Phosphaturic Mesenchymal Tumors with or without Phosphate Metabolism Derangements

Phosphaturic mesenchymal tumors (PMT) are rare neoplasms, which can give rise to a multifaceted syndrome, otherwise called tumor-induced osteomalacia (TIO). Localizing these tumors is crucial to obtain a cure for the phosphate metabolism derangement, which is often the main cause leading the patient to seek medical help, because of invalidating physical and neuromuscular symptoms. A proportion of these tumors is completely silent and may grow unnoticed, unless they become large enough to produce pain or discomfort. FGF-23 can be produced by several benign or malignant PMTs. The phosphate metabolism, radiology and histology of these rare tumors must be collectively assessed by a multidisciplinary team aimed at curing the disease locally and improving patients' quality of life. This narrative review, authored by multiple specialists of a tertiary care hospital center, will describe endocrine, radiological and histological features of these tumors, as well as present surgical and interventional strategies to manage PMTs.

Automated summary

Phosphaturic mesenchymal tumors (PMTs) can cause tumor-induced osteomalacia (TIO) due to phosphate metabolism derangement. Locating these tumors is crucial for a cure. Some tumors may not produce symptoms until they become large enough. FGF-23 can be produced by both benign and malignant PMTs. A multidisciplinary team is necessary to assess the metabolism, radiology, and histology of these rare tumors and improve patients' quality of life. This review provides insights into the endocrine, radiological, and histological features of PMTs, as well as surgical and interventional strategies for management.