耳鼻咽喉科臨床 補冊
Online ISSN : 2185-1557
Print ISSN : 0912-1870
ISSN-L : 0912-1870
浜松医科大学医学部耳鼻咽喉科・頭頸部外科学教室 峯田周幸教授退任記念論文集~症例から学ぶ~
手術的加療にて改善した側頭骨PMT(Phosphaturic Mesenchymal Tumor)例
大和谷 崇中西 啓遠藤 志織細川 誠二峯田 周幸
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2021 年 156 巻 p. 21-25

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Phosphaturic mesenchymal tumor (PMT) is a rare neoplasm that secretes fibroblast growth factor 23 (FGF23), which suppresses re-absorption of phosphorus in the renal tubules and leads to chronic hypophosphatemia, thereby resulting in tumor-induced osteomalacia (TIO). Herein, we report a patient who had suffered multiple fractures and was diagnosed as having PMT in his left temporal bone two years later the appearance of the first symptom. For his first consultation at our department, the patient was transported to the hospital on a stretcher, as he had found it difficult to turn over in bed due to generalized bone pain and multiple pathological fractures. Examination revealed that the tumor had already spread deep into the skull base; therefore, considering the general condition of the patient, we determined that radical surgery would be difficult. Transmastoid surgical debulking was performed three months after the first consultation at our department, and the patient’s quality of life improved after the surgery. However, 10 months after the surgical debulking procedure, since we did not observe any improvements in his clinical findings, we performed radical surgery together with the neurosurgeons, which yielded good results. This case suggests that radical surgery is essential for the treatment of PMT. Even in cases where radical surgery is initially deemed to be challenging, gradual steps towards radical surgical treatment may be possible.

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