Table 2.
Cases diagnosed as polyostotic fibrous dysplasia
|
Ref.
|
Year
|
Sex
|
Age
|
Location
|
Appendix
|
Treatment
|
Outcome
|
| Sagmeister et al[12] | 2016 | F | 27 | Throughout the skeleton. Transverse fracture of the distal right femur | Continuous lesions, extensive bone expansion, cyst formation, cortical loss | Skin traction for 8 wk. Intensive physiotherapy for the fracture | Recovered well, returning to baseline 3 mo later |
| Wu et al[2] | 2014 | F | 38 | Sternum, thoracic spine, ribs, right femur, and tibia | Multiple lytic, expansile lesions, continuous pathologic fractures in the thoracic spine | Surgical therapy. Diphosphate therapy with Vit D and calcium | Completely recovered. Able to participate in daily life and work 2 yr later |
| Kodama et al[10] | 2012 | F | 8 | Right pelvis, bilateral femurs, and fibula | Discontinuous lesions | Thigh coxa valga osteotomy and plate fixation. Diphosphate therapy | No complaints of severe pain in lower extremity. Low bone turn-over rate |
| Aras et al[9] | 2012 | M | 48 | Cranium, left hemithorax, bilateral upper, lower extremities, and pelvic bones | Continuous lesions, bladder cancer | No treatment reported | No outcome reported |
| Boston et al[8] | 1994 | M | 3.3 | Proximal left femur and proximal left humerus | Albright-McCune syndrome, no café-au-lait pigmentation, Cushing syndrome | Bilateral adrenalectomy at 7-yr-old with steroid replacement | Cushing syndrome removed. Still with prepubertal and elevated liver enzyme |
| Lourenço et al[11] | 2015 | F | 17 d | Multiple lesions with fracture in left ulna | Multiple organs involved, Café-au-lait pigmentation, mosaic GNAS gene mutation | Metyrapone therapy for Cushing syndrome | Cushing syndrome recovered. Death due to respiratory infection |
| The current case | F | 27 | Left ischium, left distal fibula, calcaneus, and talus | Discontinuous lesions, intractable bone pain, Cushing syndrome | Diphosphate therapy | Still severe pain. Difficulty participating in daily life and job |
M: Male; F: Female.