Tumour-induced osteomalacia: An emergent paraneoplastic syndrome

Endocrinol Nutr. 2016 Apr;63(4):181-6. doi: 10.1016/j.endonu.2015.10.011. Epub 2015 Dec 21.
[Article in English, Spanish]

Abstract

Endocrine paraneoplastic syndromes are distant manifestations of some tumours. An uncommon but increasingly reported form is tumour-induced osteomalacia, a hypophosphatemic disorder associated to fibroblast growth factor 23 (FGF-23) secretion by tumours. The main biochemical manifestations of this disorder include hypophosphatemia, inappropriately low or normal tubular reabsorption of phosphate, low serum calcitriol levels, increased serum alkaline phosphatase levels, and elevated or normal serum FGF-23 levels. These tumours, usually small, benign, slow growing and difficult to discover, are mainly localized in soft tissues of the limbs. Histologically, phosphaturic mesenchymal tumours of the mixed connective tissue type are most common. Various imaging techniques have been suggested with variable results. Treatment of choice is total surgical resection of the tumour. Medical treatment includes oral phosphorus and calcitriol supplements, octreotide, cinacalcet, and monoclonal antibodies.

Keywords: Factor de crecimiento fibroblástico 23; Fibroblast growth factor 23; Hipofosfatemia; Hypophosphatemia; Oncogenic osteomalacia; Osteomalacia oncogénica; Osteomalacia tumoral; Phosphaturic mesenchymal tumour; Tumor mesenquimal fosfatúrico; Tumour-induced osteomalacia.

MeSH terms

  • Calcitriol
  • Fibroblast Growth Factor-23
  • Humans
  • Hypophosphatemia
  • Octreotide
  • Osteomalacia / etiology*
  • Paraneoplastic Syndromes*

Substances

  • FGF23 protein, human
  • Fibroblast Growth Factor-23
  • Calcitriol
  • Octreotide