Endocrinol Metab.  2021 Apr;36(2):322-338. 10.3803/EnM.2020.908.

Diagnosis for Pheochromocytoma and Paraganglioma: A Joint Position Statement of the Korean Pheochromocytoma and Paraganglioma Task Force

Affiliations
  • 1Department of Internal Medicine, Chungbuk National University Hospital, Chungbuk National University College of Medicine, Cheongju, Korea
  • 2Department of Internal Medicine, Severance Hospital, Endocrine Research Institute, Yonsei University College of Medicine, Seoul, Korea
  • 3Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea
  • 4Division of Endocrinology and Metabolism, Department of Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea
  • 5Department of Internal Medicine, Keimyung University School of Medicine, Daegu, Korea
  • 6Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Korea
  • 7Division of Endocrinology and Metabolism, Department of Internal Medicine, Jeonbuk National University Medical School, Jeonju, Korea
  • 8Division of Endocrinology and Metabolism, Department of Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
  • 9Division of Endocrinology and Metabolism, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
  • 10Division of Endocrinology and Metabolism, Department of Internal Medicine, National Health Insurance Service Ilsan Hospital, Goyang, Korea
  • 11Department of Internal Medicine, Hallym University Dongtan Sacred Heart Hospital, Hallym University College of Medicine, Hwaseong, Korea
  • 12Department of Internal Medicine, Chonnam National University Medical School, Gwangju, Korea
  • 13Thyroid-Endocrine Surgery Division, Department of Surgery, Yonsei University College of Medicine, Seoul, Korea
  • 14Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
  • 15Department of Laboratory Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea
  • 16Department of Laboratory Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea
  • 17Department of Pathology, Seoul National University College of Medicine, Seoul, Korea
  • 18Department of Hospital Pathology, College of Medicine, The Catholic University of Korea, Seoul, Korea
  • 19Department of Nuclear Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
  • 20Department of Nuclear Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea
  • 21Department of Internal Medicine, Hallym University Chuncheon Sacred Heart Hospital, Hallym University College of Medicine, Chuncheon, Korea
  • 22Division of Endocrinology and Metabolism, Department of Internal Medicine, Sejong General Hospital, Bucheon, Korea

Abstract

Pheochromocytoma and paraganglioma (PPGLs) are rare catecholamine-secreting neuroendocrine tumors but can be life-threatening. Although most PPGLs are benign, approximately 10% have metastatic potential. Approximately 40% cases are reported as harboring germline mutations. Therefore, timely and accurate diagnosis of PPGLs is crucial. For more than 130 years, clinical, molecular, biochemical, radiological, and pathological investigations have been rapidly advanced in the field of PPGLs. However, performing diagnostic studies to localize lesions and detect metastatic potential can be still challenging and complicated. Furthermore, great progress on genetics has shifted the paradigm of genetic testing of PPGLs. The Korean PPGL task force team consisting of the Korean Endocrine Society, the Korean Surgical Society, the Korean Society of Nuclear Medicine, the Korean Society of Pathologists, and the Korean Society of Laboratory Medicine has developed this position statement focusing on the comprehensive and updated diagnosis for PPGLs.

Keyword

Pheochromocytoma; Paraganglioma; Diagnosis; Classification
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