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FGF23 Producing Mesenchymal Tumor.

Lucyna Papierska¹, Jarosław B Cwikła², Waldemar Misiorowski¹

¹Clinic of Endocrinology, Medical Center of Postgraduate Education, Warsaw, Poland.

Case Reports in Endocrinology

|March 19, 2014

Summary

A rare glomangiopericytoma caused tumor-induced osteomalacia by elevating FGF23 levels. Surgical removal of the maxillary sinus tumor successfully normalized phosphate levels and resolved symptoms.

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Area of Science:

Endocrinology
Oncology
Radiology

Background:

Hypophosphatemia, muscle pain, and low bone mineral density indicate potential bone-related metabolic disorders.
Previous treatments with phosphorus and active vitamin D metabolites were ineffective, suggesting an underlying complex cause.
Elevated Fibroblast Growth Factor 23 (FGF23) levels are strongly associated with tumor-induced osteomalacia.

Purpose of the Study:

To investigate the cause of persistent hypophosphatemia and osteomalacia in a 40-year-old male patient.
To identify the source of elevated FGF23 levels.
To evaluate the efficacy of surgical tumor removal for treating tumor-induced osteomalacia.

Main Methods:

Clinical evaluation including laboratory tests for phosphate, vitamin D metabolites, and FGF23 levels.
Diagnostic imaging: Somatostatin receptor scintigraphy (SRS) and CT scans to localize the tumor.
Tumor biopsy and cytological examination for diagnosis, followed by surgical resection and histological confirmation.

Main Results:

The patient presented with hypophosphatemia, hyperphosphaturia, and significantly elevated FGF23 levels.
SRS and CT scans identified a tumor in the right maxillary sinus, suspected to be a glomangiopericytoma.
Post-surgical removal of the tumor led to normalization of phosphatemia within 10 days.

Conclusions:

A maxillary sinus glomangiopericytoma was identified as the cause of tumor-induced osteomalacia due to FGF23 overproduction.
Surgical resection is an effective treatment for glomangiopericytoma-induced hypophosphatemia.
This case highlights the importance of considering rare tumors in the differential diagnosis of refractory hypophosphatemia.