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• W1922023177 abstract "In the course of study of a family with a high incidence of polyposis of the colon (1, 2), it became apparent that a striking coincidence existed between large bowel lesions and peculiar bony tumors of the skull and facial bones in the same members of this family. Detailed studies of the genetic aspects of the two conditions have been published elsewhere (3). The present publication represents a study of the unusual bony lesions. It is hoped that the findings will contribute some information which will help to re-evaluate the condition described in the literature as “leontiasis ossea.” Review of Literature Virchow (4) coined the term leontiasisossea in his lectures on tumors in 1865. Several well documented cases of the condition were reported in the literature prior to that date. Reiss (5), in 1935, reviewed 35 cases in the literature and summarized the findings: The disease is characterized by massive thickening of a single or of numerous facial or cranial bones. The process usually starts in childhood or adolescence but frequently remains unnoticed for years. The maxillae and mandible and the frontal and temporal bones are most frequently affected. Over a period of years, proliferation may involve most of the bones of the skull and may become very disfiguring. In 80 per cent of these cases, the onset was prior to the age of twenty years. An incident of trauma or infection was listed as a predisposing factor in 66 per cent. Sixty per cent of the patients were males. In 20 per cent, death was attributed to the disease, but in 60 per cent the condition became stationary or progressed only very slowly. In two-thirds of the cases involvement was bilateral. The skeleton outside of the cranial and facial bones was involved four times, the femur three times, the hyoid bone once. An additional case of extracranial skeletal involvement of the fibula and the hyoid bone was described by Bickersteth (6). According to Bardenheuer's review (7), the incidence of involvement of various bones in an undetermined number of cases was: Maxillae.... Always Parietal bone. 5 times Mandible... 10 times Sphenoid..... 3 times Frontal bone 8 times Hyoid....... 2 times Zygoma..... 8 times Occipital bone Squama of (anterior temporal portion).... 2 times bone...... 5 times. Accurate accounting of all reported cases which would deserve a diagnosis of leontiasis ossea is almost impossible; inadequate illustrations and few reproductions of roentgenograms and photomicrographs accompany the descriptive reports. Identical cases appear to have been reported by more than one author. Also, many cases reported under the diagnosis of leontiasis ossea should undoubtedly, in the light of our present knowledge, be reclassified as osteitis deformans (Paget's disease), osteitis fibrosa cystica (hyperparathyroidism), or fibrous dysplasia. As late as 1933, Freedman (8) reported a case under the heading of leontiasis ossea which he himself considered to be due to Paget's disease." @default.
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• W1922023177 date "1954-06-01" @default.
• W1922023177 modified "2023-10-16" @default.
• W1922023177 title "Osteomatosis (Leontiasis Ossea)" @default.
• W1922023177 doi "https://doi.org/10.1148/62.6.830" @default.
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