| Summary: | <p>Abstract</p> <p>Background</p> <p>Paragangliomas occur most commonly in head and neck region and much less frequently, they are found in the retroperitoneum. Composite paraganglioma-ganglioneuroma of the retroperitoneum is very rare.</p> <p>Case presentation</p> <p>We present an unusual case of retroperitoneal composite paraganglioma-ganglioneuroma discovered on computed tomography in a 63-year-old female patient. Routine hematological examination and biochemical tests were within normal limits. Plasma adrenaline was 0.042 ng/ml, plasma noradrenaline 0.341 ng/ml, and plasma dopamine <0.01 ng/ml. An abdominal contrast-enhanced CT scan and magnetic resonance imaging revealed a 6.5 cm heterogeneous retroperitoneal mass with a cystic component. The retroperitoneal tumor accumulated <sup>131</sup>I-Metaiodobenzylguanidine (<sup>131</sup>I-MIBG) 48 hours after radioisotope injection. Under the diagnosis of paraganglioma in the retroperitoneum, the patient underwent surgery. The resected tumor (6.5 × 5 × 3 cm) was solid and easily removed en bloc. The cut surface of the tumor and histology revealed two different components in the tumor: paraganglioma centrally and ganglioneuroma on the periphery. She remains disease-free 18 months after surgery.</p> <p>Conclusion</p> <p>This case reminds us that neuroendocrine tumor should be included in the differential diagnosis of a retroperitoneal mass although composite paraganglioma-ganglioneuroma in the retroperitoneum is very rare.</p>
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