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New guidelines recommend a minimum 10 years’ follow-up care for patients with pheochromocytoma or paraganglioma (PPGL)

Patients who have undergone surgery to remove a PPGL should receive a minimum of 10 years’ follow-up care, according to new guidelines published in the European Journal of Endocrinology, which also state that multiple parameters must be considered when determining the method of follow-up.

PPGL are rare tumours that arise from cells of the hormonal and nervous systems. They originate from the adrenal glands, which sit above the kidneys and secrete important hormones such as cortisol, or from paraganglia, which belong to the sympathetic system. Whilst the majority of PPGL are benign and can be surgically removed, a minority are malignant and therefore able to invade secondary locations within the body. Currently, a genetic cause can be identified for around 40% of PPGL, whilst those remaining occur sporadically.

The Endocrine Society recently produced guidelines focusing on the diagnosis and treatment of PPGL. However, detail on postoperative and long-term follow-up was missing, which is why the European Society of Endocrinology set up a guideline group chaired by Professor Pierre-Francois Plouin of Hopital Europeen Georges Pompidou, Paris, France. The group conducted a systematic review of 38 patient cohorts from 42 published studies, coupled with the analysis of records from 1153 patients from the European Network for the Study of Adrenal Tumours ([email protected]).

The guidelines address a variety of PPGL subtypes and recommend that whilst all patients receive a minimum of ten years’ follow-up care, high risk patient groups – such as the young and those with a genetic predisposition for the disease – should receive lifetime follow-up.

“The general follow-up procedure should include annual measurement of metanephrines, which are markers for PPGL, in both the blood and urine”, said Professor Plouin. “This would be standard for all patients. Measurement of chromogranin A, a protein elevated in some PPGL patients, is only recommended in a subgroup. Additional guidance is then given for specific conditions, such as pregnancy, the elderly, and frail patients”.