Definitive surgical treatment of osteomalacia induced by skull base tumor and determination of the half-life of serum fibroblast growth factor 23

Endocrine journal

Seiji Fukumoto¹  Shota Tanaka²  Taijun Hana²  Masaaki Shojima²  Nobuhito Saito²  Hirofumi Nakatomi²  Masako Ikemura³ 

[1] Department of Endocrinology, Faculty of Medicine, The University of Tokyo Hospital, Tokyo, Japan;Department of Neurosurgery, Faculty of Medicine, The University of Tokyo Hospital, Tokyo, Japan;Department of Pathology, Faculty of Medicine, The University of Tokyo Hospital, Tokyo, Japan

关键词: Fibroblast growth factor 23;    Half-Life;    Phosphaturic mesenchymal tumor;    Tumor induced osteomalacia;    Intraoperative measurement;   

DOI  :  10.1507/endocrj.EJ17-0177

学科分类：内分泌与代谢学

来源: Japan Endocrine Society

【 摘 要 】

Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome often associated with fibroblast growth factor 23 (FGF23)-producing tumors such as phosphaturic mesenchymal tumor, mixed connective tissue variant (PMTMCT) affecting the bone and soft tissue.We experienced a patient with progressive bone and muscle pain due to FGF23-related TIO.Venous sampling had strongly suggested the anterior skull base as a source of FGF23, which led to the discovery of a small tumor in the ethmoid sinus extending intracranially.Radical surgical resection confirmed the histological diagnosis of PMTMCT with FGF23 immunopositivity and achieved durable tumor control with complete resolution of symptoms.We serially measured serum FGF23 level before, during and after surgery and analyzed the data to determine the half-life of FGF23.Serum FGF23 level sharply declined as early as 20 minutes after en bloc tumor resection and completely normalized after surgery.The half-life of FGF23 was calculated to be approximately 18.5 minutes using single phase exponential decay model as well as semilog transformation formula.Serial measurements of serum FGF23 level can potentially declare “complete” resection of a FGF23-producing tumor and total cure of TIO; in this regard, development of its intraoperative measurement would be helpful in the management of this endocrine tumor.

【 授权许可】

Unknown