Bancel B, Patricot L M, Breton P, Bejui-Thivolet F, Vitrey D, Freidel M
Laboratoire d'Anatomie et Cytologie Pathologiques, Hôpital de la Croix-Rousse, UER Lyon Nord.
Ann Pathol. 1989;9(2):126-31.
We report a new case of aggressive juvenile fibromatosis (A.J.F.) in a 20-month-old girl. The lesion affected the inferior border of the left mandible and the adjacent soft tissues. The child presented with a painless mass, which had grown over a period of 2 months. Radiographs and computed tomographic scan showed a multilacunar bone defect with subcutaneous and gingival involvement. A biopsy was performed, followed by a partial hemimandibulectomy and a costal graft. The surgical specimen measured 4 X 3 X 3 cm. The patient did well 1 year after surgery. 16 cases of A.J.F. have been reported; with ours, 14 are mandibular. A.J.F. is a locally aggressive lesion, which doesn't metastasize. It occurs chiefly in childhood and adolescence from 1 1/2 to 18 years (median 6.5 year-old). Duration of symptoms prior to presentation varies from a few weeks to months. Clinically, it is a firm nodule. Radiographs are non-specific, but 9 cases have poorly defined destruction of the mandibular inferior border. Following a block resection of the tumor (13 cases), there is no recurrence. In this review, we discuss the clinico-pathologic diagnosis of this impressive tumor which is compared with other mandibular fibrous tumors in children.
我们报告了一例发生在一名20个月大女童身上的侵袭性幼年纤维瘤病（A.J.F.）。病变累及左下颌骨下缘及相邻软组织。患儿表现为无痛性肿块，在2个月内逐渐增大。X线片和计算机断层扫描显示为多房性骨缺损，伴有皮下和牙龈受累。进行了活检，随后实施了部分半下颌骨切除术和肋骨移植。手术标本大小为4×3×3厘米。术后1年患者恢复良好。此前已报道过16例A.J.F.；加上我们的病例，其中14例发生在下颌骨。A.J.F.是一种局部侵袭性病变，不会发生转移。它主要发生在1岁半至18岁的儿童和青少年时期（中位年龄6.5岁）。出现症状前的持续时间从几周至数月不等。临床上，它表现为质地坚硬的结节。X线片表现不具特异性，但9例可见下颌骨下缘边界不清的骨质破坏。在对肿瘤进行整块切除（13例）后，未见复发。在本综述中，我们讨论了这种令人瞩目的肿瘤的临床病理诊断，并将其与儿童期其他下颌骨纤维性肿瘤进行了比较。
