Extra-adrenal Non-functioning Paraganglioma – A Diagnostic and Surgical Challenge

Abstract Paragangliomas are rare neoplasms arising from the neuroendocrine cells. Extra-adrenal paragangliomas arise from the neuroendocrine cells spread in the retroperitoneum from the skull base to the retroperitoneum and sacrum. Non-functioning paragangliomas are a diagnostic challenge as they are clinically silent and attain large size. The clinical implication of these non-functioning paragangliomas is the greater metastatic potential of extra adrenal paraganglioma (20–42%) when compared to adrenal paraganglioma. We report a very rare case of a 60-year lady who presented with abdominal heaviness and retroperitoneal lump. Imaging was suggestive of a retroperitoneal tumor. Serum chromogranin A level was elevated with normal catecholamine levels. The tumor was resected along with metastasectomy of isolated liver metastasis. The patient has no recurrence or metastasis at one year of follow-up. To conclude paraganglioma should be considered as a differential diagnosis in all retroperitoneal tumors. Serum chromogranin A levels and plasma metanephrines should be routinely done for all retroperitoneal cases before planning for the biopsy of the lesion. Resection of the lesions should always be performed as per the oncological principles as pre-operative differentiation of benign and malignant lesions is difficult.