PTH like substance secreting mesenchymal tumor causing oncogenic osteomalacia; unravelling the difficulties in localization – A report of 2 cases

Oncogenic osteomalacia is a rare paraneoplastic association of Phosphaturic mesenchymal tumor (PMT) secreting excessive levels of a PTH like substance. They usually remain undiagnosed and patients suffer for years. The rarity of this tumor and its non-specific clinical presentations poses great chal...

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Veröffentlicht in:Journal of orthopaedics 2024-12, Vol.58, p.123-127
Hauptverfasser: Jijulal, C.U., Sreedharan, Sreeja, Naveen P., Gopinath, Surendran, Sibin, Patinharayil, Gopinathan, Fazil V.V., Muhammed, K.V., Nikhil, Sudhan S., Ram
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Sprache:eng
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Zusammenfassung:Oncogenic osteomalacia is a rare paraneoplastic association of Phosphaturic mesenchymal tumor (PMT) secreting excessive levels of a PTH like substance. They usually remain undiagnosed and patients suffer for years. The rarity of this tumor and its non-specific clinical presentations poses great challenge to the treating surgeons. Its management is poorly described in literature. We report two of such rare cases without much diagnostic delay. We had 2 cases; A 53-year-old south east Asian male with 6 months of debilitating pain over multiple sites, and another 44-year-old male patient with complaints of low back ache, and pain over both lower and upper limbs for 1.5 years. Both had low serum phosphorus and elevated FGF-23 values, but all other parameters were normal. A PMT was suspected and confirmed on a Ga68- DOTATOC scan in both cases, and on complete excision, their symptoms and the altered blood parameters got normalized. Histology was consistent with PMT. Accurate and timely diagnosis of a PMT with non-specific features are extremely challenging, but not without solutions. Even though a tumor of rarity, with the appropriate imaging modalities like Ga68- DOTATOC scan, and estimation of FGF-23 and serum phosphorus levels, they can be diagnosed. Once identified, complete removal is often curative within a few months.
ISSN:0972-978X
0972-978X
DOI:10.1016/j.jor.2024.06.034