Paragangliomas are neuroendocrine tumors and occur commonly in head and neck region and less frequently in the retroperitoneum. Multifocal paragangliomas are even rarer and highly suggestive of familial disease. To the best of our search, there are only two case reports of multiple retroperitoneal paragangliomas with no known familial association. This is the third report of this kind in the English literature.

A young adult with no significant past or family history presented with abdominal pain and anorexia. Abdominal examination revealed a soft nontender mass in the right hypochondrium. Contrast-enhanced computed tomography showed multiple retroperitoneal mass lesions. A clinicoradiological diagnosis of multicentric Castleman's disease/Lymphoma was made. Guided fine needle aspiration of the mass was suggestive of a neuroendocrine neoplasm. Tru-cut biopsy showed features of paraganglioma. Following this, the masses were excised and the diagnosis of paraganglioma was confirmed.

Multicentric retroperitoneal paragangliomas without any familial association are very rare with only two case reports in the English literature. Lack of symptoms makes the diagnosis difficult and also makes our case unique. Biopsy from paragangliomas and surgical intervention are known to cause life-threatening complications, such as profuse bleeding and abrupt changes in blood pressure. Hence, paragangliomas should be considered as a possibility, even if a remote one, in case of multicentric retroperitoneal tumors. This case also highlights the importance of cytology in the early diagnosis of retroperitoneal masses.

Singh A, Shikha D, Agarwal S, Khurana N, Jain SL, Hadke NS. Multiple Retroperitoneal Paragangliomas: An Uncommon Entity. World J Endoc Surg 2017;9(1):20-23.