[Phaeochromocytoma and paraganglioma]

E Cornu; I Belmihoub; N Burnichon; C Grataloup; F Zinzindohoué; S Baron; E Billaud; M Azizi; A-P Gimenez-Roqueplo; L Amar

doi:10.1016/j.revmed.2019.07.008

[Phaeochromocytoma and paraganglioma]

Rev Med Interne. 2019 Nov;40(11):733-741. doi: 10.1016/j.revmed.2019.07.008. Epub 2019 Sep 4.

[Article in French]

Authors

E Cornu¹, I Belmihoub¹, N Burnichon², C Grataloup³, F Zinzindohoué⁴, S Baron⁵, E Billaud⁶, M Azizi¹, A-P Gimenez-Roqueplo², L Amar⁷

Affiliations

¹ Unité d'hypertension artérielle, Centre de référence des maladies rares de la surrénale, université de Paris, hôpital européen Georges-Pompidou, AP-HP, 75015 Paris, France.
² Service de génétique, université de Paris, hôpital européen Georges-Pompidou, AP-HP, 75015 Paris, France; PARCC, Inserm UMR_970, équipe génétique et métabolisme des cancers rares, université de Paris, 75015 Paris, France.
³ Département de radiologie, université de Paris, hôpital européen Georges-Pompidou, AP-HP, 75015 Paris, France.
⁴ Service de chirurgie viscérale, université de Paris, hôpital européen Georges-Pompidou, AP-HP, 75015 Paris, France; PARCC, Inserm UMR_970, équipe génétique et métabolisme des cancers rares, université de Paris, 75015 Paris, France.
⁵ Département de physiologie, université de Paris, hôpital européen Georges-Pompidou, AP-HP, 75015 Paris, France.
⁶ Département de pharmacologie, université de Paris, hôpital européen Georges-Pompidou, AP-HP, 75015 Paris, France.
⁷ Unité d'hypertension artérielle, Centre de référence des maladies rares de la surrénale, université de Paris, hôpital européen Georges-Pompidou, AP-HP, 75015 Paris, France; PARCC, Inserm UMR_970, équipe génétique et métabolisme des cancers rares, université de Paris, 75015 Paris, France. Electronic address: laurence.amar@aphp.fr.

PMID: 31493938
DOI: 10.1016/j.revmed.2019.07.008

Abstract

Phaeochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors that arise from the adrenal medulla or sympathetic and parasympathetic ganglia. These tumors produce most often catecholamines in excess, causing hypertension and sometimes severe acute cardiovascular complications. The diagnosis is based on plasma or urines metanephrines measurements and on conventional and nuclear medicine imaging. Catecholamines-producing PPGL is very unlikely if levels are normal. The diagnosis of PPGL cannot be made without visualization of a tumor. Therapeutic management consists mostly of surgical excision, after drug preparation, and should be done in referral centers. About 40% of pheochromocytomas and paragangliomas occur in the context of an autosomal inherited syndrome, making genetic testing essential. The follow-up must be prolonged because a metastatic evolution or a recurrence can be observed in about 15% of the cases.

Keywords: Adrenal; Genetic; Génétique; Hypertension; Hypertension artérielle; Paragangliome; Paranglioma; Phaeochromocytoma; Phéochromocytome; Surrénale.

Publication types

Review

MeSH terms

Adrenal Gland Neoplasms / diagnosis*
Adrenal Gland Neoplasms / therapy*
Adrenal Glands / diagnostic imaging
Adrenalectomy
Adrenergic alpha-Antagonists / therapeutic use
Adrenergic beta-Antagonists / therapeutic use
Algorithms
Catecholamines / analysis
Continuity of Patient Care
Genetic Testing
Heart Diseases / etiology
Humans
Hypertension / etiology
Paraganglioma / diagnosis*
Paraganglioma / therapy*
Pheochromocytoma / diagnosis*
Pheochromocytoma / therapy*
Radiotherapy, Adjuvant

Substances

Adrenergic alpha-Antagonists
Adrenergic beta-Antagonists
Catecholamines