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Missed clinical clues in patients with pheochromocytoma/paraganglioma discovered by imaging

Please always quote using this URN: urn:nbn:de:bvb:20-opus-226481
  • Background: Pheochromocytomas and paragangliomas (PPGLs) are rare but potentially harmful tumors that can vary in their clinical presentation. Tumors may be found due to signs and symptoms, as part of a hereditary syndrome or following an imaging procedure. Objective: To investigate potential differences in clinical presentation between PPGLs discovered by imaging (iPPGLs), symptomatic cases (sPPGLs) and those diagnosed during follow-up because of earlier disease/known hereditary mutations (fPPGL). Design: Prospective study protocol, which hasBackground: Pheochromocytomas and paragangliomas (PPGLs) are rare but potentially harmful tumors that can vary in their clinical presentation. Tumors may be found due to signs and symptoms, as part of a hereditary syndrome or following an imaging procedure. Objective: To investigate potential differences in clinical presentation between PPGLs discovered by imaging (iPPGLs), symptomatic cases (sPPGLs) and those diagnosed during follow-up because of earlier disease/known hereditary mutations (fPPGL). Design: Prospective study protocol, which has enrolled patients from six European centers with confirmed PPGLs. Data were analyzed from 235 patients (37 iPPGLs, 36 sPPGLs, 27% fPPGLs) and compared for tumor volume, biochemical profile, mutation status, presence of metastases and self-reported symptoms. iPPGL patients were diagnosed at a significantly higher age than fPPGLs (P<0.001), found to have larger tumors (P=0.003) and higher metanephrine and normetanephrine levels at diagnosis (P=0.021). Significantly lower than in sPPGL, there was a relevant number of self-reported symptoms in iPPGL (2.9 vs 4.3 symptoms, P< 0.001). In 16.2% of iPPGL, mutations in susceptibility genes were detected, although this proportion was lower than that in fPPGL (60.9%) and sPPGL (21.5%). Patients with PPGLs detected by imaging were older, have higher tumor volume and more excessive hormonal secretion in comparison to those found as part of a surveillance program. Presence of typical symptoms indicates that in a relevant proportion of those patients, the PPGL diagnosis had been delayed. Precis: Pheochromocytoma/paraganglioma discovered by imaging are often symptomatic and carry a significant proportion of germline mutations in susceptibility genes.show moreshow less

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Metadaten
Author: Natalie Rogowski-Lehmann, Aikaterini Geroula, Aleksander Prejbisz, Henri J. L. M. Timmers, Felix Megerle, Mercedes Robledo, Martin Fassnacht, Stephanie M. J. Fliedner, Martin Reincke, Anthony Stell, Andrzej Januszewicz, Jacques W. M. Lenders, Graeme Eisenhofer, Felix Beuschlein
URN:urn:nbn:de:bvb:20-opus-226481
Document Type:Journal article
Faculties:Medizinische Fakultät / Medizinische Klinik und Poliklinik I
Language:English
Parent Title (English):Endocrine Connections
Year of Completion:2018
Volume:7
Issue:11
Pagenumber:1168-1177
Source:Endocrine Connections (2018) 7, 1168–1177
DOI:https://doi.org/10.1530/EC-18-0318
Dewey Decimal Classification:6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit
GND Keyword:Biochemical-Diagnosis; Plasma; MASS; Normetanephrine; Metanephrine; Paraganglioma; Society; Utility
Tag:imaging; paraganglioma; pheochromocytoma; prospective; signs and symptoms
Release Date:2021/07/13
Licence (German):License LogoCC BY-NC: Creative-Commons-Lizenz: Namensnennung, Nicht kommerziell 4.0 International