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

• Ku EJ
• Kim KJ
• Kim JH
• Kim MK
• Ahn CH
• Lee KA
• Lee SH
• Lee YB
• Park KH
• Choi YM
• Hong N
• Hong AR
• Kang SW
• Park BK
• Seong MW
• Kim M
• Jung KC
• Jung CK
• Cho YS
• Paeng JC
• Kim JH
• Ryu OH
• Rhee Y
• Kim CH
• Lee EJ

• KMID: 2515458
• DOI: http://doi.org/10.3803/EnM.2020.908

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

Figure

• 

  Fig. 1 Proposed clinical algorithm for nuclear (molecular) imaging studies for pheochromocytoma/paraganglioma. PPGL, pheochromocytoma/paraganglioma; PHEO, pheochromocytoma; HNPGL, head and neck paraganglioma; SDH, succinate dehydrogenase; VHL, von Hippel-Lindau; RET, rearranged during transfection; NF1, neurofibromatosis 1; MAX, myc-associated protein X; HIF2A, hypoxia-inducible factor 2A; PHD1/2, prolyl hydroxylase domain 1/2; FH, fumarate hydratase; 123I-MIBG, 123I-metaiodobenzylguanidine; 68Ga-DOTA-SSA, gallium 68 1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic acid-somatostatin receptor analogs; 18F-DOPA, fluorine-18-L-fluorodihydroxyphenylalanine; 18F-FDG, 18F-fluorodeoxyglucose; PRRT, peptide receptor radiotherapy.

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