Japan Endocrine Society Clinical Practice Guideline for the Diagnosis and Management of Pheochromocytoma and Paraganglioma 2025

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 <sup>123</sup>I-MIBG scintigraphy or <sup>18</sup>F-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 <sup>131</sup>I-MIBG or <sup>177</sup>Lu-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.

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Publication Details

Journal
Endocrine Journal
Published
2025-10-09
DOI
https://doi.org/10.1507/endocrj.ej25-0165
Citations
1
Primary Topic
Adrenal and Paraganglionic Tumors
Type
article
Field-Weighted Citation Impact
2.01

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article

Japan Endocrine Society Clinical Practice Guideline for the Diagnosis and Management of Pheochromocytoma and Paraganglioma 2025

Takamasa Ichijo, Nae Takizawa, Kazuhiro Takekoshi, Shigeatsu Hashimoto et al.
1 citations
Endocrine Journal
Adrenal and Paraganglionic Tumors
2.01
article

Japan Endocrine Society Clinical Practice Guideline for the Diagnosis and Management of Pheochromocytoma and Paraganglioma 2025

Takamasa Ichijo, Nae Takizawa, Kazuhiro Takekoshi, Shigeatsu Hashimoto, Shoichiro Izawa, Noboru Oriuchi, Takuyuki Katabami, Michio Otsuki, Masato Yonamine, Tomonobu Hasegawa, Noriko Kimura, Mika Tsuiki, Masakatsu Sone, Kanako Tanase‐Nakao, Akiyo Tanabe, Hidefumi Kinoshita, Hirotaka Shibata, Masanori Murakami, Mitsuhide Naruse, Seigo Kinuya, Koshiro Nishimoto, Katsutoshi Takahashi, Y. Nakamura
article en
1 citations

Abstract

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 <sup>123</sup>I-MIBG scintigraphy or <sup>18</sup>F-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 <sup>131</sup>I-MIBG or <sup>177</sup>Lu-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.

Endocrine JournalVol. 73(1)
Kanazawa University (JP), Kansai Medical University (JP), Fukushima Medical University (JP), University of Aizu (JP), University of Tsukuba (JP), St. Marianna University School of Medicine (JP), Oita University (JP), Keio University (JP), Kyoto Medical Center (JP), National Center for Global Health and Medicine (JP), Ijinkai Takeda General Hospital (JP), Yokohama City Seibu Hospital (JP), Showa General Hospital (JP), National Center For Child Health and Development (JP), Saitama International Medical Center (JP), Tokyo Women's Medical University (JP), Saiseikai Yokohama Eastern Hospital (JP), Hakodate National Hospital (JP), Saiseikai Ibaraki Hospital (JP), Tottori University (JP), Tohoku Medical and Pharmaceutical University (JP), Saitama Medical University (JP)
Japan Agency for Medical Research and Development, National Center for Global Health and Medicine, Keio University
Openalex Percentile: Top 11%
Adrenal and Paraganglionic Tumors
2.01
DOIOpen Access PDFOpenAlex
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