Abstract
BackgroundParagangliomas 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.Case presentationWe 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 131I-Metaiodobenzylguanidine (131I-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.ConclusionThis 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.
Highlights
Paragangliomas occur most commonly in head and neck region [4], and much less frequently, they are found in the retroperitoneum [3]
Extraadrenal paragangliomas, which account for 5-10% of these tumors, are found in the retroperitoneum, thorax, and urinary bladder [5]; composite tumor of the retroperitoneum is rare
We report an unusual case of composite paraganglioma-ganglioneuroma in the retroperitoneum successfully treated with surgery
Summary
Pheochromocytomas arising from outside the adrenal glands are called paragangliomas [1] and constitute a relatively rare disease, arising from undifferentiated cells of the primitive neural crest [2,3]. She was admitted to our hospital for left femoral shaft fracture She underwent an abdominal contrast-enhanced CT scan because she developed left leg edema and was suspected of suffering deep vein thrombosis of the lower extremity, and a large retroperitoneal mass was discovered. She had no abdominal symptoms, had been in good health, and had no specific family, past medical, or drug history. An abdominal contrast-enhanced CT scan (Fig. 1) and magnetic resonance imaging (MRI) (Fig. 2) revealed a 6.5 cm heterogeneous retroperitoneal mass with a cystic component. TaFhibgeruocrwuetn5-sruerdfalceesisohnowietdh tawcoysdtifafnedreantwchoitmispholneesinotns (inacrrluodwinsg) The cut surface showed two different components including a brown-red lesion with a cyst and a whitish lesion (arrows)
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