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Erschienen in: Clinical Orthopaedics and Related Research® 11/2013

01.11.2013 | Clinical Research

The Phosphaturic Mesenchymal Tumor: Why is Definitive Diagnosis and Curative Surgery Often Delayed?

verfasst von: Cameron K. Ledford, MD, Nicole A. Zelenski, BS, Diana M. Cardona, MD, Brian E. Brigman, MD, PhD, William C. Eward, DVM, MD

Erschienen in: Clinical Orthopaedics and Related Research® | Ausgabe 11/2013

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Abstract

Background

Tumor-induced osteomalacia is a paraneoplastic syndrome resulting in renal phosphate wasting and decreased bone mineralization. Phosphaturic mesenchymal tumors represent a rare etiology of tumor-induced osteomalacia. Nonspecific symptoms of fatigue, bone pain, and musculoskeletal weakness make the diagnosis elusive and lead to a delay in surgical treatment.

Questions/purposes

In this case series, the following three questions were asked: (1) How do the clinical presentation and features of phosphaturic mesenchymal tumors delay the diagnosis? (2) What is the clinical course after surgical treatment of phosphaturic mesenchymal tumors? (3) How frequently do phosphaturic mesenchymal tumors recur and are there factors associated with recurrence?

Methods

This study retrospectively reviewed the cases of five adults diagnosed and treated for phosphaturic mesenchymal tumors. Patients were identified through an internal orthopaedic oncology database with clinical, surgical, and histologic data obtained through a systematic chart review.

Results

Five patients presented with a long-standing history of osteomalacia, generalized fatigue, pain, and weakness before the diagnosis was reached at an average of 7.2 years (range, 2–12 years) after initial symptom onset. The diagnosis appeared to be delayed owing to the cryptic medical presentation, difficulty in locating tumor by imaging, and confirming histologic appearance. Two patients treated with wide surgical resection did not experience recurrence compared with three patients who did show recurrent signs and symptoms after marginal excision. A postoperative increase in fibroblast-derived growth factor-23 was associated with recurrent disease.

Conclusions

Although uncommon, the diagnosis of phosphaturic mesenchymal tumor should be considered in any patient who presents with hypophosphaturic osteomalacia and no other physiologic cause. Definitive treatment is early, wide surgical resection.

Level of Evidence

Level IV, therapeutic study. See the Instructions for Authors for a complete description of levels of evidence.
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Metadaten
Titel
The Phosphaturic Mesenchymal Tumor: Why is Definitive Diagnosis and Curative Surgery Often Delayed?
verfasst von
Cameron K. Ledford, MD
Nicole A. Zelenski, BS
Diana M. Cardona, MD
Brian E. Brigman, MD, PhD
William C. Eward, DVM, MD
Publikationsdatum
01.11.2013
Verlag
Springer US
Erschienen in
Clinical Orthopaedics and Related Research® / Ausgabe 11/2013
Print ISSN: 0009-921X
Elektronische ISSN: 1528-1132
DOI
https://doi.org/10.1007/s11999-013-3178-1

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