Monostotic Fibrous Dysplasia in the Femur Strongly Expressing RANKL With Concomitant Osteoporotic Vertebral Compression Fracture: A Case Report

RANKL 强表达的股骨单骨性纤维发育不良伴有骨质疏松性椎体压缩性骨折:一例病例报告

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作者:Edelyn S Azurin, Norio Yamamoto, Katsuhiro Hayashi, Akihiko Takeuchi, Shinji Miwa, Kentaro Igarashi, Takashi Higuchi, Hirotaka Yonezawa, Sei Morinaga, Yohei Asano, Shiro Saito, Hiroyuki Tsuchiya

Aim

This study aimed to present a rare case of fibrous dysplasia (FD) in a healthy young adult man with a concomitant osteoporotic vertebral compression fracture. FD is a benign lesion of the bone characterized by replacement of the medullary component with fibro-osseous tissue that contains abnormally arranged trabeculae of immature woven bone. Recently it has been reported that several bone tumors including FD express the receptor activator of nuclear factor-kappa B (RANK) and its ligand (RANKL). Therefore, we hypothesized that FD contributed to osteoporosis, linked by the RANK-RANKL pathway of osteoclastogenesis. Case report: We report the case of a healthy man with monostotic femoral fibrous dysplasia (FD) with concomitant 7 th thoracic vertebra compression fracture due to osteoporosis [young adult mean (YAM) was 79% in bone mineral density (BMD)]. After curettage of the FD, artificial bone grafting in the cavity, and administration of alendronate sodium, BMD improved considerably within 9 months. FD is a benign bone condition in which abnormal fibrous tissue replaces normal bone. The axis of the receptor activator of nuclear factor-kappa B (RANK) and its ligand (RANKL) has been implicated in osteoporosis pathogenesis. RANKL immunohistochemical staining was performed, and strong staining of stromal cells was observed compared to other FD cases that showed weak to moderate staining.

Conclusion

The presence of FD might have contributed to the low BMD due to the RANK-RANKL axis acting as osteoclastogenesis stimulator.

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