Tumor-induced phosphopenic osteomalacia: modern approaches to diagnostics and treatment

Phosphopenic osteomalacia (PPOM) is a rare variant of paraneoplastic syndrome caused by tumor synthesis of fibroblast growth factor 23 (FGF23). FGF23 secretion leads to a decrease in phosphate reabsorption and calcitriol levels, which leads to the development of severe hypophosphataemia and hypocalcaemia. FGF23 synthesis is predominantly associated with benign mesenchymal tumors, but has also been described in malignant neoplasms. The main clinical manifestations of PPOM are generalized myalgias and myopathy, ostealgia, pathological fractures, etc. The diagnosis of the disease requires a step-by-step investigation using somatostatin receptor-based imaging techniques, as these have the highest sensitivity for the detection of neoplasms causing osteomalacia. Surgical intervention is clearly the treatment of choice. Promising non-surgical methods include treatment with burosumab and somatostatin analogues.

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Journal
Modern Rheumatology Journal
Published
2024-10-19
DOI
https://doi.org/10.14412/1996-7012-2024-5-7-15
Primary Topic
Parathyroid Disorders and Treatments
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article
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article

Tumor-induced phosphopenic osteomalacia: modern approaches to diagnostics and treatment

Н. А. Демидова, А. А. Клименко, А. А. Кондрашов, D. Yu. Andriyashkina et al.
Modern Rheumatology Journal
Parathyroid Disorders and Treatments
article

Tumor-induced phosphopenic osteomalacia: modern approaches to diagnostics and treatment

Н. А. Демидова, А. А. Клименко, А. А. Кондрашов, D. Yu. Andriyashkina, Yu. M. Saakyan
article en

Abstract

Phosphopenic osteomalacia (PPOM) is a rare variant of paraneoplastic syndrome caused by tumor synthesis of fibroblast growth factor 23 (FGF23). FGF23 secretion leads to a decrease in phosphate reabsorption and calcitriol levels, which leads to the development of severe hypophosphataemia and hypocalcaemia. FGF23 synthesis is predominantly associated with benign mesenchymal tumors, but has also been described in malignant neoplasms. The main clinical manifestations of PPOM are generalized myalgias and myopathy, ostealgia, pathological fractures, etc. The diagnosis of the disease requires a step-by-step investigation using somatostatin receptor-based imaging techniques, as these have the highest sensitivity for the detection of neoplasms causing osteomalacia. Surgical intervention is clearly the treatment of choice. Promising non-surgical methods include treatment with burosumab and somatostatin analogues.

Modern Rheumatology JournalVol. 18(5)
Pirogov Russian National Research Medical University (RU), Ministry of Health of the Russian Federation (RU)
Good health and well-being
Openalex Percentile: Top 77%
Parathyroid Disorders and Treatments
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Tumor-induced phosphopenic osteomalacia: modern approaches to diagnostics and treatment — Н. А. Демидова, А. А. Клименко, et al. · Modern Rheumatology Journal (2024) | TGRS Research Map | TGRS