Copyright
©The Author(s) 2020.
World J Clin Cases. Dec 6, 2020; 8(23): 6197-6205
Published online Dec 6, 2020. doi: 10.12998/wjcc.v8.i23.6197
Published online Dec 6, 2020. doi: 10.12998/wjcc.v8.i23.6197
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 |
- Citation: Lin T, Li XY, Zou CY, Liu WW, Lin JF, Zhang XX, Zhao SQ, Xie XB, Huang G, Yin JQ, Shen JN. Discontinuous polyostotic fibrous dysplasia with multiple systemic disorders and unique genetic mutations: A case report. World J Clin Cases 2020; 8(23): 6197-6205
- URL: https://www.wjgnet.com/2307-8960/full/v8/i23/6197.htm
- DOI: https://dx.doi.org/10.12998/wjcc.v8.i23.6197