Maternal and fetal outcomes in phaeochromocytoma and pregnancy: a multicentre retrospective cohort study and systematic review of literature

Bancos, Irina; Atkinson, Elizabeth J.; Eng, Charis; Young, William F.; Neumann, Hartmut P. H.; Yukina, Marina; Cohen, Debbie L.; Waguespack, Steven G.; Pereira, Maria Adelaide Albergaria; He, Xin; Bandgar, Tushar; Donatini, Gianluca; Qi, Xiao-Ping; Cohn, Aviva; Roslyakova, Anna; Letizia, Claudio; Fishbein, Lauren; Kaur, Ravinder; Iñiguez-Ariza, Nicole M.; Murad, Mohammad Hassan; Gruber, Lucinda; Wachtel, Heather; Черенько, С. М.; Jiménez, Camilo; Else, Tobias; Ramteke-Jadhav, Swati; Fang, Xu-Dong; Vaidya, Anand; Petramala, Luigi; Beltsevich, Dmitry; Walz, Martin K.; Corssmit, Eleonora P M; Wohllk, Nelson; Tufton, Nicola; Links, Thera P.; Ferrara, Alfonso Massimiliano; Tsoy, Uliana; Donegan, Diane; Pęczkowska, Mariola; Timmers, Henri J L M; Morelli, Valentina; Ebbehøj, Andreas; Kirschner, Lawrence S.; Kunavisarut, Tada; Larsson, Catharina; Kudlai, Inna; Hasse-Lazar, Kornelia; Barczyński, Marcin; Deutschbein, Timo; Langton, Katharina; Rasmussen, Åse Krogh; Dvořáková, Šárka; Miller, Julie Ann; Liu, Longfei; Bennett, Bonita; Huang, Yi; Yu, Zhixian; Jaiswal, Sanjeet Kumar; Shah, Nalini; Diaz, Rene Eduardo; Dullaart, Robin P. F.; Akker, Scott; Drake, William M; Boaretto, Francesca; Zovato, Stefania; Barbon, Giovanni; Taschin, Elisa; Schiavi, Francesca; Grineva, Elena; Rappaport, Maximilien; Skierczynski, Paul; Fassnacht, Martin; Calissendorff, Jan; Juhlin, Carl Christofer; Vlĕek, Petr; Li, Minghao; Jonasch, Eric; Prokop, Larry J.; Jovanović, Milan; Lechan, Ronald M.; Erenler, Feyza; Garla, Vishnu; Bobryk, M.I.; Kovalenko, Andrey Y; Hodson, Emma J.; Jenner, Bernadette; Simpson, Helen; Casey, Ruth; Gimm, Oliver; Yie, Joanne Ngeow Yuen; Shafigullina, Zulfiya; Bugalho, Maria João; Rizzati, Silvia; Fraenkel, Merav; Sherlock, Mark; Sarma, Debajit; Saikia, Uma Kaimal; Riester, Anna; Quinkler, Marcus; Zschiedrich, Stefan; Seufert, Jochen; Bausch, Birke; Zavrashvili, Nino; Søndergaard, Esben; Mathiesen, Jes Sloth; Robaczyk, Maciej; Poulsen, Per Løgstrup; Vasilkova, Volha; Costa-Barbosa, Flávia A.; Kater, Cláudio E.; Şimşir, Ilgın Yıldırım; Uğurlu, M. Ümit; Soyaltin, Utku E; Makay, Özer; Ivanov, N.; Opocher, Giuseppe; Egorov, V.; Petrov, Roman; Khudiakova, Natalia Valeryevna

doi:10.1016/s2213-8587(20)30363-6

Cited by 48 publications

(99 citation statements)

References 31 publications

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“…Similar results were reported by Langton et al in a recent systematic review of 2020 in 204 pregnant women with pheochromocytoma (maternal and foetal/neonatal mortalities were 9.0% (18/200) and 14.2% (29/204), respectively) [15]. Table 1 describes maternal and foetal mortality in series of pheochromocytoma and PGLs during pregnancy [14][15][16][17][18][19].…”

Section: Epidemiologysupporting

confidence: 82%

“…In these patients, caesarean delivery is preferred, as it appears to carry a lower risk of maternal death than vaginal delivery [7,10], although the latter could be considered in selected patients previously treated [10]. In this way, a recent systematic review found that the type of delivery was not associated with adverse outcomes (caesarean 78% vs vaginal 22%, P = 0.24), although caesarean section was two times more common than vaginal delivery and usually reserved for patients with a higher degree of catecholamine excess [16]. Management of PGL during pregnancy may require modification of these recommendations depending on the location of the tumour [29] (Fig.…”

Section: Treatmentmentioning

confidence: 99%

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Special situations in pheochromocytomas and paragangliomas: pregnancy, metastatic disease, and cyanotic congenital heart diseases

et al. 2021

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The aim of our study was to describe the epidemiology, diagnosis, and treatment of the most complex pheochromocytoma and paraganglioma (PGL) cases, including pheochromocytoma/PGL during pregnancy, in cyanotic congenital heart diseases (CCHDs), and metastatic pheochromocytoma. The English and Spanish literature was thoroughly evaluated searching for articles reporting clinical studies, case reports, or reviews of pheochromocytoma/PGL in pregnancy and in CCHD and metastatic pheochromocytoma/PGL. Particular settings in the diagnosis and management of pheochromocytoma and PGLs remain challenging. Those special situations include the diagnosis during pregnancy or in the context of CCHD since the typical clinical features of pheochromocytoma may be confounded with preeclampsia during pregnancy and with the complications commonly observed in CCHD. In addition, although some clinical and genetic features have been associated with higher risk of metastatic pheochromocytoma, the detection and prediction of the development of metastatic disease involve another complex situation that may require special hormonal determinations as plasmatic 3-methoxytyramine and nuclear medicine studies including 18 FDG PET-CT or 18 F-FDOPA PET-CT, among others. Furthermore, the selection of the most appropriate treatment in these situations, as well as the moment to carry it out, requires special care as limited evidence is available. This article reviews the epidemiology, diagnosis, and treatment of the pheochromocytoma/PGL during pregnancy, metastatic pheochromocytoma/PGL, and pheochromocytoma/PGL in CCHD. The diagnosis, and especially the treatment, of metastatic pheochromocytomas and pheochromocytoma/PGL during pregnancy and in CCHD is challenging. Thus, these cases should be management in reference centres by multidisciplinary teams specialized in the pheochromocytoma/PGL treatment.

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Section: Epidemiologysupporting

confidence: 82%

Section: Treatmentmentioning

confidence: 99%

Special situations in pheochromocytomas and paragangliomas: pregnancy, metastatic disease, and cyanotic congenital heart diseases

et al. 2021

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“…Furthermore, prospective morphological and biochemical screening in a French series of 156 NF1 patients found a pheochromocytoma prevalence of 7.7% (12 patients), half (n = 6) of whom were secreting, and with only two of them symptomatic ( 81 ). This study and others showing the positive impact of genetic testing on the management and outcome of patients with paraganglioma-pheochromocytoma, and the poor prognosis of PPGL during pregnancy suggest that despite current recommendations, a screening for PPGL, at least before pregnancy, would improve the prognosis of these patients ( 82 – 85 ).…”

Section: Pheochromocytoma/paraganglioma (Ppgl) In Phakomatosesmentioning

confidence: 71%

Phakomatoses and Endocrine Gland Tumors: Noteworthy and (Not so) Rare Associations

Chevalier

Dupuis²,

Jannin

et al. 2021

Front. Endocrinol.

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Phakomatoses encompass a group of rare genetic diseases, such as von Hippel-Lindau syndrome (VHL), neurofibromatosis type 1 (NF1), tuberous sclerosis complex (TSC) and Cowden syndrome (CS). These disorders are due to molecular abnormalities on the RAS-PI3K-Akt-mTOR pathway for NF1, TSC and CS, and to hypoxia sensing for VHL. Phakomatoses share some phenotypic traits such as neurological, ophthalmological and cutaneous features. Patients with these diseases are also predisposed to developing multiple endocrine tissue tumors, e.g., pheochromocytomas/paragangliomas are frequent in VHL and NF1. All forms of phakomatoses except CS may be associated with digestive neuroendocrine tumors. More rarely, thyroid cancer and pituitary or parathyroid adenomas have been reported. These susceptibilities are noteworthy, because their occurrence rate, prognosis and management differ slightly from the sporadic forms. The aim of this review is to summarize current knowledge on endocrine glands tumors associated with VHL, NF1, TSC, and CS, especially neuroendocrine tumors and pheochromocytomas/paragangliomas. We particularly detail recent advances concerning prognosis and management, especially parenchyma-sparing surgery and medical targeted therapies such as mTOR, MEK and HIF-2 α inhibitors, which have shown truly encouraging results.

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“…PPGL in pregnancy is rare, and many times goes unrecognized until later, potentially causing severe fetal and maternal complications of catecholamine excess [ 139 ]. In a recent combined retrospective multi-center study and systematic review of 249 pregnancies in 232 women with PPGL, only 15% of pregnancies were reported in women with known PPGL prior to conceiving [ 140 ]. Around half of the cohort were women discovered with PPGL during the course of pregnancy, and a third were diagnosed with PPGL only after and within 1 year after delivery or miscarriage.…”

Section: Resultsmentioning

confidence: 99%

“…Notably, no complications of catecholamine excess occurred in women with known PPGLs prior to conceiving, despite the higher rate of metastatic disease in this subgroup, possibly due to more intense monitoring during pregnancy, lower degree of catecholamine excess, and optimal management during pregnancy. Unrecognized PPGL (odds ratio of 27), abdominal or pelvic location of PPGL (odds ratio of 11), and severe catecholamine excess of at least 10 times the upper normal limit (odds ratio of 4.7) were associated with adverse outcomes [ 140 ]. Optimal alpha-blockade during pregnancy was associated with fewer complications (odds ratio of 3.6 for absence of alpha-blockade), suggesting that initiation, dose titration and optimal medical management during pregnancy is key in pregnant women with PPGL.…”

Section: Resultsmentioning

confidence: 99%

Pheochromocytomas and Abdominal Paragangliomas: A Practical Guidance

Calissendorff

Juhlin

Bancos

et al. 2022

Cancers

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Pheochromocytomas and abdominal paragangliomas (PPGLs) are rare tumors arising from the adrenal medulla or the sympathetic nervous system. This review presents a practical guidance for clinicians dealing with PPGLs. The incidence of PPGLs has risen. Most cases are detected via imaging and less present with symptoms of catecholamine excess. Most PPGLs secrete catecholamines, with diffuse symptoms. Diagnosis is made by imaging and tests of catecholamines. Localized disease can be cured by surgery. PPGLs are the most heritable of all human tumors, and germline variants are found in approximately 30–50% of cases. Such variants can give information regarding the risk of developing recurrence or metastases as well as the risk of developing other tumors and may identify relatives at risk for disease. All PPGLs harbor malignant potential, and current histological and immunohistochemical algorithms can aid in the identification of indolent vs. aggressive tumors. While most patients with metastatic PPGL have slowly progressive disease, a proportion of patients present with an aggressive course, highlighting the need for more effective therapies in these cases. We conclude that PPGLs are rare but increasing in incidence and management should be guided by a multidisciplinary team.

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Maternal and fetal outcomes in phaeochromocytoma and pregnancy: a multicentre retrospective cohort study and systematic review of literature

Cited by 48 publications

References 31 publications

Special situations in pheochromocytomas and paragangliomas: pregnancy, metastatic disease, and cyanotic congenital heart diseases

Special situations in pheochromocytomas and paragangliomas: pregnancy, metastatic disease, and cyanotic congenital heart diseases

Phakomatoses and Endocrine Gland Tumors: Noteworthy and (Not so) Rare Associations

Pheochromocytomas and Abdominal Paragangliomas: A Practical Guidance

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