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

Burckhardt, Marie-Anne ; Schifferli, Alexandra ; Krieg, Andreas ; Baumhoer, Daniel ; Szinnai, Gabor ; Rudin, Christoph

In: Pediatric Nephrology, 2015, vol. 30, no. 1, p. 179-182

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    Titel
    • Tumor-associated FGF-23-induced hypophosphatemic rickets in children: a case report and review of the literature
    Autor
    • Burckhardt, Marie-Anne. Pediatric Endocrinology, University Children's Hospital Basel, Basel, Switzerland
    • Schifferli, Alexandra. Pediatric Oncology, University Children's Hospital Basel, Basel, Switzerland
    • Krieg, Andreas. Pediatric Orthopedics, University Children's Hospital Basel, Basel, Switzerland
    • Baumhoer, Daniel. Bone Tumor Reference Center at the Institute of Pathology, University Hospital Basel, Basel, Switzerland
    • Szinnai, Gabor. Pediatric Endocrinology, University Children's Hospital Basel, Basel, Switzerland
    • Rudin, Christoph. Pediatric Nephrology, University Children's Hospital Basel, Spitalstrasse 33, 4031, Basel, Switzerland
    Dokumententyp
    • Postprint
    Sprache
    • Englisch
    Veröffentlicht in
    • Pediatric Nephrology, 2015, vol. 30, no. 1, p. 179-182. Springer Berlin Heidelberg
    Andere elektronische Ausgabe
    OAI-PMH-ID
    • oai:doc.rero.ch:332219
    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 34months. 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.