Retroperitoneal paraganglioma, a rare cause of arterial hypertension

Abstract

Introduction: Paragangliomas are rare tumors in pediatric age, derived from sympathetic tissue of extra-adrenal location or parasympathetic tissue. Some of these tumors secrete catecholamines, being a rare cause of hypertension.
Clinical Case: A female adolescent, with 14 years old and unremarkable medical history, was referred to the Pediatric consultation due to episodes of holocranial headache associated with blurred vision with six months of evolution. Physical examination and brain magnetic resonance imaging revealed no abnormalities. Physical examination showed stage 1 hypertension, later confirmed by ambulatory blood pressure measurement. Analytical study, chest X-ray, electrocardiogram, and echocardiogram were normal. Renal echography revealed a heterogeneous mass contiguous to the left renal sinus and abdominal computed tomography showed a retroperitoneal lesion in the medial side of the left perirenal space. Due to retroperitoneal paraganglioma suspicion, the patient was referred to Porto’s Portuguese Institute of Oncology, where surgical excision of the lesion was performed. Anatomopathological examination confirmed diagnosis of sympathetic paraganglioma.
Conclusion: Although rare in pediatric age, paragangliomas represent the most common endocrine tumor. Biochemical and imaging evaluation are key for diagnosis, after which a genetic study is recommended due to its relevance for patient management and follow-up. Treatment consists in surgical excision, and complete remission is achieved in approximately 90% of cases.