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Metastatic pheochromocytoma: Does the size and age matter?

Eur J Clin Invest 2011; 41 (10): 1121–1128 Background  Pheochromocytomas are tumours arising from chromaffin tissue located in the adrenal medulla associated with typical symptoms and signs which may occasionally develop metastases, which are defined as the presence of tumour cells at sites where th...

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Published in:European journal of clinical investigation 2011-10, Vol.41 (10), p.1121-1128
Main Authors: Zelinka, Tomáš, Musil, Zdeněk, Dušková, Jaroslava, Burton, Deborah, Merino, Maria J., Milosevic, Dragana, Widimský Jr, Jiří, Pacak, Karel
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container_issue 10
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container_title European journal of clinical investigation
container_volume 41
creator Zelinka, Tomáš
Musil, Zdeněk
Dušková, Jaroslava
Burton, Deborah
Merino, Maria J.
Milosevic, Dragana
Widimský Jr, Jiří
Pacak, Karel
description Eur J Clin Invest 2011; 41 (10): 1121–1128 Background  Pheochromocytomas are tumours arising from chromaffin tissue located in the adrenal medulla associated with typical symptoms and signs which may occasionally develop metastases, which are defined as the presence of tumour cells at sites where these cells are not found. This retrospective analysis was focused on clinical, genetic and histopathologic characteristics of primary metastatic versus primary benign pheochromocytomas. Materials and methods  We identified 41 subjects with metastatic pheochromocytoma and 108 subjects with apparently benign pheochromocytoma. We assessed dimension and biochemical profile of the primary tumour, age at presentation and time to develop metastases. Results  Subjects with metastatic pheochromocytoma presented at a significantly younger age (41·4 ± 14·7 vs. 50·2 ± 13·7 years; P 
doi_str_mv 10.1111/j.1365-2362.2011.02518.x
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This retrospective analysis was focused on clinical, genetic and histopathologic characteristics of primary metastatic versus primary benign pheochromocytomas. Materials and methods  We identified 41 subjects with metastatic pheochromocytoma and 108 subjects with apparently benign pheochromocytoma. We assessed dimension and biochemical profile of the primary tumour, age at presentation and time to develop metastases. Results  Subjects with metastatic pheochromocytoma presented at a significantly younger age (41·4 ± 14·7 vs. 50·2 ± 13·7 years; P &lt; 0·001) with larger primary tumours (8·38 ± 3·27 vs. 6·18 ± 2·75 cm; P &lt; 0·001) and secreted more frequently norepinephrine (95·1% vs. 83·3%; P = 0·046) compared to subjects with apparently benign pheochromocytomas. No significant differences were found in the incidence of genetic mutations in both groups of subjects (25·7% in the metastatic group and 14·7% in the benign group; P = 0·13). From available histopathologic markers of potential malignancy, only necrosis occurred more frequently in subjects with metastatic pheochromocytoma (27·6% vs. 0%; P &lt; 0·001). The median time to develop metastases was 3·6 years with the longest interval 24 years. Conclusions  In conclusion, regardless of a genetic background, the size of a primary pheochromocytoma and age of its first presentation are two independent risk factors associated with the development of metastatic disease.</description><identifier>ISSN: 0014-2972</identifier><identifier>EISSN: 1365-2362</identifier><identifier>DOI: 10.1111/j.1365-2362.2011.02518.x</identifier><identifier>PMID: 21692797</identifier><language>eng</language><publisher>Oxford, UK: Blackwell Publishing Ltd</publisher><subject>Adrenal Gland Neoplasms - genetics ; Adrenal Gland Neoplasms - metabolism ; Adrenal Gland Neoplasms - pathology ; Adrenals. Adrenal axis. Renin-angiotensin system (diseases) ; Adult ; Age Factors ; Biological and medical sciences ; Biomarkers, Tumor ; Case-Control Studies ; Endocrinopathies ; Epinephrine ; Epinephrine - secretion ; Female ; General aspects ; Humans ; Male ; malignant pheochromocytoma ; Medical sciences ; Middle Aged ; Non tumoral diseases. Target tissue resistance. Benign neoplasms ; norepinephrine ; Norepinephrine - secretion ; Pheochromocytoma - genetics ; Pheochromocytoma - metabolism ; Pheochromocytoma - pathology ; Retrospective Studies ; Risk Assessment ; Risk Factors ; Time Factors ; Tumor Burden</subject><ispartof>European journal of clinical investigation, 2011-10, Vol.41 (10), p.1121-1128</ispartof><rights>European Journal of Clinical Investigation © 2011 Stichting European Society for Clinical Investigation Journal Foundation. 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This retrospective analysis was focused on clinical, genetic and histopathologic characteristics of primary metastatic versus primary benign pheochromocytomas. Materials and methods  We identified 41 subjects with metastatic pheochromocytoma and 108 subjects with apparently benign pheochromocytoma. We assessed dimension and biochemical profile of the primary tumour, age at presentation and time to develop metastases. Results  Subjects with metastatic pheochromocytoma presented at a significantly younger age (41·4 ± 14·7 vs. 50·2 ± 13·7 years; P &lt; 0·001) with larger primary tumours (8·38 ± 3·27 vs. 6·18 ± 2·75 cm; P &lt; 0·001) and secreted more frequently norepinephrine (95·1% vs. 83·3%; P = 0·046) compared to subjects with apparently benign pheochromocytomas. No significant differences were found in the incidence of genetic mutations in both groups of subjects (25·7% in the metastatic group and 14·7% in the benign group; P = 0·13). From available histopathologic markers of potential malignancy, only necrosis occurred more frequently in subjects with metastatic pheochromocytoma (27·6% vs. 0%; P &lt; 0·001). The median time to develop metastases was 3·6 years with the longest interval 24 years. Conclusions  In conclusion, regardless of a genetic background, the size of a primary pheochromocytoma and age of its first presentation are two independent risk factors associated with the development of metastatic disease.</description><subject>Adrenal Gland Neoplasms - genetics</subject><subject>Adrenal Gland Neoplasms - metabolism</subject><subject>Adrenal Gland Neoplasms - pathology</subject><subject>Adrenals. Adrenal axis. Renin-angiotensin system (diseases)</subject><subject>Adult</subject><subject>Age Factors</subject><subject>Biological and medical sciences</subject><subject>Biomarkers, Tumor</subject><subject>Case-Control Studies</subject><subject>Endocrinopathies</subject><subject>Epinephrine</subject><subject>Epinephrine - secretion</subject><subject>Female</subject><subject>General aspects</subject><subject>Humans</subject><subject>Male</subject><subject>malignant pheochromocytoma</subject><subject>Medical sciences</subject><subject>Middle Aged</subject><subject>Non tumoral diseases. Target tissue resistance. 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Adrenal axis. Renin-angiotensin system (diseases)</topic><topic>Adult</topic><topic>Age Factors</topic><topic>Biological and medical sciences</topic><topic>Biomarkers, Tumor</topic><topic>Case-Control Studies</topic><topic>Endocrinopathies</topic><topic>Epinephrine</topic><topic>Epinephrine - secretion</topic><topic>Female</topic><topic>General aspects</topic><topic>Humans</topic><topic>Male</topic><topic>malignant pheochromocytoma</topic><topic>Medical sciences</topic><topic>Middle Aged</topic><topic>Non tumoral diseases. Target tissue resistance. 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This retrospective analysis was focused on clinical, genetic and histopathologic characteristics of primary metastatic versus primary benign pheochromocytomas. Materials and methods  We identified 41 subjects with metastatic pheochromocytoma and 108 subjects with apparently benign pheochromocytoma. We assessed dimension and biochemical profile of the primary tumour, age at presentation and time to develop metastases. Results  Subjects with metastatic pheochromocytoma presented at a significantly younger age (41·4 ± 14·7 vs. 50·2 ± 13·7 years; P &lt; 0·001) with larger primary tumours (8·38 ± 3·27 vs. 6·18 ± 2·75 cm; P &lt; 0·001) and secreted more frequently norepinephrine (95·1% vs. 83·3%; P = 0·046) compared to subjects with apparently benign pheochromocytomas. No significant differences were found in the incidence of genetic mutations in both groups of subjects (25·7% in the metastatic group and 14·7% in the benign group; P = 0·13). From available histopathologic markers of potential malignancy, only necrosis occurred more frequently in subjects with metastatic pheochromocytoma (27·6% vs. 0%; P &lt; 0·001). The median time to develop metastases was 3·6 years with the longest interval 24 years. Conclusions  In conclusion, regardless of a genetic background, the size of a primary pheochromocytoma and age of its first presentation are two independent risk factors associated with the development of metastatic disease.</abstract><cop>Oxford, UK</cop><pub>Blackwell Publishing Ltd</pub><pmid>21692797</pmid><doi>10.1111/j.1365-2362.2011.02518.x</doi><tpages>8</tpages><oa>free_for_read</oa></addata></record>
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source Wiley:Jisc Collections:Wiley Read and Publish Open Access 2024-2025 (reading list)
subjects Adrenal Gland Neoplasms - genetics
Adrenal Gland Neoplasms - metabolism
Adrenal Gland Neoplasms - pathology
Adrenals. Adrenal axis. Renin-angiotensin system (diseases)
Adult
Age Factors
Biological and medical sciences
Biomarkers, Tumor
Case-Control Studies
Endocrinopathies
Epinephrine
Epinephrine - secretion
Female
General aspects
Humans
Male
malignant pheochromocytoma
Medical sciences
Middle Aged
Non tumoral diseases. Target tissue resistance. Benign neoplasms
norepinephrine
Norepinephrine - secretion
Pheochromocytoma - genetics
Pheochromocytoma - metabolism
Pheochromocytoma - pathology
Retrospective Studies
Risk Assessment
Risk Factors
Time Factors
Tumor Burden
title Metastatic pheochromocytoma: Does the size and age matter?
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