Endocrinology and Metabolism

Special Article

Adrenal gland
Diagnosis for Pheochromocytoma and Paraganglioma: A Joint Position Statement of the Korean Pheochromocytoma and Paraganglioma Task Force: Eu Jeong Ku, Kyoung Jin Kim, Jung Hee Kim, Mi Kyung Kim, Chang Ho Ahn, Kyung Ae Lee, Seung Hun Lee, You-Bin Lee, Kyeong Hye Park, Yun Mi Choi, Namki Hong, A Ram Hong, Sang-Wook Kang, Byung Kwan Park, Moon-Woo Seong, Myungshin Kim, Kyeong Cheon Jung, Chan Kwon Jung, Young Seok Cho, Jin Chul Paeng, Jae Hyeon Kim, Ohk-Hyun Ryu, Yumie Rhee, Chong Hwa Kim, Eun Jig Lee; Endocrinol Metab. 2021;36(2):322-338. Published online April 6, 2021; DOI: https://doi.org/10.3803/EnM.2020.908

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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.

Citations

Citations to this article as recorded by

A Prospective Comparative Study of 18F-FDOPA PET/CT Versus 123I-MIBG Scintigraphy With SPECT/CT for the Diagnosis of Pheochromocytoma and Paraganglioma
Changhwan Sung, Hyo Sang Lee, Dong Yun Lee, Yong-il Kim, Jae Eun Kim, Sang Ju Lee, Seung Jun Oh, Tae-Yon Sung, Yu-Mi Lee, Young Hoon Kim, Beom-Jun Kim, Jung-Min Koh, Seung Hun Lee, Jin-Sook Ryu
Clinical Nuclear Medicine.2024; 49(1): 27. CrossRef
Preoperative prediction of metastatic pheochromocytoma and paraganglioma using clinical, genetic, and biochemical markers: A cohort study
Seung Shin Park, Chang Ho Ahn, Seunghoo Lee, Woochang Lee, Won Woong Kim, Yu‐Mi Lee, Su Jin Kim, Tae‐Yon Sung, Kyu Eun Lee, Jung Hee Kim, Seung Hun Lee, Jung‐Min Koh
Journal of Internal Medicine.2024;[Epub] CrossRef
Evaluation and Management of Bone Health in Patients with Thyroid Diseases: A Position Statement of the Korean Thyroid Association
A Ram Hong, Ho-Cheol Kang
Endocrinology and Metabolism.2023; 38(2): 175. CrossRef
Lesion-based indicators predict long-term outcomes of pheochromocytoma and paraganglioma– SIZEPASS
Helena Hanschell, Salvador Diaz-Cano, Alfredo Blanes, Nadia Talat, Gabriele Galatá, Simon Aylwin, Klaus Martin Schulte
Frontiers in Endocrinology.2023;[Epub] CrossRef
Interleukin-6-producing paraganglioma as a rare cause of systemic inflammatory response syndrome: a case report
Yin Young Lee, Seung Min Chung
Journal of Yeungnam Medical Science.2023; 40(4): 435. CrossRef
(Extremely rare intrapericardial location of paraganglioma)
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Chae Moon Hong, Young Jin Jeong, Hae Won Kim, Byeong-Cheol Ahn
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Change of Computed Tomography-Based Body Composition after Adrenalectomy in Patients with Pheochromocytoma
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Evaluation and Management of Bone Health in Patients with Thyroid Diseases: a Position Statement from the Korean Thyroid Association
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Case Report

A Case of Multiple Endocrine Neoplasia Type 1.: Soon Jib Yoo, Bong Yun Cha, Kwang Woo Lee, Ho Young Son, Sung Koo Kang, Jong Man Won, Young Seok Cho, Yong Jik Sung, Kyung Sub Song, Jin Young Yoo, Chul Woo Lee; J Korean Endocr Soc. 1997;12(1):111-119. Published online January 1, 2001

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Abstract PDF: Multiple endocrine neoplasia type 1 (MEN 1) is the association of neoplastic transformation of parathyroid, pituitary, and pancreatic islet cells. This syndrome is inherited as an autosomal dominant trait. A 38-year-old woman presented with general weakness and mental changes. She had experienced same symptoms 7 years ago, after then her weight gradually increased. Insulinoma was suspected by markedly decreased blood glucose level (20mg/dL) and the increased insulin/glucose ratio (0.43) that sampled in emergency room at the time of mental change. Unusually large pancreatic tail mass and Lt, adrenal gland mass were detected by abdominal CT. Percutaneous transhepatic portal vein catheterization with insulin sampling showed sudden step up of insulin/glucose ratio at the middle portion of pancreas. Measuring of basal pituitary hormones as a screening procedure of MEN showed increased basal prolactin level. Combined pituitary stimulation test showed blunted response of prolactin to TRH and sellar magnetic resonance imaging showed intrasellar mass. Thyroid nodule was palpated on her anterior neck. Thyroid scintigram showed cold nodule, and there was no lymphadenopathy around the nodule by the thyroid sonogram. Fine needle aspiration cytology showed benign hyperplastic follicular cells only. Serum ionized calcium and parathyroid hormone level were normal. Under the impression of MEN type I, the distal pancreatectomy, splenectomy, Lt. thyroid lobectomy and Lt. adrenalectomy was performed at the same time. Histologic examination of the surgically removed tissues revealed pancreas islet cell tumor, adrenal cortical adenoma and thyroid adenoma. Transient hyperglycemia was developed after surgical intervention, but thereafter she never felt any symptoms of hypoglycemia till now and her blood glucose showed completely normal level with oral glucose tolerance tests. We present this case with a review of literature.

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