Laparoscopic Resection of a Large Functioning Paraganglioma at the Organ of Zuckerkandl: A Rare Case Report and Review of the Literature

Abstract

Paragangliomas are rare neuroendocrine tumors that arise from extra-adrenal sympathetic or paratympanic ganglia. Intra-abdominal paragangliomas are particularly uncommon, with an estimated incidence of approximately 1 in 500,000. Paratympanic paragangliomas are typically located in the head and neck region, while sympathetic paragangliomas are more often found in the abdomen, followed by the chest and pelvis. A large paraganglioma of the organ of Zuckerkandl (POZ) is extremely rare. Notably, about 53% of paragangliomas occur in the organ of Zuckerkandl, which lies anterolateral to the distal abdominal aorta, between the origin of the inferior mesenteric or renal artery and the aortic bifurcation. The clinical presentation of abdominal paragangliomas can include the classical triad of headache, palpitations, and excessive sweating. Many paragangliomas remain asymptomatic and are detected incidentally during imaging studies. A rare case of a large abdominal functioning paraganglioma arising from the organ of Zuckerkandl in a 20-year-old female patient was reported and diagnosed through abdominal ultrasonography and CT imaging. Abdominal ultrasonography revealed a rounded, homogeneous, hypoechoic, vascular lesion measuring 6 × 4.5 cm, located just below the umbilicus on the right side of the abdomen. A laparoscopic excision was performed with the patient in the supine position and steep Trendelenburg. The patient remained in the ICU on inotropic support for 48 hours, after which her blood pressure stabilized at 120/80 mmHg. Findings were consistent with a benign extra-adrenal paraganglioma. In conclusion, abdominal paragangliomas of the organ of Zuckerkandl are rare endocrine tumors. Among surgical options, the laparoscopic approach is considered the gold standard treatment.