钙化性腱膜纤维瘤5例临床病理分析

Clinicapathologic features of calcifying aponeurotic fibroma: an analysis of 5 cases

目的探讨钙化性腱膜纤维瘤（CAF）的临床病理特征及鉴别诊断。方法分析5例CAF病例的组织学形态和免疫组化结果，同时复查临床资料并文献复习。结果5例CAF中男性3例，女性2例；年龄1～14岁，平均9岁。其中3例肿瘤位于手指和手掌部，2例位于足部。5例中有1例局部复发。病变位于筋膜、腱膜，呈实性，边界不清，直径〈3cm；切面灰白色，硬韧，有砂砾感。镜下见肿瘤向周围脂肪、肌肉组织呈浸润性生长，为卵圆形、肥胖的纤维母细胞增生，瘤细胞沿一定方向线性排列，呈鱼群样；中等量的致密胶原纤维透明变性，散在的软骨岛伴钙化；2例钙化灶周边见多核巨细胞。病灶无坏死、无明显异型性，核分裂象少见。免疫组化为vimentin、SMA和MSA（＋）。结论CAF是一种好发于儿童手掌和足底、易局部复发的良性肿瘤，临床需与婴幼儿纤维瘤病、腹壁外纤维瘤病、婴幼儿纤维性错构瘤和软组织软骨瘤等鉴别。

Objective To study the clinicapathologic characteritics and differential diagnosis of calcifying aponeurotic fibroma （CAF）. Methods Five cases diagnosed CAF were investigated with clinical data analyses,light microscopy and immunohistochemistry, and the literature was also reviewed. Results Among 5 cases, 3 cases were males and 2 cases were females, with age ranging 1 to 14 years, （mean 9 years）. CAF arose near tendons, fascia and aponeuroses. The sites of involvement were the palm and finger（n = 3 ） , and feet region （ n = 2）. Pathological features included an ill-defined, firm, pale, and infiltrative mass. Tumors sized less than 3 cm in greateat diameter in 5 cases, with a grithy cut surface. Micoroscopic examination showed that CAF lesion had nodular deposits of calcification, each surrounded by a palisade of rounded chondrocyte-like cells, arranged in short, parallel arrays. A less cellular spindled, bibroblastic component between the coalescent calcified nodules and emanating into the surrunding soft tissues was noted. Osteoclstic giant cells might border the caluium. Degenerated nuclei were present in the calcified areas but necrosis and mitoses were not features of CAF. Immunohistochemically, 3 cases examined had variably expressed vimentin, SMA, MAS and S-100 protein. Conclusions CAF is a small tumour of the palms and soles of children with a propensity for local recurnence. Familiarity with this entity should help to avoid confusion with other similar tumors such as infantile and extranldominal fibromatoses, chrodroma of soft parts and fibrous hamartoma of infancy.