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Characteristics of Pediatric vs Adult Pheochromocytomas and Paragangliomas

Context: Pheochromocytomas and paragangliomas (PPGLs) in children are often hereditary and may present with different characteristics compared with adults. Hereditary PPGLs can be separated into cluster 1 and cluster 2 tumors due to mutations impacting hypoxia and kinase receptor signaling pathways,... Full description

Journal Title: The Journal of Clinical Endocrinology & Metabolism 2017, Vol.102 (4), p.1122-1132
Main Author: Pamporaki, C
Other Authors: Hamplova, B , Peitzsch, M , Prejbisz, A , Beuschlein, F , Timmers, H.J.L.M , Fassnacht, M , Klink, B , Lodish, M , Stratakis, C.A , Huebner, A , Fliedner, S , Robledo, M , Sinnott, R.O , Januszewicz, A , Pacak, K , Eisenhofer, G
Format: Electronic Article Electronic Article
Language: English
Subjects:
Publisher: Washington, DC: Endocrine Society
ID: ISSN: 0021-972X
Link: https://www.ncbi.nlm.nih.gov/pubmed/28324046
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recordid: cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_5460722
title: Characteristics of Pediatric vs Adult Pheochromocytomas and Paragangliomas
format: Article
creator:
  • Pamporaki, C
  • Hamplova, B
  • Peitzsch, M
  • Prejbisz, A
  • Beuschlein, F
  • Timmers, H.J.L.M
  • Fassnacht, M
  • Klink, B
  • Lodish, M
  • Stratakis, C.A
  • Huebner, A
  • Fliedner, S
  • Robledo, M
  • Sinnott, R.O
  • Januszewicz, A
  • Pacak, K
  • Eisenhofer, G
subjects:
  • 610 Medicine & health
  • Adolescent
  • Adrenal Gland Neoplasms - epidemiology
  • Adrenal Gland Neoplasms - genetics
  • Adrenal Gland Neoplasms - pathology
  • Adult
  • Adults
  • Age of Onset
  • Child
  • Children
  • Clinic for Endocrinology
  • Clinical Research Articles
  • Clinical s
  • Cross-Sectional Studies
  • Diabetology
  • Female
  • Genetic screening
  • Genetic Testing
  • Health care facilities
  • Humans
  • Hypoxia
  • Male
  • Metastases
  • Metastasis
  • Middle Aged
  • Mutation
  • Neoplasm Metastasis
  • Neoplasm Recurrence, Local - epidemiology
  • Neoplasm Recurrence, Local - genetics
  • Norepinephrine
  • Paraganglioma
  • Paraganglioma - epidemiology
  • Paraganglioma - genetics
  • Paraganglioma - pathology
  • Pediatrics
  • Pheochromocytoma - epidemiology
  • Pheochromocytoma - genetics
  • Pheochromocytoma - pathology
  • Prevalence
  • Retrospective Studies
  • Tumors
ispartof: The Journal of Clinical Endocrinology & Metabolism, 2017, Vol.102 (4), p.1122-1132
description: Context: Pheochromocytomas and paragangliomas (PPGLs) in children are often hereditary and may present with different characteristics compared with adults. Hereditary PPGLs can be separated into cluster 1 and cluster 2 tumors due to mutations impacting hypoxia and kinase receptor signaling pathways, respectively. Objective: To identify differences in presentation of PPGLs between children and adults. Design: A retrospective cross-sectional clinical study. Setting: Seven tertiary medical centers. Patients: The study included 748 patients with PPGLs, including 95 with a first presentation during childhood. Genetic testing was available in 611 patients. Other data included locations of primary tumors, presence of recurrent or metastatic disease, and plasma concentrations of metanephrines and 3-methoxytyramine. Results: Children showed higher (P < 0.0001) prevalence than adults of hereditary (80.4% vs 52.6%), extra-adrenal (66.3% vs 35.1%), multifocal (32.6% vs 13.5%), metastatic (49.5% vs 29.1%), and recurrent (29.5% vs 14.2%) PPGLs. Tumors due to cluster 1 mutations were more prevalent among children than adults (76.1% vs 39.3%; P < 0.0001), and this paralleled a higher prevalence of noradrenergic tumors, characterized by relative lack of increased plasma metanephrine, in children than in adults (93.2% vs 57.3%; P < 0.0001). Conclusions: The higher prevalence of hereditary, extra-adrenal, multifocal, and metastatic PPGLs in children than adults represents interrelated features that, in part, reflect the lower age of disease presentation of noradrenergic cluster 1 than adrenergic cluster 2 tumors. The differences in disease presentation are important to consider in children at risk for PPGLs due to a known mutation or previous history of tumor.
language: eng
source:
identifier: ISSN: 0021-972X
fulltext: no_fulltext
issn:
  • 0021-972X
  • 1945-7197
url: Link


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titleCharacteristics of Pediatric vs Adult Pheochromocytomas and Paragangliomas
creatorPamporaki, C ; Hamplova, B ; Peitzsch, M ; Prejbisz, A ; Beuschlein, F ; Timmers, H.J.L.M ; Fassnacht, M ; Klink, B ; Lodish, M ; Stratakis, C.A ; Huebner, A ; Fliedner, S ; Robledo, M ; Sinnott, R.O ; Januszewicz, A ; Pacak, K ; Eisenhofer, G
creatorcontribPamporaki, C ; Hamplova, B ; Peitzsch, M ; Prejbisz, A ; Beuschlein, F ; Timmers, H.J.L.M ; Fassnacht, M ; Klink, B ; Lodish, M ; Stratakis, C.A ; Huebner, A ; Fliedner, S ; Robledo, M ; Sinnott, R.O ; Januszewicz, A ; Pacak, K ; Eisenhofer, G
descriptionContext: Pheochromocytomas and paragangliomas (PPGLs) in children are often hereditary and may present with different characteristics compared with adults. Hereditary PPGLs can be separated into cluster 1 and cluster 2 tumors due to mutations impacting hypoxia and kinase receptor signaling pathways, respectively. Objective: To identify differences in presentation of PPGLs between children and adults. Design: A retrospective cross-sectional clinical study. Setting: Seven tertiary medical centers. Patients: The study included 748 patients with PPGLs, including 95 with a first presentation during childhood. Genetic testing was available in 611 patients. Other data included locations of primary tumors, presence of recurrent or metastatic disease, and plasma concentrations of metanephrines and 3-methoxytyramine. Results: Children showed higher (P < 0.0001) prevalence than adults of hereditary (80.4% vs 52.6%), extra-adrenal (66.3% vs 35.1%), multifocal (32.6% vs 13.5%), metastatic (49.5% vs 29.1%), and recurrent (29.5% vs 14.2%) PPGLs. Tumors due to cluster 1 mutations were more prevalent among children than adults (76.1% vs 39.3%; P < 0.0001), and this paralleled a higher prevalence of noradrenergic tumors, characterized by relative lack of increased plasma metanephrine, in children than in adults (93.2% vs 57.3%; P < 0.0001). Conclusions: The higher prevalence of hereditary, extra-adrenal, multifocal, and metastatic PPGLs in children than adults represents interrelated features that, in part, reflect the lower age of disease presentation of noradrenergic cluster 1 than adrenergic cluster 2 tumors. The differences in disease presentation are important to consider in children at risk for PPGLs due to a known mutation or previous history of tumor.
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subject610 Medicine & health ; Adolescent ; Adrenal Gland Neoplasms - epidemiology ; Adrenal Gland Neoplasms - genetics ; Adrenal Gland Neoplasms - pathology ; Adult ; Adults ; Age of Onset ; Child ; Children ; Clinic for Endocrinology ; Clinical Research Articles ; Clinical s ; Cross-Sectional Studies ; Diabetology ; Female ; Genetic screening ; Genetic Testing ; Health care facilities ; Humans ; Hypoxia ; Male ; Metastases ; Metastasis ; Middle Aged ; Mutation ; Neoplasm Metastasis ; Neoplasm Recurrence, Local - epidemiology ; Neoplasm Recurrence, Local - genetics ; Norepinephrine ; Paraganglioma ; Paraganglioma - epidemiology ; Paraganglioma - genetics ; Paraganglioma - pathology ; Pediatrics ; Pheochromocytoma - epidemiology ; Pheochromocytoma - genetics ; Pheochromocytoma - pathology ; Prevalence ; Retrospective Studies ; Tumors
ispartofThe Journal of Clinical Endocrinology & Metabolism, 2017, Vol.102 (4), p.1122-1132
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7Klink, B
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9Stratakis, C.A
10Huebner, A
11Fliedner, S
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13Sinnott, R.O
14Januszewicz, A
15Pacak, K
16Eisenhofer, G
title
0Characteristics of Pediatric vs Adult Pheochromocytomas and Paragangliomas
1The Journal of Clinical Endocrinology & Metabolism
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descriptionContext: Pheochromocytomas and paragangliomas (PPGLs) in children are often hereditary and may present with different characteristics compared with adults. Hereditary PPGLs can be separated into cluster 1 and cluster 2 tumors due to mutations impacting hypoxia and kinase receptor signaling pathways, respectively. Objective: To identify differences in presentation of PPGLs between children and adults. Design: A retrospective cross-sectional clinical study. Setting: Seven tertiary medical centers. Patients: The study included 748 patients with PPGLs, including 95 with a first presentation during childhood. Genetic testing was available in 611 patients. Other data included locations of primary tumors, presence of recurrent or metastatic disease, and plasma concentrations of metanephrines and 3-methoxytyramine. Results: Children showed higher (P < 0.0001) prevalence than adults of hereditary (80.4% vs 52.6%), extra-adrenal (66.3% vs 35.1%), multifocal (32.6% vs 13.5%), metastatic (49.5% vs 29.1%), and recurrent (29.5% vs 14.2%) PPGLs. Tumors due to cluster 1 mutations were more prevalent among children than adults (76.1% vs 39.3%; P < 0.0001), and this paralleled a higher prevalence of noradrenergic tumors, characterized by relative lack of increased plasma metanephrine, in children than in adults (93.2% vs 57.3%; P < 0.0001). Conclusions: The higher prevalence of hereditary, extra-adrenal, multifocal, and metastatic PPGLs in children than adults represents interrelated features that, in part, reflect the lower age of disease presentation of noradrenergic cluster 1 than adrenergic cluster 2 tumors. The differences in disease presentation are important to consider in children at risk for PPGLs due to a known mutation or previous history of tumor.
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0610 Medicine & health
1Adolescent
2Adrenal Gland Neoplasms - epidemiology
3Adrenal Gland Neoplasms - genetics
4Adrenal Gland Neoplasms - pathology
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7Age of Onset
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10Clinic for Endocrinology
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14Diabetology
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16Genetic screening
17Genetic Testing
18Health care facilities
19Humans
20Hypoxia
21Male
22Metastases
23Metastasis
24Middle Aged
25Mutation
26Neoplasm Metastasis
27Neoplasm Recurrence, Local - epidemiology
28Neoplasm Recurrence, Local - genetics
29Norepinephrine
30Paraganglioma
31Paraganglioma - epidemiology
32Paraganglioma - genetics
33Paraganglioma - pathology
34Pediatrics
35Pheochromocytoma - epidemiology
36Pheochromocytoma - genetics
37Pheochromocytoma - pathology
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39Retrospective Studies
40Tumors
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titleCharacteristics of Pediatric vs Adult Pheochromocytomas and Paragangliomas
authorPamporaki, C ; Hamplova, B ; Peitzsch, M ; Prejbisz, A ; Beuschlein, F ; Timmers, H.J.L.M ; Fassnacht, M ; Klink, B ; Lodish, M ; Stratakis, C.A ; Huebner, A ; Fliedner, S ; Robledo, M ; Sinnott, R.O ; Januszewicz, A ; Pacak, K ; Eisenhofer, G
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1Adolescent
2Adrenal Gland Neoplasms - epidemiology
3Adrenal Gland Neoplasms - genetics
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7Age of Onset
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29Norepinephrine
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37Pheochromocytoma - pathology
38Prevalence
39Retrospective Studies
40Tumors
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abstractContext: Pheochromocytomas and paragangliomas (PPGLs) in children are often hereditary and may present with different characteristics compared with adults. Hereditary PPGLs can be separated into cluster 1 and cluster 2 tumors due to mutations impacting hypoxia and kinase receptor signaling pathways, respectively. Objective: To identify differences in presentation of PPGLs between children and adults. Design: A retrospective cross-sectional clinical study. Setting: Seven tertiary medical centers. Patients: The study included 748 patients with PPGLs, including 95 with a first presentation during childhood. Genetic testing was available in 611 patients. Other data included locations of primary tumors, presence of recurrent or metastatic disease, and plasma concentrations of metanephrines and 3-methoxytyramine. Results: Children showed higher (P < 0.0001) prevalence than adults of hereditary (80.4% vs 52.6%), extra-adrenal (66.3% vs 35.1%), multifocal (32.6% vs 13.5%), metastatic (49.5% vs 29.1%), and recurrent (29.5% vs 14.2%) PPGLs. Tumors due to cluster 1 mutations were more prevalent among children than adults (76.1% vs 39.3%; P < 0.0001), and this paralleled a higher prevalence of noradrenergic tumors, characterized by relative lack of increased plasma metanephrine, in children than in adults (93.2% vs 57.3%; P < 0.0001). Conclusions: The higher prevalence of hereditary, extra-adrenal, multifocal, and metastatic PPGLs in children than adults represents interrelated features that, in part, reflect the lower age of disease presentation of noradrenergic cluster 1 than adrenergic cluster 2 tumors. The differences in disease presentation are important to consider in children at risk for PPGLs due to a known mutation or previous history of tumor.
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