# What should we focus on in pregnancy complicated by pheochromocytoma? a bibliometric analysis (1990-2024)

**Authors:** Shiyun Deng, Fan Li, Jing Tian, Congcong Sun, Yanqing Zhang

PMC · DOI: 10.3389/fonc.2025.1557376 · Frontiers in Oncology · 2025-07-17

## TL;DR

This paper analyzes research trends in pheochromocytoma during pregnancy to identify key areas for future study and improve maternal and fetal outcomes.

## Contribution

The study provides a bibliometric analysis of research on pheochromocytoma in pregnancy, highlighting gaps and suggesting future research directions.

## Key findings

- The United States and the Mayo Clinic are leading in research output on pheochromocytoma during pregnancy.
- Improved awareness and treatment have reduced maternal and infant mortality in this condition.
- Abdominal/pelvic tumor location and high catecholamine levels are linked to adverse outcomes.

## Abstract

The incidence of pheochromocytoma and paraganglioma (PPGL) in pregnancy is extremely low, yet it poses a significant threat to maternal and fetal safety. While studies are published annually, progress in this field remains slow. A key question is how researchers can better utilize limited case data to gain valuable insights. This bibliometric review summarizes the current research landscape, highlights recent findings, and suggests areas for future investigations.

A comprehensive search was conducted in the Web of Science Core Collection (WoSCC), PubMed, and Embase databases for PPGL in pregnancy from 1990 to 2024. Data analysis was performed using Excel 2022 and CiteSpace.

A total of 391 articles were included in the analysis. The United States was the most prolific country, and the Mayo Clinic was the most productive institution. Lenders JWM was identified as both the most published and most co-cited author. Journal of the Endocrine Society was the most frequently targeted journal for publication, while the Journal of Clinical Endocrinology & Metabolism had the highest number of co-citations. Evidence-based practice in this field primarily depends on case reports, case series, case-control studies, and systematic reviews. The primary focus in this research area is on clinical management and pregnancy complications.

Maternal and infant mortality in pheochromocytoma during pregnancy has significantly decreased due to improved awareness and advances in diagnosis and treatment. Antepartum diagnosis is the most vital element in reducing mortality; hypertension at admission and history of PPGL were independent factors of antepartum diagnosis. Abdominal/pelvic tumor location and catecholamine levels ≥10 times the upper limit of the reference range were associated with adverse outcomes. Women with hereditary disease and risk of developing PPGL should be screened before becoming pregnant for occult PPGL and should be treated adequately.

Enhanced collaboration between countries and institutions is needed to advance the field. Diagnostic and therapeutic strategies, as well as complications associated with PPGL during pregnancy, have consistently been core areas of research. Future studies should prioritize the clarification of detailed clinical management protocols and the underlying pathophysiological mechanisms, with the goal of generating high-quality evidence to guide the care of this high-risk population.

## Linked entities

- **Diseases:** pheochromocytoma (MONDO:0004974), paraganglioma (MONDO:0000448)

## Full-text entities

- **Genes:** ATRX (ATRX chromatin remodeler) [NCBI Gene 546] {aka JMS, MRX52, RAD54, RAD54L, XH2, XNP}, SDHA (succinate dehydrogenase complex flavoprotein subunit A) [NCBI Gene 6389] {aka CMD1GG, FP, MC2DN1, NDAXOA, PGL5, PPGL5}, EGLN2 (egl-9 family hypoxia inducible factor 2) [NCBI Gene 112398] {aka EIT-6, EIT6, HIF-PH1, HIFPH1, HPH-1, HPH-3}, NF1 (neurofibromin 1) [NCBI Gene 4763] {aka NFNS, VRNF, WSS}, SDHB (succinate dehydrogenase complex iron sulfur subunit B) [NCBI Gene 6390] {aka CWS2, IP, MC2DN4, PGL4, PPGL4, SDH}, CGB5 (chorionic gonadotropin subunit beta 5) [NCBI Gene 93659] {aka CGB, HCG}, SDHC (succinate dehydrogenase complex subunit C) [NCBI Gene 6391] {aka CYB560, CYBL, PGL3, PPGL3, QPS1, SDH3}, RET (ret proto-oncogene) [NCBI Gene 5979] {aka CDHF12, CDHR16, HSCR1, MEN2A, MEN2B, MTC1}, CDKN2B (cyclin dependent kinase inhibitor 2B) [NCBI Gene 1030] {aka CDK4I, INK4B, MTS2, P15, TP15, p15INK4b}, EGLN1 (egl-9 family hypoxia inducible factor 1) [NCBI Gene 54583] {aka C1orf12, ECYT3, HALAH, HIF-PH2, HIFPH2, HPH-2}, ALB (albumin) [NCBI Gene 213] {aka FDAHT, HSA, PRO0883, PRO0903, PRO1341}, SDHAF2 (succinate dehydrogenase complex assembly factor 2) [NCBI Gene 54949] {aka C11orf79, PGL2, PPGL2, SDH5, hSDH5}, SDHD (succinate dehydrogenase complex subunit D) [NCBI Gene 6392] {aka CBT1, CII-4, CWS3, MC2DN3, PGL, PGL1}, TMEM127 (transmembrane protein 127) [NCBI Gene 55654], IDH1 (isocitrate dehydrogenase (NADP(+)) 1) [NCBI Gene 3417] {aka HEL-216, HEL-S-26, IDCD, IDH, IDP, IDPC}, KIF1B (kinesin family member 1B) [NCBI Gene 23095] {aka CMT2, CMT2A, CMT2A1, HMSNII, KLP, NBLST1}, EPAS1 (endothelial PAS domain protein 1) [NCBI Gene 2034] {aka ECYT4, HIF2A, HLF, MOP2, PASD2, bHLHe73}, NPPB (natriuretic peptide B) [NCBI Gene 4879] {aka BNP, Iso-ANP}
- **Diseases:** vomiting (MESH:D014839), cardiac complications (MESH:D006331), acute renal failure (MESH:D058186), cerebrovascular stroke (MESH:D020521), Tumors (MESH:D009369), hypotension (MESH:D007022), renal failure (MESH:D051437), preterm labor (MESH:D007752), von Hippel-Lindau (VHL) disease (MESH:D006623), placental insufficiency (MESH:D010927), fetal distress (MESH:D005316), hereditary disease (MESH:D030342), IRDS (MESH:D012127), deaths (MESH:D003643), pulmonary edema (MESH:D011654), chest pain (MESH:D002637), miscarriage (MESH:D000022), hypovolemia (MESH:D020896), placental vasospasm (MESH:D020301), cardiovascular and multi-organ damage (MESH:D002318), PPGL (MESH:D010673), reflex tachycardia (MESH:D013610), orthostatic hypotension (MESH:D007024), adrenal tumors (MESH:D000310), proteinuria (MESH:D011507), Cushing's syndrome (MESH:D003480), ischemic bowel obstruction (MESH:D012778), uteroplacental insufficiency (MESH:D000309), arterial hypertension (MESH:D000081029), hypoxia (MESH:D000860), preterm delivery (MESH:D047928), breast cancer (MESH:D001943), malignant hypertension (MESH:D006974), intrauterine fetal loss (MESH:D005315), nausea (MESH:D009325), Carney Triad (MESH:C565803), Diabetes (MESH:D003920), Paraganglioma (MESH:D010235), pregnancy complications (MESH:D011248), dyspnea (MESH:D004417), spasm (MESH:D013035), Takotsubo cardiomyopathy (MESH:D054549), neuroendocrine tumors (MESH:D018358), myocardial infarction (MESH:D009203), respiratory depression (MESH:D012131), MEN2A (MESH:D018813), Hypertension (MESH:D006973), endocrine disorders (MESH:D004700), end-organ damage (MESH:C564816), placental abruption (MESH:D000037), gastrointestinal dysfunction (MESH:D005767)
- **Chemicals:** metanephrine (MESH:D008676), histamine (MESH:D006632), thiopental (MESH:D013874), propofol (MESH:D015742), metoclopramide (MESH:D008787), metoprolol (MESH:D008790), Doxazosin (MESH:D017292), succinylcholine (MESH:D013390), dopamine (MESH:D004298), cortisol (MESH:D006854), morphine (MESH:D009020), mivacurium (MESH:D000077590), rocuronium (MESH:D000077123), epinephrine (MESH:D004837), catecholamine (MESH:D002395), magnesium sulfate (MESH:D008278), glucose (MESH:D005947), remifentanil (MESH:D000077208), meperidine (MESH:D008614), alpha-adrenergic blockade (-), methyldopa (MESH:D008750), Labetalol (MESH:D007741), ephedrine (MESH:D004809), creatinine (MESH:D003404), sodium (MESH:D012964), norepinephrine (MESH:D009638), Phenoxybenzamine (MESH:D010643)
- **Species:** Homo sapiens (human, species) [taxon 9606]

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## References

79 references — full list in the complete paper: https://tomesphere.com/paper/PMC12312014/full.md

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Source: https://tomesphere.com/paper/PMC12312014