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Normal life expectancy for paraganglioma patients Vrije Universiteit Brussel

Jeanette de Flines, Jeroen J Jansen, Reinier Elders, Maaike Siemers, Annette H.J.T. Vriends, Frederik Jan Hes, Jean-Pierre Bayley, Andel Gl van der Mey, Eleonora Pm Corssmit

The objective of this study was to assess the long-term survival of patients with a paraganglioma of the head and neck compared with the survival of the general Dutch population. This historic cohort study was conducted using nationwide historical data of paraganglioma patients. We retrieved a cohort of 86 patients diagnosed with a paraganglioma of the head and neck between 1945 and 1960 in the Netherlands. Dates of death were retrieved from ...

Parent-of-origin tumourigenesis is mediated by an essential imprinted modifier in SDHD-linked paragangliomas Vrije Universiteit Brussel

Attje S Hoekstra, Ruben D Addie, Cor Ras, Reza M Seifar, Claudia A Ruivenkamp, Inge H Briaire-de Bruijn, Frederik Jan Hes, Jeroen C Jansen, Eleonora P M Corssmit, Willem E Corver, et al.

Mutations in SDHD and SDHAF2 (both located on chromosome 11) give rise to hereditary paraganglioma almost exclusively after paternal transmission of the mutation, and tumours often show loss of the entire maternal copy of chromosome 11. The 'Hensen' model postulates that a tumour modifier gene located on chromosome 11p15, a region known to harbour a cluster of imprinted genes, is essential to tumour formation. We observed decreased protein ...

Modern IMRT for head and neck cancer: a paradoxical paradigm of simultaneous dose-escalation and de-escalation Universiteit Gent

Fréderic Duprez
Advanced dose distributions and high dose gradients defined the superiority of intensity-modulated radiotherapy (IMRT) in organ-function sparing over conventional and 3-dimentional (3D)-conformal radiotherapy and encouraged treatment intensification for head and neck cancer. Various combinations of higher radiation doses, altered fractionation and chemotherapy have been associated with an increase in frequency, severity and duration of ...

Investigation of the role of GARP by using cell specific knockout mice KU Leuven

Elien Vermeersch
Glycoprotein A repetitions predominant protein (GARP) is a transmembrane protein that captures latent transforming growth factor beta 1 (TGF-β1) on the surface of amongst others regulatory T cells (Treg), platelets and endothelial cells. Latent TGF-b consists of the mature TGF-b and the latency associated peptide (LAP), which prevents mature TGF-b from binding to the TGF-b receptor. In order to be able to exert its function, latent TGF-b thus ...

High prevalence of occult paragangliomas in asymptomatic carriers of SDHD and SDHB gene mutations Vrije Universiteit Brussel

Berdine L Heesterman, Jean-Pierre Bayley, Carli Mj Tops, Frederik Jan Hes, Bernadette T.J. Van Brussel, Eleonora P M Corssmit, Jaap F. Hamming, Andel G L van der Mey, Jeroen C Jansen

Hereditary paraganglioma is a benign tumor syndrome with an age-dependent penetrance. Carriers of germline mutations in the SDHB or SDHD genes may develop parasympathetic paragangliomas in the head and neck region or sympathetic catecholamine-secreting abdominal and thoracic paragangliomas (pheochromocytomas). In this study, we aimed to establish paraganglioma risk in 101 asymptomatic germline mutation carriers and evaluate the results of our ...

Mathematical Models for Tumor Growth and the Reduction of Overtreatment Vrije Universiteit Brussel

Berdine L Heesterman, John-Melle Bokhorst, Lisa M H de Pont, Berit M Verbist, Jean-Pierre Bayley, Andel G L van der Mey, Eleonora P M Corssmit, Frederik Jan Hes, Peter Paul G van Benthem, Jeroen C Jansen, et al.

Background To improve our understanding of the natural course of head and neck paragangliomas (HNPGL) and ultimately differentiate between cases that benefit from early treatment and those that are best left untreated, we studied the growth dynamics of 77 HNPGL managed with primary observation. Methods Using digitally available magnetic resonance images, tumor volume was estimated at three time points. Subsequently, nonlinear least squares ...

Head and neck paragangliomas Vrije Universiteit Brussel

Christian Offergeld, Christoph Brase, Svetlana Yaremchuk, Irina Mader, Hans Christian Rischke, Sven Gläsker, Kurt W Schmid, Thorsten Wiech, Simon F Preuss, Carlos Suárez, et al.

Head and neck paragangliomas are tumors arising from specialized neural crest cells. Prominent locations are the carotid body along with the vagal, jugular, and tympanic glomus. Head and neck paragangliomas are slowly growing tumors, with some carotid body tumors being reported to exist for many years as a painless lateral mass on the neck. Symptoms depend on the specific locations. In contrast to paraganglial tumors of the adrenals, abdomen ...

Quality of life is decreased in patients with paragangliomas Vrije Universiteit Brussel

Leonie T van Hulsteijn, A Louisse, B Havekes, Ad A Kaptein, Julie Jansen, Frederik Jan Hes, Johanna Smit, Eleonora P M Corssmit

CONTEXT: Germline mutations in succinate dehydrogenase (SDH) genes predispose carriers for developing paragangliomas, and studies on their quality of life (QoL) are scarce.

OBJECTIVES: The objectives of this study were to assess QoL in patients with paragangliomas (PGL), to evaluate long-term QoL, and to explore potential differences in QoL between SDH mutation carriers and paraganglioma patients without an SDH mutation.

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Age and Tumor Volume Predict Growth of Carotid and Vagal Body Paragangliomas Vrije Universiteit Brussel

Berdine L Heesterman, Lisa M H de Pont, Berit M Verbist, Andel G L van der Mey, Eleonora P M Corssmit, Frederik Jan Hes, Peter Paul G van Benthem, Jeroen C Jansen

Objective  Treatment for head and neck paragangliomas (HNGPL) can be more harmful than the disease. After diagnosis, an initial period of surveillance is often indicated, and surgery or radiotherapy is reserved for progressive disease. With the aim to optimize this "wait and scan" strategy, we studied growth and possible predictors. Design  A retrospective cohort study was conducted. Setting  This study was conducted at a tertiary referral ...