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

G van Boekel, J Ruinemans-Koerts… - European Journal of …, 2008 - academic.oup.com
G van Boekel, J Ruinemans-Koerts, F Joosten, P Dijkhuizen, A van Sorge, H de Boer
European Journal of Endocrinology, 2008academic.oup.com
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 …
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.
Oxford University Press