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Tumor-induced osteomalacia with elevated fibroblast growth factor 23: a case of phosphaturic mesenchymal tumor mixed with connective tissue variants and review of the literature

Fang-Ke Hu Fang Yuan Cheng-Ying Jiang Da-Wei Lv Bei-Bei Mao Qiang Zhang Zeng-Qiang Yuan Yan Wang

癌症(英文版)2011,Vol.30Issue(11):794-804,11.
癌症(英文版)2011,Vol.30Issue(11):794-804,11.DOI:10.5732/cjc.011.10013

Tumor-induced osteomalacia with elevated fibroblast growth factor 23: a case of phosphaturic mesenchymal tumor mixed with connective tissue variants and review of the literature

Tumor-induced osteomalacia with elevated fibroblast growth factor 23: a case of phosphaturic mesenchymal tumor mixed with connective tissue variants and review of the literature

Fang-Ke Hu 1Fang Yuan 2Cheng-Ying Jiang 1Da-Wei Lv 3Bei-Bei Mao 2Qiang Zhang 1Zeng-Qiang Yuan 3Yan Wang1

作者信息

  • 1. Orthopedic Department, Chinese PLA General Hospital, Beijing 100853, P. R. China
  • 2. Medical College, Nankai University,Tianjin 300071, P. R. China
  • 3. institute of Biophysics, Chinese Academy of Sciences, Beijing 100101, P. R. China
  • 折叠

摘要

Abstract

Tumor-induced osteomalacia (TIO),or oncogenic osteomalacia (OOM),is a rare acquired paraneoplastic disease characterized by renal phosphate wasting and hypophosphatemia.Recent evidence shows that tumor-overexpressed fibroblast growth factor 23 (FGF23) is responsible for the hypophosphatemia and osteomalacia.The tumors associated with TIO are usually phosphaturic mesenchymal tumor mixed connective tissue variants (PMTMCT).Surgical removal of the responsible tumors is clinically essential for the treatment of TIO.However,identifying the responsible tumors is often difficult.Here,we report a case of a TIO patient with elevated serum FGF23 levels suffering from bone pain and hypophosphatemia for more than three years.A tumor was finally located in first metacarpal bone by octreotide scintigraphy and she was cured by surgery.After complete excision of the tumor,serum FGF23 levels rapidly decreased,dropping to 54.7% of the preoperative level one hour after surgery and eventually to a little below normal.The patient's serum phosphate level rapidly improved and returned to normal level in four days.Accordingly,her clinical symptoms were greatly improved within one month after surgery.There was no sign of tumor recurrence during an 18-month period of follow-up.According to pathology,the tumor was originally diagnosed as “glomangioma” based upon a biopsy sample,“proliferative giant cell tumor of tendon sheath” based upon sections of tumor,and finally diagnosed as PMTMCT by consultation one year after surgery.In conclusion,although an extremely rare disease,clinicians and pathologists should be aware of the existence of TIO and PMTMCT,respectively.

关键词

Tumor-induced osteomalacia (TIO)/ fibroblast growth factor 23 (FGF23)/ phosphaturic mesenchymal tumors mixed connective tissue variants (PMTMCT)/ hypophosphatemia

Key words

Tumor-induced osteomalacia (TIO)/ fibroblast growth factor 23 (FGF23)/ phosphaturic mesenchymal tumors mixed connective tissue variants (PMTMCT)/ hypophosphatemia

引用本文复制引用

Fang-Ke Hu,Fang Yuan,Cheng-Ying Jiang,Da-Wei Lv,Bei-Bei Mao,Qiang Zhang,Zeng-Qiang Yuan,Yan Wang..Tumor-induced osteomalacia with elevated fibroblast growth factor 23: a case of phosphaturic mesenchymal tumor mixed with connective tissue variants and review of the literature[J].癌症(英文版),2011,30(11):794-804,11.

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