Delayed diagnosis of retroperitoneal paraganglioma in an 8-year-old boy with persistent hypertension: a case report and review of diagnostic challenges in pediatric secondary hypertension
-
Dongxue Pan
Abstract
Objectives
This study aims to highlight the rare yet significant cause of secondary hypertension in pediatric patients, specifically pheochromocytoma (PCC) and paraganglioma (PGL) and to discuss the diagnostic challenges and lessons learned from misdiagnosis in a pediatric case.
Case presentation
We present the case of an 8-year-old boy with a 2-year history of sustained hypertension, initially attributed to brainstem encephalitis. Despite antihypertensive therapy, his blood pressure remained elevated. After 2 years, symptoms such as diaphoresis and failure to thrive prompted further investigation. Biochemical tests revealed elevated urinary normetanephrine levels, and imaging identified a retroperitoneal mass. The diagnosis of retroperitoneal paraganglioma was confirmed, and surgical intervention resulted in the normalization of blood pressure and symptom resolution during the 1-year follow-up.
Conclusions
This case underscores the importance of considering rare causes of secondary hypertension, such as PGL, even in the absence of classic symptoms. Early biochemical testing, appropriate imaging, and multidisciplinary follow-up are essential for accurate diagnosis and optimal management in pediatric hypertension. Genetic testing is also crucial, particularly in pediatric cases where hereditary factors are prevalent.
Acknowledgments
The authors thank the departments of pediatrics, surgery, pathology, and the nursing staff in providing care to this patient.
-
Research ethics: Informed written consent was taken from the participants’ parents. Institutional ethics review is not required for case reports.
-
Informed consent: Informed consent was obtained from the participant and participants’ parents.
-
Author contributions: The authors confirm contribution to the paper as follows: study conception and design: D.P., S.F.; draft manuscript preparation: D.P., S.F., S.C., C.P. All authors reviewed and approved the final version of the manuscript.
-
Use of Large Language Models, AI and Machine Learning Tools: Language improvement support was provided by ChatGPT. However, all text was written and approved by the authors, who take full responsibility for the content.
-
Conflict of interest: Authors declare no conflict of interest.
-
Research funding: None.
-
Data availability: All relevant data sources are cited in this article.
References
1. Flynn, JT, Kaelber, DC, Baker-Smith, CM, Blowey, D, Carroll, AE, Daniels, SR, et al.. Subcommittee on screening and management of high blood pressure in children. Clinical practice guideline for screening and management of high blood pressure in children and adolescents. Pediatrics;140:e20171904. https://doi.org/10.1542/peds.2017-1904.Suche in Google Scholar PubMed
2. Song, P, Zhang, Y, Yu, J, Zha, M, Zhu, Y, Rahimi, K, et al.. Global prevalence of hypertension in children: a systematic review and meta-analysis. JAMA Pediatr;173:1154–63. https://doi.org/10.1001/jamapediatrics.2019.3310.Suche in Google Scholar PubMed PubMed Central
3. Arar, MY, Hogg, RJ, Arant, BSJ, Seikaly, MG. Etiology of sustained hypertension in children in the southwestern United States. Pediatr Nephrol 1994;8:186–9. https://doi.org/10.1007/bf00865475.Suche in Google Scholar
4. Lenders, JW, Eisenhofer, G, Mannelli, M, Pacak, K. Phaeochromocytoma. Lancet 2005;366:665–75. https://doi.org/10.1016/s0140-6736(05)67139-5.Suche in Google Scholar PubMed
5. Linet, MS, Ries, LA, Smith, MA, Tarone, RE, Devesa, SS. Cancer surveillance series: recent trends in childhood cancer incidence and mortality in the United States. J Natl Cancer Inst 1999;91:1051–8. https://doi.org/10.1093/jnci/91.12.1051.Suche in Google Scholar PubMed
6. Whalen, RK, Althausen, AF, Daniels, GH. Extra-adrenal pheochromocytoma. J Urol 1992;147:1. https://doi.org/10.1016/s0022-5347(17)37119-7.Suche in Google Scholar PubMed
7. Soltani, A, Pourian, M, Davani, BM. Does this patient have pheochromocytoma? A systematic review of clinical signs and symptoms. J Diabetes Metab Disord 2015;15:6. https://doi.org/10.1186/s40200-016-0226-x.Suche in Google Scholar PubMed PubMed Central
8. Bonomaully, M, Khong, T, Fotriadou, M, Tully, J. Anxiety and depression related to elevated dopamine in a patient with multiple mediastinal paragangliomas. Gen Hosp Psychiatr 2014;36:449.e7–449.e8. https://doi.org/10.1016/j.genhosppsych.2014.03.003.Suche in Google Scholar PubMed
9. Dorji, T, Verma, V, Menon, A. Epistaxis, paroxysmal anxiety episodes, and hypertension in a child with SDHB-associated paraganglioma: a case report. Clin Case Rep 2022;10:e6683. https://doi.org/10.1002/ccr3.6683.Suche in Google Scholar PubMed PubMed Central
10. Jain, V, Yadav, J, Satapathy, AK. Pheochromocytoma presenting as diabetes insipidus. Indian Pediatr 2013;50:1056–7.Suche in Google Scholar
11. Pamporaki, C, Hamplova, B, Peitzsch, M, Prejbisz, A, Beuschlein, F, Timmers, HJLM, et al.. Characteristics of pediatric vs adult pheochromocytomas and paragangliomas. J Clin Endocrinol Metab 2017;102:1122–32. https://doi.org/10.1210/jc.2016-3829.Suche in Google Scholar PubMed PubMed Central
12. Bausch, B, Wellner, U, Bausch, D, Schiavi, F, Barontini, M, Sanso, G, et al.. Long-term prognosis of patients with pediatric pheochromocytoma. Endocr Relat Cancer 2013;21:17–25. https://doi.org/10.1530/erc-13-0415.Suche in Google Scholar PubMed
13. Lenders, JW, Duh, QY, Eisenhofer, G, Gimenez-Roqueplo, AP, Grebe, SK, Murad, MH, et al.. Endocrine Society. Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab 2014;99:1915–42. https://doi.org/10.1210/jc.2014-1498.Suche in Google Scholar PubMed
14. Pham, TH, Moir, C, Thompson, GB, Zarroug, AE, Hamner, CE, Farley, D, et al.. Pheochromocytoma and paraganglioma in children: a review of medical and surgical management at a tertiary care center. Pediatrics 2006;118:1109–17. https://doi.org/10.1542/peds.2005-2299.Suche in Google Scholar PubMed
15. Eisenhofer, G, Lenders, JW, Siegert, G, Bornstein, SR, Friberg, P, Milosevic, D, et al.. Plasma methoxytyramine: a novel biomarker of metastatic pheochromocytoma and paraganglioma in relation to established risk factors of tumour size, location and SDHB mutation status. Eur J Cancer 2012;48:1739–49. https://doi.org/10.1016/j.ejca.2011.07.016.Suche in Google Scholar PubMed PubMed Central
16. Pamporaki, C, Berends, AMA, Filippatos, A, Prodanov, T, Meuter, L, Prejbisz, A, et al.. Prediction of metastatic pheochromocytoma and paraganglioma: a machine learning modelling study using data from a cross-sectional cohort. Lancet Digit Health 2023;5:e551–9. https://doi.org/10.1016/s2589-7500(23)00094-8.Suche in Google Scholar
17. Eisenhofer, G, Goldstein, DS, Sullivan, P, Csako, G, Brouwers, FM, Lai, EW, et al.. Biochemical and clinical manifestations of dopamine-producing paragangliomas: utility of plasma methoxytyramine. J Clin Endocrinol Metab 2005;90:2068–75. https://doi.org/10.1210/jc.2004-2025.Suche in Google Scholar PubMed
© 2025 Walter de Gruyter GmbH, Berlin/Boston
Artikel in diesem Heft
- Frontmatter
- Editorial
- Endocrine treatment in Duchenne muscular dystrophy – current practices and future directions
- Reviews
- Pubertal induction therapy in pediatric patients with Duchenne muscular dystrophy
- Evaluating obesity and fat cells as possible important metabolic players in childhood leukemia
- Biological effects of recombinant human growth hormone therapy on metabolism in children with growth hormone deficiency: a review
- Original Articles
- The use of bisphosphonate and testosterone in young people with Duchenne muscular dystrophy: an international clinician survey
- Characterizing the metabolome of children with growth hormone deficiency
- Is L-dopa test effective in detecting adrenal insufficiency with preliminary diagnosis of growth hormone deficiency in children with short stature?
- Comparison of the clinical characteristics of children with Silver–Russell syndrome genetically confirmed or not and their response to growth hormone therapy: a national multicenter study
- Testicular adrenal rest tumors in Indonesian boys with congenital adrenal hyperplasia
- Oxidative stress in branched-chain organic acidemias using thiol-disulfide homeostasis
- Case Reports
- Delayed diagnosis of retroperitoneal paraganglioma in an 8-year-old boy with persistent hypertension: a case report and review of diagnostic challenges in pediatric secondary hypertension
- Pediatric iatrogenic Cushing’s syndrome: a series of seven cases induced by topical corticosteroid use
- Wolcott–Rallison syndrome: late-onset diabetes, multiple epiphyseal dysplasia, and acute liver failure – a case report
Artikel in diesem Heft
- Frontmatter
- Editorial
- Endocrine treatment in Duchenne muscular dystrophy – current practices and future directions
- Reviews
- Pubertal induction therapy in pediatric patients with Duchenne muscular dystrophy
- Evaluating obesity and fat cells as possible important metabolic players in childhood leukemia
- Biological effects of recombinant human growth hormone therapy on metabolism in children with growth hormone deficiency: a review
- Original Articles
- The use of bisphosphonate and testosterone in young people with Duchenne muscular dystrophy: an international clinician survey
- Characterizing the metabolome of children with growth hormone deficiency
- Is L-dopa test effective in detecting adrenal insufficiency with preliminary diagnosis of growth hormone deficiency in children with short stature?
- Comparison of the clinical characteristics of children with Silver–Russell syndrome genetically confirmed or not and their response to growth hormone therapy: a national multicenter study
- Testicular adrenal rest tumors in Indonesian boys with congenital adrenal hyperplasia
- Oxidative stress in branched-chain organic acidemias using thiol-disulfide homeostasis
- Case Reports
- Delayed diagnosis of retroperitoneal paraganglioma in an 8-year-old boy with persistent hypertension: a case report and review of diagnostic challenges in pediatric secondary hypertension
- Pediatric iatrogenic Cushing’s syndrome: a series of seven cases induced by topical corticosteroid use
- Wolcott–Rallison syndrome: late-onset diabetes, multiple epiphyseal dysplasia, and acute liver failure – a case report
