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Paraganglioma presenting with marked proteinuria: a case report
Suna Emir1, Hacı A Demir, Burcu Güven
1Departments of *Pediatric Hematology Oncology †Pathology, SB Ankara Children's Hematology Oncology Education and Research Hospital ‡Department of Pediatric Surgery, Başkent University Faculty of Medicine, Ankara, Turkey.
Journal of Pediatric Hematology/Oncology
|November 17, 2012
Summary
This study reports the first pediatric case of a functional paraganglioma presenting with massive proteinuria. Surgical removal of the neuroendocrine tumor resolved hypertension and proteinuria in an 8-year-old boy.
Area of Science:
- Pediatric Endocrinology
- Oncology
- Nephrology
Background:
- Paragangliomas are rare neuroendocrine tumors originating from sympathetic and parasympathetic paraganglia.
- In children, these tumors are often functional, leading to symptoms from catecholamine excess like hypertension, headaches, and palpitations.
- While proteinuria is a rare manifestation in adults, its presentation in children is less documented.
Observation:
- An 8-year-old boy presented with significant hypertension, a heart murmur, and marked proteinuria.
- Imaging identified a large retroaortic and suprarenal mass.
- Biochemical tests confirmed high catecholamine levels, indicative of paraganglioma.
Findings:
- Pathological examination confirmed the diagnosis of paraganglioma.
- The child underwent complete surgical resection of the tumor.
- Post-operatively, the patient's hypertension, proteinuria, and cardiac abnormalities resolved.
Implications:
- This case highlights massive proteinuria as a rare presenting symptom of pediatric paraganglioma.
- Early diagnosis and surgical intervention are crucial for favorable outcomes in children with functional paragangliomas.
- Further research may elucidate the mechanisms linking paragangliomas to proteinuria in pediatric populations.
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