Determinants of disease-specific survival in patients with and without metastatic pheochromocytoma and paraganglioma

Pamporaki, Christina; Prodanov, Tamara; Meuter, Leah; Berends, Annika M A; Bechmann, Nicole; Constantinescu, Georgiana; Beuschlein, Felix; Remde, Hanna; Januszewicz, Andrzej; Kerstens, Michiel N.; Timmers, Henri J L M; Taïeb, David; Robledo, Mercedes; Lenders, Jacques W.M.; Pacák, Karel; Eisenhofer, Graeme

doi:10.1016/j.ejca.2022.03.032

Cited by 33 publications

(25 citation statements)

References 39 publications

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“…It has recently been further demonstrated that metastatic HN-PGLs have a longer diseasespecific survival than metastatic PPGLs, and that extra-adrenal PPGLs (i.e., PGL as compared to PCC) have worse prognosis 36 , in line with our data.…”

Section: Discussionsupporting

confidence: 92%

“…This strengthens the benefit of the WHO classification of these three subgroups for diagnostics, when assessing treatment regimens and patient follow-up schemes. It has recently been further demonstrated that metastatic HN-PGLs have a longer disease-specific survival than metastatic PPGLs, and that extra-adrenal PPGLs (i.e., PGL as compared to PCC) have worse prognosis 36 , in line with our data.…”

Section: Discussionsupporting

confidence: 92%

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Cytoplasmic HIF-2α correlates to proliferation and predicts worse outcome in sympathetic paraganglioma

Karakaya

Gunnesson

Elias

et al. 2022

Preprint

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Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare neuroendocrine tumors. PGLs can further be divided into sympathetic (sPGLs) and head-and-neck (HN-PGLs). There are virtually no treatment options, and no cure, for metastatic PCCs and PGLs (PPGLs). Here, we composed a tissue microarray (TMA) consisting of 149 PPGLs, reflecting clinical features and presenting as a useful resource. Mutations in the pseudohypoxic marker EPAS1/HIF-2a correlates to an aggressive tumor phenotype. We show that HIF-2a unexpectedly localized to the cytoplasm in PPGLs. This subcompartmentalized protein expression differed between tumor subtypes, and strongly correlated to proliferation. Half of all sPGLs were metastatic at time of diagnosis. Cytoplasmic HIF-2a was strongly expressed in metastatic sPGLs and predicted poor outcome in this subgroup. We propose that cytoplasmic HIF-2a expression serves as a useful clinical marker to differentiate subtypes and predicting outcome, and hence can be used for improved targeted treatment in PPGLs.

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Section: Discussionsupporting

confidence: 92%

Section: Discussionsupporting

confidence: 92%

Cytoplasmic HIF-2α correlates to proliferation and predicts worse outcome in sympathetic paraganglioma

Karakaya

Gunnesson

Elias

et al. 2022

Preprint

Self Cite

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show abstract

“…Additionally, though a study on a large group of more than 600 patients with PPGL allowed the identification of factors favouring the development of metastases, such as the diagnosis of a tumour in older age, in terms of individual patients it is impossible to predict the development of metastases at the time of PPGL diagnosis 49 .…”

Section: Metabolomic Profile Of Pheochromocytomas-paragangliomas As A...mentioning

confidence: 99%

Updates in neuroendocrine neoplasms: From mechanisms to the clinic

Fodil-Cherif

Desailloud

Brue

2023

Annales d'Endocrinologie

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“…Such tumors, similar to those that produce predominantly dopamine, tend to reach a large size before diagnosis, which is usually due to local mass effects and incidental discovery on imaging. Biochemically negative PPGL that are characterized by an immature biochemical phenotype and low tissue stores of catecholamines, only some of which may be truly “non-functional” ( 58 ), are associated with an aggressive phenotype in terms of higher rates of malignancy ( 140 – 144 ).…”

Section: Proposed Definitions For a Standardized Approachmentioning

confidence: 99%

Silent pheochromocytoma and paraganglioma: Systematic review and proposed definitions for standardized terminology

et al. 2022

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Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors with heterogeneous clinical presentations and potential lethal outcomes. The diagnosis is based on clinical suspicion, biochemical testing, imaging and histopathological confirmation. Increasingly widespread use of imaging studies and surveillance of patients at risk of PPGL due to a hereditary background or a previous tumor is leading to the diagnosis of these tumors at an early stage. This has resulted in an increasing use of the term “silent” PPGL. This term and other variants are now commonly found in the literature without any clear or unified definition. Among the various terms, “clinically silent” is often used to describe the lack of signs and symptoms associated with catecholamine excess. Confusion arises when these and other terms are used to define the tumors according to their ability to synthesize and/or release catecholamines in relation to biochemical test results. In such cases the term “silent” and other variants are often inappropriately and misleadingly used. In the present analysis we provide an overview of the literature and propose standardized terminology in an attempt at harmonization to facilitate scientific communication.

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Determinants of disease-specific survival in patients with and without metastatic pheochromocytoma and paraganglioma

Cited by 33 publications

References 39 publications

Cytoplasmic HIF-2α correlates to proliferation and predicts worse outcome in sympathetic paraganglioma

Cytoplasmic HIF-2α correlates to proliferation and predicts worse outcome in sympathetic paraganglioma

Updates in neuroendocrine neoplasms: From mechanisms to the clinic

Silent pheochromocytoma and paraganglioma: Systematic review and proposed definitions for standardized terminology

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