Clinical Presentation and Treatment Outcomes of Children and Adolescents With Pheochromocytoma and Paraganglioma in a Single Center in Korea
PurposePheochromocytoma (PCC) and paraganglioma (PGL) (PPGL) are rare neuroendocrine tumors, and data on managing these conditions in children and adolescents are lacking. The objective of this study was to demonstrate the clinical presentation and treatment outcomes in children and adolescents with...
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Frontiers Media S.A.
2021-01-01
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| Series: | Frontiers in Endocrinology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fendo.2020.610746/full |
| _version_ | 1818558794475503616 |
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| author | Hyojung Park Min-Sun Kim Jiwon Lee Jung-Han Kim Byong Chang Jeong Sanghoon Lee Suk-Koo Lee Sung Yoon Cho Dong-Kyu Jin |
| author_facet | Hyojung Park Min-Sun Kim Jiwon Lee Jung-Han Kim Byong Chang Jeong Sanghoon Lee Suk-Koo Lee Sung Yoon Cho Dong-Kyu Jin |
| author_sort | Hyojung Park |
| collection | DOAJ |
| description | PurposePheochromocytoma (PCC) and paraganglioma (PGL) (PPGL) are rare neuroendocrine tumors, and data on managing these conditions in children and adolescents are lacking. The objective of this study was to demonstrate the clinical presentation and treatment outcomes in children and adolescents with PPGL in a single tertiary care center in Korea.MethodsThis retrospective study included 23 patients diagnosed with PCC (n = 14) and PGL (n = 9) before the age of 21 at Samsung Medical Center (from June 1994 to June 2019). We describe age, gender, family history, clinical characteristics, laboratory findings, pathologic findings, therapeutic approaches, and treatment outcomes.ResultsOf the 23 patients, 14 had PCC and nine had PGL. The median age at diagnosis was 16.8 years (range, 6.8–20.8 years). The common presenting symptoms were hypertension (n = 10), headache (n = 9), palpitation (n = 4), and sweating (n = 4). The plasma or 24-hour urine catecholamine and/or metabolite concentrations were markedly elevated in 22 patients with PPGL, but were normal in one patient with carotid body PGL. All tumors were visualized on computed tomography. Genetic tests were performed in 15 patients, and seven patients showed mutations in RET (n = 3), SDHB (n = 3), and VHL (n = 1). All patients underwent surgery, and complete excision was performed successfully. Three patients with metastasis underwent postoperative adjuvant therapy.ConclusionThis study suggests that pediatric PPGL tends to be extra-adrenal and bilateral and shows a higher potential for genetic mutations. Considering the hereditary predisposition of pediatric PPGL, genetic screening tests are strongly recommended, and lifelong follow-up is needed to detect recurrence and metastasis. Further research with a larger sample size and routine genetic screening is needed to better understand the genetic conditions and long-term prognosis of PPGL. |
| first_indexed | 2024-12-14T00:16:56Z |
| format | Article |
| id | doaj.art-496b575e247842ac8e72eca9b04cd23d |
| institution | Directory Open Access Journal |
| issn | 1664-2392 |
| language | English |
| last_indexed | 2024-12-14T00:16:56Z |
| publishDate | 2021-01-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Endocrinology |
| spelling | doaj.art-496b575e247842ac8e72eca9b04cd23d2022-12-21T23:25:27ZengFrontiers Media S.A.Frontiers in Endocrinology1664-23922021-01-011110.3389/fendo.2020.610746610746Clinical Presentation and Treatment Outcomes of Children and Adolescents With Pheochromocytoma and Paraganglioma in a Single Center in KoreaHyojung Park0Min-Sun Kim1Jiwon Lee2Jung-Han Kim3Byong Chang Jeong4Sanghoon Lee5Suk-Koo Lee6Sung Yoon Cho7Dong-Kyu Jin8Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South KoreaDepartment of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South KoreaDepartment of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South KoreaDepartment of Pediatric Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South KoreaDepartment of Urology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South KoreaDepartment of Pediatric Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South KoreaDepartment of Pediatric Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South KoreaDepartment of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South KoreaDepartment of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South KoreaPurposePheochromocytoma (PCC) and paraganglioma (PGL) (PPGL) are rare neuroendocrine tumors, and data on managing these conditions in children and adolescents are lacking. The objective of this study was to demonstrate the clinical presentation and treatment outcomes in children and adolescents with PPGL in a single tertiary care center in Korea.MethodsThis retrospective study included 23 patients diagnosed with PCC (n = 14) and PGL (n = 9) before the age of 21 at Samsung Medical Center (from June 1994 to June 2019). We describe age, gender, family history, clinical characteristics, laboratory findings, pathologic findings, therapeutic approaches, and treatment outcomes.ResultsOf the 23 patients, 14 had PCC and nine had PGL. The median age at diagnosis was 16.8 years (range, 6.8–20.8 years). The common presenting symptoms were hypertension (n = 10), headache (n = 9), palpitation (n = 4), and sweating (n = 4). The plasma or 24-hour urine catecholamine and/or metabolite concentrations were markedly elevated in 22 patients with PPGL, but were normal in one patient with carotid body PGL. All tumors were visualized on computed tomography. Genetic tests were performed in 15 patients, and seven patients showed mutations in RET (n = 3), SDHB (n = 3), and VHL (n = 1). All patients underwent surgery, and complete excision was performed successfully. Three patients with metastasis underwent postoperative adjuvant therapy.ConclusionThis study suggests that pediatric PPGL tends to be extra-adrenal and bilateral and shows a higher potential for genetic mutations. Considering the hereditary predisposition of pediatric PPGL, genetic screening tests are strongly recommended, and lifelong follow-up is needed to detect recurrence and metastasis. Further research with a larger sample size and routine genetic screening is needed to better understand the genetic conditions and long-term prognosis of PPGL.https://www.frontiersin.org/articles/10.3389/fendo.2020.610746/fullpheochromocytomaparagangliomaadrenal masshypertensionpediatrics |
| spellingShingle | Hyojung Park Min-Sun Kim Jiwon Lee Jung-Han Kim Byong Chang Jeong Sanghoon Lee Suk-Koo Lee Sung Yoon Cho Dong-Kyu Jin Clinical Presentation and Treatment Outcomes of Children and Adolescents With Pheochromocytoma and Paraganglioma in a Single Center in Korea Frontiers in Endocrinology pheochromocytoma paraganglioma adrenal mass hypertension pediatrics |
| title | Clinical Presentation and Treatment Outcomes of Children and Adolescents With Pheochromocytoma and Paraganglioma in a Single Center in Korea |
| title_full | Clinical Presentation and Treatment Outcomes of Children and Adolescents With Pheochromocytoma and Paraganglioma in a Single Center in Korea |
| title_fullStr | Clinical Presentation and Treatment Outcomes of Children and Adolescents With Pheochromocytoma and Paraganglioma in a Single Center in Korea |
| title_full_unstemmed | Clinical Presentation and Treatment Outcomes of Children and Adolescents With Pheochromocytoma and Paraganglioma in a Single Center in Korea |
| title_short | Clinical Presentation and Treatment Outcomes of Children and Adolescents With Pheochromocytoma and Paraganglioma in a Single Center in Korea |
| title_sort | clinical presentation and treatment outcomes of children and adolescents with pheochromocytoma and paraganglioma in a single center in korea |
| topic | pheochromocytoma paraganglioma adrenal mass hypertension pediatrics |
| url | https://www.frontiersin.org/articles/10.3389/fendo.2020.610746/full |
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