Abdominal catecholamine producing paragangliomas: A rare disease

14144 Background: Paragangliomas are rare extra-adrenal neuroendocrine tumors of chromaffin cells. It is estimated that 90 percent of paragangliomas are intra-abdominal. 50% of which are catecholamine secreting. Patients with abdominal masses are not routinely screened. A literature review and our experience is reported. Methods: A retrospective literature review of 85 cases reported over the past 26 years are discussed with regard to age, gender, clinical presentation, catecholamine status, preoperative evaluation, clinical course and outcome. Additionally, a review of compiled data from 1997 to 2005 identified 4 cases at our institution. Results: Males numbered 57.3% of the cases reviewed. The mean age was 57.9 years (range 11–76). The primary site of tumor included: 42 retroperitoneal, 44 intra-abdominal and 1 multifocal. 61/89 (68.5%) reported elevated urine and/or plasma catecholamines. Clinical presentations included, abdominal pain (10%), hematuria (3%), and a history of hypertension (46%). Only one case presented as an incidental finding. Primary work-up consisted of a CT scan in 86/89 (96.6%) of cases. Tumor size ranged from 1.2 cm to 25 cm. CAT scan preceded appropriate medical therapy with alpha blockade, and subsequent surgical excision via laparoscopy, laparotomy and one case of ERCP. Of the 63 cases which reported follow-up, 2 patients developed metastatic disease, 1 patient had unresolved hypertension and 60 were disease free. Conclusions: This retrospective review demonstrates the importance of pre-operative screening for safe and efficacious treatment of patients with paragangliomas. No significant financial relationships to disclose.