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Open access

Influence of secretory phenotype and preoperative preparation on surgical outcome in pheochromocytoma

Raluca Maria Furnica, Muhammad Muddaththir Dusoruth, Alexandre Persu, Damien Gruson, Michel Mourad, and Dominique Maiter

Objectives

Surgery of pheochromocytomas (PCs) still carries a high risk of haemodynamic complications during the perioperative period. We aimed to evaluate the influence of their secretory phenotype and preoperative alpha-blocker treatment on surgical outcome.

Design

A retrospective monocentric study at a tertiary medical centre.

Patients

In this study, 80 consecutive patients operated by the same team for a PC between 1988 and 2018.

Results

Diagnosis was based on typical symptoms and signs in 58 patients, genetic testing in 12 and work-up of an adrenal incidentaloma in 9. It was made during surgery in one patient. A genetic predisposition was found in one-third of index cases (21/62). The majority of the patients (73/79) had a secreting PC; more than 2/3 had an adrenergic phenotype and less than 1/3 a noradrenergic phenotype. The rate of perioperative haemodynamic complications was not influenced by the secretory phenotype, but persistent hypertension after surgery, recurrence and malignancy were more frequently observed in patients with a noradrenergic tumour. Preoperative alpha-blocker treatment was given for ≥ 14 days in 29 patients and, although being more symptomatic at diagnosis, these patients had less haemodynamic complications (3/29 vs 12/51 non-treated patients, P = 0.05).

Conclusions

The occurrence of haemodynamic complications during surgery was not significantly affected by the secretory phenotype in our study, but noradrenergic tumours show a worse post-surgical outcome. Our data also provide additional support in favour of a sufficient preoperative alpha-blockade in patients with pheochromocytoma.

DOI:
https://doi.org/10.1530/EC-20-0537
Volume 10: Issue 1,
Pages:
92–101 (10 total)
Open access

PAI-BEL: a Belgian multicentre survey of primary adrenal insufficiency

Natacha Driessens, Madhu Prasai, Orsalia Alexopoulou, Christophe De Block, Eva Van Caenegem, Guy T’Sjoen, Frank Nobels, Christophe Ghys, Laurent Vroonen, Corinne Jonas, Bernard Corvilain, and Dominique Maiter

Objective

Primary adrenal insufficiency (PAI) is a rare disease with an increasing prevalence, which may be complicated by life-threatening adrenal crisis (AC). Good quality epidemiological data remain scarce. We performed a Belgian survey to describe the aetiology, clinical characteristics, treatment regimens, comorbidities and frequency of AC in PAI.

Methods

A nationwide multicentre study involving 10 major university hospitals in Belgium collected data from adult patients with known PAI.

Results

Two hundred patients were included in this survey. The median age at diagnosis was 38 years (IQR 25–48) with a higher female prevalence (F/M sex ratio = 1.53). The median disease duration was 13 years (IQR 7–25). Autoimmune disease was the most common aetiology (62.5%) followed by bilateral adrenalectomy (23.5%) and genetic variations (8.5%). The majority (96%) of patients were treated with hydrocortisone at a mean daily dose of 24.5 ± 7.0 mg, whereas 87.5% of patients also received fludrocortisone. About one-third of patients experienced one or more AC over the follow-up period, giving an incidence of 3.2 crises per 100 patient-years. There was no association between the incidence of AC and the maintenance dose of hydrocortisone. As high as 27.5% of patients were hypertensive, 17.5% had diabetes and 17.5% had a diagnosis of osteoporosis.

Conclusion

This study provides the first information on the management of PAI in large clinical centres in Belgium, showing an increased frequency of postsurgical PAI, a nearly normal prevalence of several comorbidities and an overall good quality of care with a low incidence of adrenal crises, compared with data from other registries.

DOI:
https://doi.org/10.1530/EC-23-0044
Volume 12: Issue 6