| Summary: | <p>Abstract</p> <p>Background</p> <p>Extra-adrenal, intra-abdominal paraganglioma constitutes a rare neoplasm and, moreover, its location in the greater omentum is extremely infrequent.</p> <p>Case presentation</p> <p>A 46-year-old woman with an unremarkable medical history presented with an asymptomatic greater omentum mass that was discovered incidentally during ultrasonographic evaluation due to menstrual disturbances. Clinical examination revealed a mobile, non-tender, well-circumscribed mass in the right upper and lower abdominal quadrant. Blood tests were normal. Contrast-enhanced abdominal computed tomography (CT) scan confirmed a huge (15 × 15 cm), well-demarcated, solid and cystic, heterogeneously enhanced mass between the right liver lobe and right kidney. Exploratory laparotomy revealed a large mass in the greater omentum. The tumor was completely excised along with the greater omentum. Histopathology offered the diagnosis of benign greater omentum paraganglioma. After an uneventful postoperative course, the patient was discharged on the 4<sup>th </sup>postoperative day. She remains free of disease for 2 years as appears on repeated CT scans as well as magnetic resonance imaging (MRI) and scintigraphy performed with radiotracer-labeled metaiodobenzyl-guanidine (MIBG) scans.</p> <p>Conclusion</p> <p>This is the second reported case of greater omentum paraganglioma. Clinical and imaging data of patients with extra-adrenal, intra-abdominal paragangliomas are variable while many of them may be asymptomatic even when the lesion is quite large. Thorough histopathologic evaluation is imperative for diagnosis and radical excision is the treatment of choice. Since there are no definite microscopic criteria for the distinction between benign and malignant tumors, prolonged follow-up is necessary.</p>
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