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

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.

A Prospective Comparative Study of 18F-FDOPA PET/CT Versus 123I-MIBG Scintigraphy With SPECT/CT for the Diagnosis of Pheochromocytoma and Paraganglioma

“…Given these clinical scenarios, our study’s findings support the suggestion that 18 F-FDOPA PET/CT should be the preferred initial modality compared with 123 I-MIBG scintigraphy with SPECT/CT for patients with high-risk PPGL or those with known genetic mutations. Nevertheless, contemplating the treatment with 131 I-MIBG or peptide receptor radionuclide therapy, 123 I-MIBG SPECT or 68 Ga-DOTA-SSA PET/CT might take precedence, in accordance with the guidelines 1 …”

“…This prospective, noninferiority, head-to-head comparative study was conducted at a single institution. Participants were included based on one of the following criteria: (1) suspected PPGL based on a biochemical screening, including 24-hour urinary normetanephrine, metanephrine, norepinephrine, and epinephrine, and/or plasma-free concentrations of normetanephrine and metanephrine conducted within the past 6 months; (2) an adrenal incidentaloma, suggestive of PPGL on anatomical imaging (CT or MRI) performed within the past 6 months; (3) characteristic symptoms and signs of PPGL; and (4) suspected of recurrence or metastases of known PPGL. The study excluded pregnant and lactating women, individuals aged less than 15 years, patients with a serum creatinine level greater than 3 mg/dL due to chronic renal failure, and those with a second primary cancer that was not in complete remission.…”

“…Nevertheless, contemplating the treatment with 131 I-MIBG or peptide receptor radionuclide therapy, 123 I-MIBG SPECT or 68 Ga-DOTA-SSA PET/CT might take precedence, in accordance with the guidelines. 1 As commercial supply improves in Europe and other regions, the availability of 18 F-FDOPA PET/CT is on the rise. However, its recommendation is questionable in patients with SDHx mutations, due to reports of a false-negative rate as high as 39%.…”

“…Pheochromocytomas and paragangliomas (PPGLs) are chromaffin cells-derived tumors that originate from the adrenal medulla (80%~85%) and the extra-adrenal sympathetic paraganglia in thorax, abdomen, and pelvis (15%~20%) or parasympathetic paraganglia in the head and neck region (~1%), respectively 1–3 . Pheochromocytomas and paragangliomas release catecholamines, causing symptom hypertension, headache, sweating, and palpitations 1–3 . Despite the rarity of PPGLs, their early diagnosis is crucial; undetected, they can have fatal consequences, particularly if they are not identified before surgery or delivery 1–3 …”

Evaluation and Management of Bone Health in Patients with Thyroid Diseases: A Position Statement of the Korean Thyroid Association

“…The grading system included the following types of evidence: well-designed randomized controlled trials, meta-analysis results, cohort studies, patient-control studies, and expert opinions on clinical experiences. The guideline committee used the grading levels of A, B, C, and E to grade the evidence supporting each recommendation, as described in previous publications ( Table 1 ) [ 12 , 13 ].…”