Pheochromocytoma and paraganglioma in Fontan patients

Common more than expected

Research output: Contribution to journalArticleResearchpeer-review

Abstract

Objective: Pheochromocytoma and paraganglioma (extra-adrenal pheochromocytoma) are rare neuroendocrine tumors that arise from the neuroendocrine cells. Chronic hypoxia is known as a possible cause, and a strong link between cyanotic congenital heart disease and these tumors has been reported. However, reports of phechromocytoma/paraganglioma in Fontan patients were scarce. We herein report seven cases of phechromocytoma/paraganglioma after Fontan operation at a single tertiary center. Methods: We retrospectively reviewed medical records and imaging studies who diagnosed as phechromocytoma/paraganglioma after Fontan operation in Seoul National University Children's Hospital. Results: Seven patients were identified during follow-up after Fontan operation, and the prevalence was 2.5% among Fontan patients greater than 10 years old on active follow-up. Three patients were diagnosed as phechromocytoma and 4 patients as paraganglioma. Median time interval between Fontan operation and diagnosis of pheochromocytoma/paraganglioma was 21.4 years (range, 10.4–29.7 years). Resting percutaneous oxygen saturation varied from 77% to 94%. All patients underwent complete tumor resection. Phechromocytoma recurred in two patients, of whom one patient died at the age of 18 years due to the tumor progression with multiple metastasis and aggravation of heart failure with profound cyanosis. Pheochromocytoma/paraganglioma developed after hepatocellular carcinoma in two patients. Conclusion: Phechromocytoma/paraganglioma could occur in Fontan patients more than expected. Because it is curable by tumor resection during its early phase, early diagnosis and treatment of pheochromocytoma are crucial in Fontan patients not to make hemodynamic deterioration and aggravation of heart failure.

Original languageEnglish
Pages (from-to)608-616
Number of pages9
JournalCongenital Heart Disease
Volume13
Issue number4
DOIs
StatePublished - 1 Jul 2018

Fingerprint

Paraganglioma
Pheochromocytoma
Fontan Procedure
Heart Failure
Neoplasms
Neuroendocrine Cells
Cyanosis
Heart Neoplasms
Neuroendocrine Tumors
Diagnostic Imaging
Medical Records
Early Diagnosis
Hepatocellular Carcinoma
Heart Diseases
Hemodynamics

Keywords

  • Fontan procedure
  • cyanosis
  • heart diseases
  • pheochromocytoma

Cite this

@article{0cc9122eedac4b0682369f728276c37f,
title = "Pheochromocytoma and paraganglioma in Fontan patients: Common more than expected",
abstract = "Objective: Pheochromocytoma and paraganglioma (extra-adrenal pheochromocytoma) are rare neuroendocrine tumors that arise from the neuroendocrine cells. Chronic hypoxia is known as a possible cause, and a strong link between cyanotic congenital heart disease and these tumors has been reported. However, reports of phechromocytoma/paraganglioma in Fontan patients were scarce. We herein report seven cases of phechromocytoma/paraganglioma after Fontan operation at a single tertiary center. Methods: We retrospectively reviewed medical records and imaging studies who diagnosed as phechromocytoma/paraganglioma after Fontan operation in Seoul National University Children's Hospital. Results: Seven patients were identified during follow-up after Fontan operation, and the prevalence was 2.5{\%} among Fontan patients greater than 10 years old on active follow-up. Three patients were diagnosed as phechromocytoma and 4 patients as paraganglioma. Median time interval between Fontan operation and diagnosis of pheochromocytoma/paraganglioma was 21.4 years (range, 10.4–29.7 years). Resting percutaneous oxygen saturation varied from 77{\%} to 94{\%}. All patients underwent complete tumor resection. Phechromocytoma recurred in two patients, of whom one patient died at the age of 18 years due to the tumor progression with multiple metastasis and aggravation of heart failure with profound cyanosis. Pheochromocytoma/paraganglioma developed after hepatocellular carcinoma in two patients. Conclusion: Phechromocytoma/paraganglioma could occur in Fontan patients more than expected. Because it is curable by tumor resection during its early phase, early diagnosis and treatment of pheochromocytoma are crucial in Fontan patients not to make hemodynamic deterioration and aggravation of heart failure.",
keywords = "Fontan procedure, cyanosis, heart diseases, pheochromocytoma",
author = "Song, {Mi Kyoung} and Kim, {Gi Beom} and Eunjung Bae and Lee, {Young Ah} and Hyun-Young Kim and Seung-Kee Min and Kim, {Jung Hee} and Won, {Jae Kyung}",
year = "2018",
month = "7",
day = "1",
doi = "10.1111/chd.12625",
language = "English",
volume = "13",
pages = "608--616",
journal = "Congenital Heart Disease",
issn = "1747-079X",
publisher = "Wiley-Blackwell Publishing Ltd",
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}

Pheochromocytoma and paraganglioma in Fontan patients : Common more than expected. / Song, Mi Kyoung; Kim, Gi Beom; Bae, Eunjung; Lee, Young Ah; Kim, Hyun-Young; Min, Seung-Kee; Kim, Jung Hee; Won, Jae Kyung.

In: Congenital Heart Disease, Vol. 13, No. 4, 01.07.2018, p. 608-616.

Research output: Contribution to journalArticleResearchpeer-review

TY - JOUR

T1 - Pheochromocytoma and paraganglioma in Fontan patients

T2 - Common more than expected

AU - Song, Mi Kyoung

AU - Kim, Gi Beom

AU - Bae, Eunjung

AU - Lee, Young Ah

AU - Kim, Hyun-Young

AU - Min, Seung-Kee

AU - Kim, Jung Hee

AU - Won, Jae Kyung

PY - 2018/7/1

Y1 - 2018/7/1

N2 - Objective: Pheochromocytoma and paraganglioma (extra-adrenal pheochromocytoma) are rare neuroendocrine tumors that arise from the neuroendocrine cells. Chronic hypoxia is known as a possible cause, and a strong link between cyanotic congenital heart disease and these tumors has been reported. However, reports of phechromocytoma/paraganglioma in Fontan patients were scarce. We herein report seven cases of phechromocytoma/paraganglioma after Fontan operation at a single tertiary center. Methods: We retrospectively reviewed medical records and imaging studies who diagnosed as phechromocytoma/paraganglioma after Fontan operation in Seoul National University Children's Hospital. Results: Seven patients were identified during follow-up after Fontan operation, and the prevalence was 2.5% among Fontan patients greater than 10 years old on active follow-up. Three patients were diagnosed as phechromocytoma and 4 patients as paraganglioma. Median time interval between Fontan operation and diagnosis of pheochromocytoma/paraganglioma was 21.4 years (range, 10.4–29.7 years). Resting percutaneous oxygen saturation varied from 77% to 94%. All patients underwent complete tumor resection. Phechromocytoma recurred in two patients, of whom one patient died at the age of 18 years due to the tumor progression with multiple metastasis and aggravation of heart failure with profound cyanosis. Pheochromocytoma/paraganglioma developed after hepatocellular carcinoma in two patients. Conclusion: Phechromocytoma/paraganglioma could occur in Fontan patients more than expected. Because it is curable by tumor resection during its early phase, early diagnosis and treatment of pheochromocytoma are crucial in Fontan patients not to make hemodynamic deterioration and aggravation of heart failure.

AB - Objective: Pheochromocytoma and paraganglioma (extra-adrenal pheochromocytoma) are rare neuroendocrine tumors that arise from the neuroendocrine cells. Chronic hypoxia is known as a possible cause, and a strong link between cyanotic congenital heart disease and these tumors has been reported. However, reports of phechromocytoma/paraganglioma in Fontan patients were scarce. We herein report seven cases of phechromocytoma/paraganglioma after Fontan operation at a single tertiary center. Methods: We retrospectively reviewed medical records and imaging studies who diagnosed as phechromocytoma/paraganglioma after Fontan operation in Seoul National University Children's Hospital. Results: Seven patients were identified during follow-up after Fontan operation, and the prevalence was 2.5% among Fontan patients greater than 10 years old on active follow-up. Three patients were diagnosed as phechromocytoma and 4 patients as paraganglioma. Median time interval between Fontan operation and diagnosis of pheochromocytoma/paraganglioma was 21.4 years (range, 10.4–29.7 years). Resting percutaneous oxygen saturation varied from 77% to 94%. All patients underwent complete tumor resection. Phechromocytoma recurred in two patients, of whom one patient died at the age of 18 years due to the tumor progression with multiple metastasis and aggravation of heart failure with profound cyanosis. Pheochromocytoma/paraganglioma developed after hepatocellular carcinoma in two patients. Conclusion: Phechromocytoma/paraganglioma could occur in Fontan patients more than expected. Because it is curable by tumor resection during its early phase, early diagnosis and treatment of pheochromocytoma are crucial in Fontan patients not to make hemodynamic deterioration and aggravation of heart failure.

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U2 - 10.1111/chd.12625

DO - 10.1111/chd.12625

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JF - Congenital Heart Disease

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