Eur J Endocrinol
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DOI: 10.1530/EJE-07-0779
European Journal of Endocrinology, Vol 158, Issue 3, 431-437
Copyright © 2008 by European Society of Endocrinology
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CASE REPORT

Tumor producing fibroblast growth factor 23 localized by two-staged venous sampling

Gerben van Boekel1, Janneke Ruinemans-Koerts2, Frank Joosten3, Paul Dijkhuizen4, Adriaan van Sorge5 and Hans de Boer1

Departments of 1 Internal Medicine, 2 Clinical Chemistry, 3 Radiology, 4 , Gynecology and 5 Clinical Pharmacy, Ziekenhuis Rijnstate, Wagnerlaan 55, 6800 TA, Arnhem, The Netherlands

(Correspondence should be addressed to H de Boer; Email: hdeboer{at}alysis.nl)

Abstract

Background: Tumor-induced osteomalacia is a rare paraneoplastic syndrome characterized by hypophosphatemia, renal phosphate wasting, suppressed 1,25-dihydroxyvitamin D production, and osteomalacia. It is caused by a usually benign mesenchymal tumor producing fibroblast growth factor 23 (FGF-23). Surgical excision of the tumor is the first choice of treatment because complete resection is curative. Unfortunately, localization often fails due to the small size of these neoplasms. According to the current standards, supportive care with oral phosphate and calcitriol is the only feasible option in such cases.

Case: In this report, we describe the diagnostic value of two-staged venous sampling to localize the FGF-23 secreting tumor in a case where conventional imaging failed. In addition, we examined the effect of dipyridamole on renal phosphate excretion, explored the efficacy of octreotide and calcitonin to suppress the FGF-23 production, and closely evaluated the hormonal changes following successful removal of the tumor. The latter observations indicate that calcitonin may be useful to suppress tumor-FGF-23 production and that FGF-23 may be a clinically relevant inhibitor of parathyroid hormone secretion in man.







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