| 論文種別 | 原著(症例報告除く) |
| 言語種別 | 英語 |
| 査読の有無 | その他(不明) |
| 表題 | Japan Endocrine Society Clinical Practice Guideline for the Diagnosis and Management of Pheochromocytoma and Paraganglioma 2025. |
| 掲載誌名 | 正式名：Endocrine journal 略 称：Endocr J ISSNコード：13484540/09188959 |
| 掲載区分 | 国外 |
| 巻・号・頁 | pp.Online ahead of print |
| 著者・共著者 | Akiyo Tanabe, Takuyuki Katabami, Shigeatsu Hashimoto, Shoichiro Izawa, Takamasa Ichijo, Michio Otsuki, Noboru Oriuchi, Seigo Kinuya, Hidefumi Kinoshita, Noriko Kimura, Hirotaka Shibata, Masakatsu Sone, Katsutoshi Takahashi, Nae Takizawa, Kazuhiro Takekoshi, Mika Tsuiki, Kanako Tanase-Nakao, Yasuhiro Nakamura, Koshiro Nishimoto, Tomonobu Hasegawa, Masanori Murakami, Masato Yonamine, Mitsuhide Naruse |
| 発行年月 | 2025/10 |
| 概要 | Pheochromocytomas and paragangliomas are characterized by two key features: endocrine disorders with excessive catecholamine secretion and a hereditary or metastatic nature, making early diagnosis and treatment crucial. This clinical practice guideline is a revision of the 2018 edition, which considers recent advances in clinical practice and changes to the health insurance coverage in Japan. Patients presenting with symptoms such as palpitations, headaches, hypertension, or abdominal tumors should undergo screening and confirmation with measurement of fractionated catecholamines and their metabolites in the blood and urine. When the tumor is located in the adrenal glands, it is diagnosed as a pheochromocytoma; when it is located outside the adrenal gland and confirmed by 123I-MIBG scintigraphy or 18F-FDG PET, it is diagnosed as a paraganglioma. Treatment begins with inhibiting catecholamine action using α-blockers, and if that is insufficient, metyrosine is used in combination, followed by laparoscopic tumor removal. Given the metastatic potential, long-term postoperative follow-up is essential. Even in cases of metastasis, tumor debulking should be considered. Treatment options are selected based on the amount of remaining tumor, symptom severity, and lesion progression, including CVD chemotherapy or radionuclide therapies such as 131I-MIBG or 177Lu-DOTATATE. Genetic testing guides the management of different variants, and significant progress has been made in molecularly targeted drug trials. Therefore, further advances in individualized and long-term management are required. |
| DOI | 10.1507/endocrj.EJ25-0165 |
| PMID | 41083371 |