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Tumor producing fibroblast growth factor 23 localized by two-staged venous sampling
Gerben van Boekel1, Janneke Ruinemans-Koerts, Frank Joosten
1Departments of Internal Medicine, Ziekenhuis Rijnstate, Wagnerlaan 55, 6800 TA, Arnhem, The Netherlands.
European Journal of Endocrinology
|February 27, 2008
Summary
Tumor-induced osteomalacia, a rare condition caused by FGF-23 secreting tumors, can be localized using two-staged venous sampling when imaging fails. This technique aids in surgical tumor removal, offering a curative treatment for this phosphate-wasting disorder.
Area of Science:
- Endocrinology
- Oncology
- Nephrology
Background:
- Tumor-induced osteomalacia is a rare paraneoplastic syndrome characterized by hypophosphatemia and renal phosphate wasting.
- It is caused by fibroblast growth factor 23 (FGF-23) secreting mesenchymal tumors, often difficult to localize due to small size.
- Current management for unresectable tumors involves supportive care with oral phosphate and calcitriol.
Observation:
- This report details the successful localization of a challenging FGF-23 secreting tumor using two-staged venous sampling after conventional imaging failed.
- Investigated the effects of dipyridamole on renal phosphate excretion and the efficacy of octreotide and calcitonin in suppressing FGF-23 production.
- Monitored hormonal changes post-tumor resection.
Findings:
- Two-staged venous sampling proved effective in localizing the elusive FGF-23 secreting tumor.
- Calcitonin demonstrated potential in suppressing tumor-derived FGF-23 production.
- Fibroblast growth factor 23 (FGF-23) may act as a significant inhibitor of parathyroid hormone secretion in humans.
Implications:
- Two-staged venous sampling offers a viable diagnostic strategy for localizing occult FGF-23 secreting tumors in tumor-induced osteomalacia.
- Calcitonin emerges as a potential therapeutic agent for managing FGF-23 overproduction.
- Understanding FGF-23's role in parathyroid hormone regulation could reveal new therapeutic targets.
