Pheochromocytoma is a rare cause of hypertension in pregnancy. Unrecognized, it carries a great risk for both mother and the foetus. The main reason for missing the diagnosis is the misconception that any hypertension occurring in pregnancy is gestational hypertension or pre (eclampsia). As many as 90% of patients report one or more pheochromocytoma-related symptoms antenatally, but the diagnosis is made in 75% of patients, meaning that 3 out of 10 patients are diagnosed after childbirth or post-mortem. The symptoms are similar to other more common causes of hypertension in pregnancy, which presents a major diagnostic challenge. The diagnosis is based on determination of metanephrines in plasma or 24-hour urine. Magnetic resonance imaging (MRI) and ultrasound (US) are used to localize the tumour. If the diagnosis is made before the 24th week of pregnancy, laparoscopic removal of the tumour in the second trimester is recommended. If diagnosed later, the tumour could be removed during or after delivery. Preoperative preparation with alpha blockers is required to stabilize blood pressure. The decision on the mode of delivery depends on several factors, so an experienced multidisciplinary team is needed to minimize maternal and foetal mortality.
ans that 3 out of 10 patients are diagnosed after childbirth or post-mortem (1).
2.
Donatini G, Kraimps J, Caillard C, Mirallie E, Pierre F, De Calan L, et al. Pheochromocytoma diagnosed during pregnancy: lessons learned from a series of ten patients. Surg Endosc. 2018;3890–900.
3.
Langton K, Tufton N, Akker S, Deinum J, Eisenhofer G, Timmers H, et al. Pregnancy and phaeochromocytoma/paraganglioma: clinical clues affecting diagnosis and outcome -a systematic review. BJOG. 2021;1264–72.
4.
Neumann H, Pawlu C, Peczkowska M, Bausch B, Mcwhinney S, Muresan M, et al. European-American Paraganglioma Study Group. Distinct clinical features of paraganglioma syndromes associated with SDHB and SDHD gene mutations. JAMA. 2004;943–51.
5.
Aufforth R, Ramakant P, Sadowski S, Mehta A, Trebska-Mcgowan K, Nilubol N, et al. Kebebew E. Pheochromocytoma screening initiation and frequency in von Hippel-Lindau syndrome. J Clin Endocrinol Metab. 2015;4498–504.
6.
Karasek D, Shah U, Frysak Z, Stratakis C, Pacak K. An update on the genetics of pheochromocytoma. J Hum Hypertens. 2013;141–7.
7.
Rončević T, Željković-Vrkić T, Kos J, Fištrek M. Feokromocitom -dijagnostički i terapijski izazov koji traje. (Pheochromocytoma -an ongoing diagnostic and therapeutic challenge). 2007;205–7.
Fishbein L. Pheochromocytoma and paraganglioma: genetics, diagnosis, and treatment. Hematol Oncol Clin North Am. 2016;135–50.
10.
Gruber L, Hartman R, Thompson G, Mckenzie T, Lyden M, Dy B, et al. Pheochromocytoma Characteristics and behaviour differ depending on method of discovery. J Clin Endocrinol Metab. 2019;1386–93.
11.
Kronenberg H, Melmed S, Polonsky K, Larsen R, Williams P. Textbook of Endocrinology. 2007;509–20.
12.
In conclusion, early diagnosis of pheochromocytoma significantly reduces the risk to mother and child and helps prevent potential complications. FUNDING No specific funding was received for this study. TRANSPARENCY DECLARATION Competing interests: None to declare.
13.
Lenders J, Langton K, Langenhuijsen J, Eisenhofer G. Pheochromocytoma and Pregnancy Endocrinol Metab Clin North Am. 2019;605–17.
14.
Sturgeon C, Angelos P. Current approach to pheochromocytoma. Oncology. 2006;1450–1.
15.
Pacak K, Linehan M, Eisenhofer G, Walther M, Goldstein D. Recent advances in genetics, diagnosis, localization, and treatment of pheochromocytoma. Annals of Internal Medicine. 2001;315–29.
16.
Plouin P, Amar L, Dekkers O, Fassnacht M, Gimenez-Roqueplo A, Lenders J, et al. Clinical Practice Guideline for long-term follow-up of patients operated on for a phaeochromocytoma or a paraganglioma. Eur J Endocrinol. 2016;1–10.
17.
Lenders J, Eisenhofer G. Update on modern management of pheochromocytoma and paraganglioma. Endocrinol Metab (Seoul). 2017;152–61.
18.
Rana S, Lemoine E, Granger J, Karumanchi S. Preeclampsia: Pathophysiology, challenges, and perspectives. Circ Res. 2019;1094–112.
19.
Sarathi V, Lila A, Bandgar T, Menon P, Shah N. Pheochromocytoma and pregnancy: a rare but dangerous combination. Endocr Pract. 2010;300–9.
20.
Santos D, Barbisan C, Marcellini C, Santos R. Pheochromocytoma and pregnancy: A case report and review. J Bras Nefrol. 2015;496–500.
21.
Orioli L, Debiève F, Donckier J, Mourad M, Lois F, Maiter D. Pheochromocytoma during pregnancy: case report and review of recent literature. Ann Endocrinol. 2017;480–4.
22.
Corsello S, Paragliola R. Evaluation and management of endocrine hypertension during pregnancy. Endocrinol Metab Clin North Am. 2019;829–42.
23.
Van Der Weerd K, Van Noord C, Loeve M, Knapen M, Visser W, De Herder W, et al. Endocrinology in pregnancy: Pheochromocytoma in pregnancy: case series and review of literature. Eur J Endocrinol. 2017;49–58.
24.
Farrugia F, Charalampopoulos A. Endocr Regul. 2019;191–212.
The statements, opinions and data contained in the journal are solely those of the individual authors and contributors and not of the publisher and the editor(s). We stay neutral with regard to jurisdictional claims in published maps and institutional affiliations.
,
