Desmoplastic fibroma (DF) of bone is a rare, nonmetastasizing but locally aggressive tumor that has been discussed infrequently in the radiology literature. The radiographs from 107 previously published cases of DF and seven cases from the authors' institution were analyzed to better understand and define its radiographic characteristics. DF was most common in the mandible, pelvis, and femur. A geographic pattern of bone destruction, with a narrow zone of transition and nonsclerotic margins, was seen in 80 (96%) patients with intraosseous DF for whom radiographs were available (83 patients). Internal pseudotrabeculation was seen in 76 (91%). Although widening of the host bone due to gradual apposition of periosteal new bone was common, occurring in 74 (89%) patients, distinct periosteal new bone occurred in only two (2%) patients with DF of intraosseous origin. The cortex was breached in 23 (28%) patients. Three cases of DF arising in the periosteum were identified and were differentiated radiographically from desmoid tumors of intraosseous or soft-tissue origin.

This study describes the clinical presentation and the course of Ribbing disease in six patients and illustrates imaging features on plain radiography, conventional and computed tomography, and 99mTc methylene diphosphonate bone scans. MATERIALS AND METHODS. Between 1982 and 1990, six female patients presented with painful bony lesions that were believed to be Ribbing disease. Ten bones were affected: both tibiae in three patients, a unilateral tibia in one, both femora in one, and a unilateral femur in one. Plain radiographs and either conventional or computed tomography were available for all patients and 99' 'Tc-methylene diphosphonate bone scans, for five patients. All patients underwent open biopsy and/or surgical decompression. RESULTS. The diagnosis was reached in all patients through a combination of clinical findings (lack of systemic signs of infection or laboratory values suggesting metabolic bone disease), imaging, histologic evaluation, and specimen cultures. Radiographs and tomographic studies showed benign-appearing endosteal and periosteal cortical thickening. Intense uptake of radionuclide tracer was confined to the shaft of all involved bones. All pathologic specimens revealed nonspecific changes that included a slow increase in the mass of cortical and endosteal bone. These specimens also assisted in excluding neoplastic or infectious causes for the new bone formation. CONCLUSION. Ribbing disease is a rare disorder that, on imaging studies. may simulate stress fracture, chronic infection, bone-forming neoplasia, or a systemic metabolic or endocrine disorder. Clinical and imaging features may suggest the correct diagnosis.

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