Shapeero L G, Vanel D, Ackerman L V, Terrier-Lacombe M J, Housin D, Schwaab G, Sigal R, Masselot J
Department of Radiology, Institut Gustave-Roussy, Villejuif, France.
Skeletal Radiol. 1993 Nov;22(8):563-8. doi: 10.1007/BF00197136.
Fibrous dysplasia is usually a slowly progressive, benign disease that develops over several years and presents with deformity or mild symptomatology. Five of 34 patients (ages 4-21 years), who were subsequently diagnosed histologically as having fibrous dysplasia of the maxillary sinus, rapidly developed soft tissue masses of the malar region over a period of less than 4 months with accompanying pain (2 patients) and nasal obstruction and exophthalmos (2 patients). Each was clinically suspected of having a sarcoma; two had been thought to have an "osteofibrosarcoma" on initial biopsy at outside hospitals. After resection, all lesions developed regrowth. At histopathologic examination, both initial and recurrent masses proved to be typical fibrous dysplasia with spicules of woven bone in cellular, sometimes vascular, fibrous tissue. No malignant degeneration was found. On conventional radiography, aggressive fibrous dysplasia produced opacification and expansion of the maxillary sinus and apparent disruption of its wall with an associated soft tissue mass. Computed tomography (CT) demonstrated voluminous heterogeneous masses with "ground glass appearance", calcifications, areas of enhancement, low attenuation, cystic areas, and a thinned, sometimes interrupted, maxillary wall. Despite the aggressive clinical course for both initial and recurrent lesions, the CT findings of a "ground glass" mass with calcifications surrounded by a maxillary sinus wall, even if incomplete, can suggest the diagnosis of aggressive fibrous dysplasia.
骨纤维异常增殖症通常是一种缓慢进展的良性疾病，病程长达数年，表现为畸形或轻度症状。34例患者（年龄4 - 21岁）中，有5例随后经组织学诊断为上颌窦骨纤维异常增殖症，在不到4个月的时间内迅速出现颧部软组织肿块，并伴有疼痛（2例）、鼻塞和眼球突出（2例）。每例临床均怀疑患有肉瘤；其中2例在外院初次活检时被认为患有“骨纤维肉瘤”。切除后，所有病变均复发。组织病理学检查显示，初次及复发性肿块均为典型的骨纤维异常增殖症，在细胞丰富、有时有血管的纤维组织中可见编织骨小梁。未发现恶性变。在传统X线片上，侵袭性骨纤维异常增殖症表现为上颌窦混浊、扩大，窦壁明显破坏并伴有软组织肿块。计算机断层扫描（CT）显示大量不均匀肿块，呈“磨玻璃样外观”、有钙化、强化区域、低密度区、囊性区，上颌窦壁变薄，有时中断。尽管初次及复发性病变的临床过程具有侵袭性，但CT表现为有钙化的“磨玻璃”样肿块被上颌窦壁包绕，即使不完整，也可提示侵袭性骨纤维异常增殖症的诊断。
