Noradrenergic Pheochromocytoma: A Case Report.

Area of Science:

• Endocrinology
• Pediatric Oncology
• Surgical Oncology

Background:

• Pheochromocytomas and paragangliomas are rare neuroendocrine tumors originating from chromaffin cells.
• These tumors are exceptionally uncommon in children and often present with atypical symptoms.
• Early diagnosis and management are crucial for favorable outcomes.

Observation:

• A 17-year-old male presented with diffuse abdominal pain and a left retroperitoneal mass identified via CT scan.
• Diagnostic workup revealed exclusive 24-hour urinary normetanephrine hypersecretion, with normal metanephrine and 3-ortho-methyldopamine levels.
• The patient's blood pressure was managed with an alpha-blocker prior to surgical intervention.

Findings:

• Histopathological examination confirmed a pheochromocytoma with a low Pheochromocytoma of the Adrenal gland Scaled Score (PASS) of 2, indicating non-aggressive potential.
• Surgical adrenalectomy was performed, leading to a favorable clinical evolution.
• Post-operative follow-up demonstrated normalization of blood pressure and urinary catecholamines at one week, three months, six months, and one year.

Implications:

• This case highlights the importance of considering pheochromocytoma in pediatric patients with abdominal masses and atypical symptoms.
• Effective preoperative management of hypertension and surgical resection are key to successful treatment.
• Genetic testing for hereditary syndromes is recommended, though initial screening was negative in this patient.