Smith N M, Byard R W, Foster B, Morris L, Clark B, Bourne A J
Department of Histopathology, Adelaide Children's Hospital, South Australia.
Pediatr Radiol. 1991;21(6):449-51. doi: 10.1007/BF02026689.
Ossifying fibromas of the long bones of the leg are benign lesions occurring in the pediatric age group identical in histological appearance to the similarly named tumor of the jaw in adults. Most frequently presentation occurs after minor trauma with symptoms of a swelling of the tibia or fibula which may be painful. Pathological fracture or limp are also occasional presentations. Congenital cases are extremely rare. We describe an otherwise normal male neonate who presented at birth with a bowed right lower leg. The limb was 1 cm shorter than the other side, with tibia vara and a firm mass situated anteriorly. X-ray showed a mixed lytic and sclerotic lesion in the proximal metaphysis of the tibia. Biopsy showed collagenous stroma containing spindle cells and irregular trabeculae of woven bone rimmed by plump osteoblasts. As the appearances were typical of an ossifying fibroma (osteofibrous dysplasia) no surgical treatment was given. The patient was well with no growth of the tumor and with radiological evidence of healing at 1 year follow up. This case is presented to draw attention to the clinicopathological features of this unusual lesion which must be considered in the differential diagnosis of congenital lesions of the tibia.
小腿长骨骨化性纤维瘤是发生于儿童年龄组的良性病变,其组织学表现与成人颌骨中同名肿瘤相同。最常见的表现是在轻微外伤后出现胫骨或腓骨肿胀症状,可能伴有疼痛。病理性骨折或跛行也时有发生。先天性病例极为罕见。我们描述了一名其他方面正常的男性新生儿,出生时右小腿弯曲。该肢体比另一侧短1厘米,有膝内翻,且前部有一质地硬的肿块。X线显示胫骨近端干骺端有溶骨性和硬化性混合病变。活检显示胶原性基质中含有梭形细胞和由丰满成骨细胞包绕的不规则编织骨小梁。由于其表现典型为骨化性纤维瘤(骨纤维发育异常),故未给予手术治疗。患者情况良好,肿瘤未生长,随访1年时有影像学愈合证据。本文报告此病例是为了引起对这种不寻常病变临床病理特征的关注,在胫骨先天性病变的鉴别诊断中必须考虑到这些特征。