Endocrine Abstracts

Introduction: Pheochromocytoma and paraganglioma are rare in the pediatric population occurring in approximately 1 in 50 000 children. The clinical presentation may range from asymptomatic to the classic triad of episodic diaphoresis, headache, and palpitations. Among hypertensive children, the incidence of surgically confirmed pheochromocytoma or catecholamine-secreting paraganglioma ranges from 0.8 to 1.7 percent. Catecholamine-secreting paragangliomas are often located in the superior and inferior para-aortic areas (75 percent of extra-adrenal tumors); the bladder (10 percent); the thorax (10 percent); and the skull base, neck, and pelvis (5 percent). Surgery plays an important role in managing the disease. Paragangliomas usually require an open surgical approach. Meanwhiles, laparoscopic surgery may be challenging in pediatric patients with paraganglioma especially in retroperitoneal location. We herein report a case of a paraganglioma that was located retroperitoneal area successfully resected by laparoscopic surgery.

Case presentation: We report a 16-year-old male admitted to the hospital due to intermittent generalized tonic – clonic seizures. With recent history of hypertension being treated irregularly with unknown medication. Further evaluation conducted revealed his hypertension was paroxysmal and in accompanied with classic triad of adrenergic symptoms consisting of headache, palpitations, diaphoresis and elevated levels of catecholamines in the blood and urine samples. Abdominal computed tomography revealed a 4×3 cm retroperitoneal mass likely to be paraganglioma. The patient underwent laparoscopic surgery and the tumor was dissected completely with no complications.

Discussion: Incidence rates of pheochromocytoma and paraganglioma are estimated at 0.3 cases per million per year, with approximately 20 percent of cases diagnosed during childhood. In children, approximately 80 percent of catecholamine--secreting tumors are pheochromocytomas and 20 percent are paragangliomas. Seizure without any other neurological diseases is a rare symptom in pediatric paraganglioma. This condition could be a consequence of paroxysmal hypertension. Surgical resection is the primary treatment in which open surgical approach is more favorable in the management of paraganglioma. Moreover, resection of retroperitoneal paragangliomas is often a surgical challenge especially in pediatric population. However, endoscopic surgery has been properly performed in this patient without any complication.

Conclusion: Paragangliomas presented with seizure are extremely rare in pediatric population. Laparoscopic surgery which is a challenging procedure to this patient group was conducted successfully in the present case.