Local-Regional Recurrence of Pheochromocytoma/Paraganglioma: Characteristics, Risk Factors and Outcomes

ObjectiveTo study the characteristics, risk factors, and outcomes of local-regional recurrence of pheochromocytoma and paraganglioma (PPGL).MethodsClinical data of 96 PPGL patients with local-regional recurrence and 112 patients without recurrence were retrospectively analyzed.ResultsRecurrent patie...

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Autores principales:	Yunying Cui, Xiaosen Ma, Yinjie Gao, Xiaoyan Chang, Shi Chen, Lin Lu, Anli Tong
Formato:	article
Lenguaje:	EN
Publicado:	Frontiers Media S.A. 2021
Materias:	recurrence characteristics risk factors outcomes pheochromocytoma/paraganglioma Diseases of the endocrine glands. Clinical endocrinology RC648-665
Acceso en línea:	https://doaj.org/article/49627f37d5c24641aeaf8cb68a43c342
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spelling	oai:doaj.org-article:49627f37d5c24641aeaf8cb68a43c3422021-11-09T14:28:20ZLocal-Regional Recurrence of Pheochromocytoma/Paraganglioma: Characteristics, Risk Factors and Outcomes1664-239210.3389/fendo.2021.762548https://doaj.org/article/49627f37d5c24641aeaf8cb68a43c3422021-10-01T00:00:00Zhttps://www.frontiersin.org/articles/10.3389/fendo.2021.762548/fullhttps://doaj.org/toc/1664-2392ObjectiveTo study the characteristics, risk factors, and outcomes of local-regional recurrence of pheochromocytoma and paraganglioma (PPGL).MethodsClinical data of 96 PPGL patients with local-regional recurrence and 112 patients without recurrence were retrospectively analyzed.ResultsRecurrent patients exhibited a median recurrence time of 6.0 (4.0, 9.0) years after resection of the primary tumor. SDHB mutation [HR 4.1 (1.7, 9.5), p=0.001), primary tumor size ≥5cm [HR 2.3 (1.1, 4.7), p=0.028], and average Ki-67 count ≥3% in the primary tumor [HR 2.6 (1.4, 4.9), p=0.003] were independent predictors for recurrence of PPGL. Primary tumor sizes ≥5cm [HR 5.1 (1.7, 15.3), p=0.003] and average Ki-67 counts ≥3% of the primary tumor [HR 2.4 (1.1, 5.2), p=0.035] were independent predictors for recurrence of pheochromocytoma, while SDHB mutation [HR 4.6 (1.5, 13.9), p=0.007] was a predictor for paraganglioma recurrence. Among recurrent patients, 47% (45/96) had multiple nodules in recurrent sites, and 58% (56/96) had metastases, with 20% (19/96) being implantation metastases. The risk of metastases (42% vs. 25%, p=0.030) and death (15% vs. 8%, p=0.003) was significantly increased in untreated patients after recurrence compared with treated patients.ConclusionLong-term follow-up is necessary for all PPGL patients. Risk factors for recurrence of pheochromocytoma and paraganglioma differ, with primary tumor size and average Ki-67 count representing independent predictors for pheochromocytoma patients and SDHB mutations predicting paraganglioma recurrence. Although the treatment of recurrence can be difficult, patients should be treated once recurrence is detected as it decreases the risk of metastases and death.Yunying CuiXiaosen MaYinjie GaoXiaoyan ChangShi ChenLin LuAnli TongFrontiers Media S.A.articlerecurrencecharacteristicsrisk factorsoutcomespheochromocytoma/paragangliomaDiseases of the endocrine glands. Clinical endocrinologyRC648-665ENFrontiers in Endocrinology, Vol 12 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	recurrence characteristics risk factors outcomes pheochromocytoma/paraganglioma Diseases of the endocrine glands. Clinical endocrinology RC648-665
spellingShingle	recurrence characteristics risk factors outcomes pheochromocytoma/paraganglioma Diseases of the endocrine glands. Clinical endocrinology RC648-665 Yunying Cui Xiaosen Ma Yinjie Gao Xiaoyan Chang Shi Chen Lin Lu Anli Tong Local-Regional Recurrence of Pheochromocytoma/Paraganglioma: Characteristics, Risk Factors and Outcomes
description	ObjectiveTo study the characteristics, risk factors, and outcomes of local-regional recurrence of pheochromocytoma and paraganglioma (PPGL).MethodsClinical data of 96 PPGL patients with local-regional recurrence and 112 patients without recurrence were retrospectively analyzed.ResultsRecurrent patients exhibited a median recurrence time of 6.0 (4.0, 9.0) years after resection of the primary tumor. SDHB mutation [HR 4.1 (1.7, 9.5), p=0.001), primary tumor size ≥5cm [HR 2.3 (1.1, 4.7), p=0.028], and average Ki-67 count ≥3% in the primary tumor [HR 2.6 (1.4, 4.9), p=0.003] were independent predictors for recurrence of PPGL. Primary tumor sizes ≥5cm [HR 5.1 (1.7, 15.3), p=0.003] and average Ki-67 counts ≥3% of the primary tumor [HR 2.4 (1.1, 5.2), p=0.035] were independent predictors for recurrence of pheochromocytoma, while SDHB mutation [HR 4.6 (1.5, 13.9), p=0.007] was a predictor for paraganglioma recurrence. Among recurrent patients, 47% (45/96) had multiple nodules in recurrent sites, and 58% (56/96) had metastases, with 20% (19/96) being implantation metastases. The risk of metastases (42% vs. 25%, p=0.030) and death (15% vs. 8%, p=0.003) was significantly increased in untreated patients after recurrence compared with treated patients.ConclusionLong-term follow-up is necessary for all PPGL patients. Risk factors for recurrence of pheochromocytoma and paraganglioma differ, with primary tumor size and average Ki-67 count representing independent predictors for pheochromocytoma patients and SDHB mutations predicting paraganglioma recurrence. Although the treatment of recurrence can be difficult, patients should be treated once recurrence is detected as it decreases the risk of metastases and death.
format	article
author	Yunying Cui Xiaosen Ma Yinjie Gao Xiaoyan Chang Shi Chen Lin Lu Anli Tong
author_facet	Yunying Cui Xiaosen Ma Yinjie Gao Xiaoyan Chang Shi Chen Lin Lu Anli Tong
author_sort	Yunying Cui
title	Local-Regional Recurrence of Pheochromocytoma/Paraganglioma: Characteristics, Risk Factors and Outcomes
title_short	Local-Regional Recurrence of Pheochromocytoma/Paraganglioma: Characteristics, Risk Factors and Outcomes
title_full	Local-Regional Recurrence of Pheochromocytoma/Paraganglioma: Characteristics, Risk Factors and Outcomes
title_fullStr	Local-Regional Recurrence of Pheochromocytoma/Paraganglioma: Characteristics, Risk Factors and Outcomes
title_full_unstemmed	Local-Regional Recurrence of Pheochromocytoma/Paraganglioma: Characteristics, Risk Factors and Outcomes
title_sort	local-regional recurrence of pheochromocytoma/paraganglioma: characteristics, risk factors and outcomes
publisher	Frontiers Media S.A.
publishDate	2021
url	https://doaj.org/article/49627f37d5c24641aeaf8cb68a43c342
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Local-Regional Recurrence of Pheochromocytoma/Paraganglioma: Characteristics, Risk Factors and Outcomes

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