或发生于2岁以内患儿的孤立性无痛性清表性结节是本病最为显著的临床特点，有自限性，局部切除即可治愈，与硬纤维瘤显著不同。但累及内脏以及全身广泛性病变者预后不良。

Clinical characteristics of infantile myofibromatosis

Objective To characterize the clinical presentation of infantile myofibromatosis (IMF).Methods Three cases (male 2,female 1) with myofibromatosis were reported. CT or barium examination was performed preoperatively,and the diagnosis was confirmed intra operatively and histologically.Results All of them presented with painless mass located in pelvic cavity,perineum or stomach and underwent a local resection.Conclusions IMF is an uncommon tumor which presents at birth or before 2 years of age.Solitary painless subcutaneous nodule is the most obvious characteristic,and the nodule can regress spontaneously.If the tumor fails to regress,local resection is recommended.They differ markedly from desmoid.Multiple lesions with visceral involvement has poor prognosis.