Tumor-associated FGF-23-induced hypophosphatemic rickets in children: a case report and review of the literature

Background Tumor-associated fibroblast growth factor 23 (FGF-23)-induced hypophosphatemic rickets is a rare but known pediatric entity first described in 1959. It results from local production of phosphatonins by benign and malignant mesenchymal tumors. Case-Diagnosis/Treatment We report an 8-year-o...

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Published in:Pediatric nephrology (Berlin, West) West), 2015-01, Vol.30 (1), p.179-182
Main Authors: Burckhardt, Marie-Anne, Schifferli, Alexandra, Krieg, Andreas H., Baumhoer, Daniel, Szinnai, Gabor, Rudin, Christoph
Format: Article
Language:English
Subjects:
Bone Neoplasms - complications
Bone Neoplasms - metabolism
Bone surgery
Brief Report
Care and treatment
Case reports
Child
Diagnosis
Fibroblast Growth Factors - biosynthesis
Fibroblasts
Growth factors
Humans
Immunohistochemistry
Literature reviews
Male
Medicine
Medicine & Public Health
Nephrology
Patients
Pediatrics
Plasma
Proteins
Rickets
Rickets, Hypophosphatemic - etiology
Risk factors
Tomography
Tumors
Urology
Vitamin deficiency
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Summary:Background Tumor-associated fibroblast growth factor 23 (FGF-23)-induced hypophosphatemic rickets is a rare but known pediatric entity first described in 1959. It results from local production of phosphatonins by benign and malignant mesenchymal tumors. Case-Diagnosis/Treatment We report an 8-year-old boy with tumor-associated hypophosphatemic rickets due to paraneoplastic FGF-23 secretion from a benign mesenchymal pelvic-bone tumor. Excessive FGF-23 production was visualized by immunohistochemistry in the resected tumor. Phosphate wasting stopped immediately after tumor resection. We reviewed 26 reports of pediatric patients with tumor-induced hypophosphatemic rickets; paraneoplastic FGF-23 secretion was documented in only three of them. All tumors developed inside bone, were benign in 21/26 cases, and were localized in femur/tibia (13/26), radius/ulna/humerus (7/26), pelvis (4/26), rib (1/26), and craniofacial (1/26) bones. Mean interval between onset of signs and/or symptoms and diagnosis was 34 months. Conclusions In patients with hypophosphatemic rickets acquired beyond infancy, radiologic investigations for bone tumors need to be performed rapidly. In contrast to biochemical screening for increased circulating FGF-23 levels, immunohistochemical confirmation of FGF-23 production in resected tumor tissue can be regarded as being well established.
ISSN:0931-041X
1432-198X
DOI:10.1007/s00467-014-2979-0