Retroperitoneal Nonchromaffin Paraganglioma

Abstract

The paragangliomas, also known as chemodectoma or nonchromaffin paraganglioma tumor, belong to neuroendocrine tumors. They are generally named according to anatomical sites, such as the carotid body tumor and jugular body tumor (referring to paragangliomas located in the carotid body). Paraganglioma is common in adults aged 30–60 years, while patients with retroperitoneal paraganglioma are younger (mostly 30–40 years old) than those with head and neck paraganglioma. Malignant paraganglioma may occur at a younger age. Retroperitoneal paragangliomas are rare tumors, with the incidence equivalent between men and women. They are mostly solitary and sporadic and occasionally multiple. Patients often have a family history. Some cases are accompanied by paragangliomas in other parts of the body or complicated with gastrointestinal stromal tumor (GIST) and pulmonary chondroma as Carney’s triad.