Imaging Gorham's disease (vanishing bone)

Transcription

1 Imaging Gorham's disease (vanishing bone) Poster No.: C-2201 Congress: ECR 2010 Type: Scientific Exhibit Topic: Musculoskeletal Authors: D. Vanel, P. Ruggieri, M. Alberghini; Bologna/IT Keywords: Gorham, vascular tumor, imaging,bone tumor DOI: /ecr2010/C-2201 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 26

2 Purpose Gorham desease was described by Jackson, 1838 and 1872: first report of spontaneous resorption of bone. Gorham and Stout, 1955: established the clinical features and pathological description. It si rare (about 190 cases in literature) and more common in patients around 30 years. No gender or racial predilection nor familial bone disorder are known. It is a benign vascular proliferation of thin-walled endothelial capillaries, with dipose involution of the bone marrow, and extreme thinning of bony trabeculae We report the imaging features of an homogeneous series of the same center, with a long follow-up. Methods and Materials 11 cases with imaging studies are analysed in this study. They come from the data base cases were found, but only the 11 including imaging studies were selected. There were 11 males and 4 females. Results Locations were : Proximal femur 5 Pelvis 1 Page 2 of 26

3 Hip and knee 1 each Calcaneus 1 Humerus 1 Cervical spine 1 Main location (fig 1) : proximal femur 31 year old. Initial fracture.well 30 years later, after several surgeries and radiation therapy. Bone location Unifocal lesion : 4 cases Several lesions in the same bone : 1 case Multiple bones involved : 6 cases Unifocal involvement (fig 2-5 ) 68 year old woman. Pathologic fracture of the left humerus. Vascularised fibula, which resorbed. Resection and prothesis with good clinical result. Multiple locations in the same bone Fig year old woman. Involvement of the right iliac bone. Treated by biphosphonates, and embolisation. Death of pulmonary infection. Page 3 of 26

4 Multiple bones involved Fig 10 : 4 year old boy. Neck pain. Multiple lytic lesions of the cervical spine (C3-6). Biopsy, followed by local contention. No change for 4 years. Multiple bones involved fig year old man. Initial fracture. Involvement of the femur and iliac bone. Locally well with Calcitonin. Multiple bones involved Fig : 8 year old when diagnosed (multiple lytic lesions of the foot, the main in the os calcis). Treated by magneto therapy, steroid injections, calcitonin. Well with a 20 year follow up. Multiple bones involved Fig : 38 year old man. Lytic lesions of the left femur, tibia and foot. Treated by calcitonin. 24 years of follow up. Widespread remaining pain. Various treatments were used: No treatment 2pts. Conservative treatments 2pts(cast or brace). Surgery 5 pts. Medical treatment 6pts (byphosphonates, calcitonin, zoledronic acid, interferon, steroids). Radiotherapy 1pt. Selective arterial embolization 2pts No patient died of the disease Page 4 of 26

5 Images for this section: Page 5 of 26

6 Fig. 1: Lytic fractured porrly limited lesion. Page 6 of 26

7 Page 7 of 26

8 Fig. 2: Initial fracture. Page 8 of 26

9 Page 9 of 26

10 Fig. 3: Vascurarised fibula inserted in the broken bone. Page 10 of 26

11 Page 11 of 26

12 Fig. 4: Resortion of the bone and graft. Page 12 of 26

13 Page 13 of 26

14 Fig. 5: Final prothesis. Fig. 6: Lytic poorly limited tumor. Page 14 of 26

15 Fig. 7: CT : the bone has disappeared. Page 15 of 26

16 Fig. 8: Other lesions are detected on the iliac wing. Page 16 of 26

17 Fig. 9: T1W axial MR : mass destroying the bone. Page 17 of 26

18 Fig. 10: Lytic tumor of 4 vertebral bodies, with minimal soft tissue involvement. Page 18 of 26

19 Fig. 11: Lytic tumor of the broken proximal femur and of the acetabulum. Page 19 of 26

20 Fig. 12: Lytic lesions involving several bones of the foot. Page 20 of 26

21 Fig. 13: Collapse of the calcaneus. Page 21 of 26

22 Page 22 of 26

23 Fig. 14: Lytic tumor of the femur. Page 23 of 26

24 Page 24 of 26

25 Fig. 15: Involvement of the tibia by muultiple lytic lesions. Fig. 16: And multiple lytic lesions of the foot. Page 25 of 26

26 Conclusion Multiple lytic poorly limited lesions involving multiple bones in the same area are suggestive of the diagnosis Survival is excellent whatever the treatment References Massive osteolysis (acute spontaneous absorption of bone, phantom bone, disappearing bone); its relation to hemangiomatosis. GORHAM LW, STOUT AP. J Bone Joint Surg Am Oct;37-A(5): Personal Information Page 26 of 26