Functional Malignant Retroperitoneal Paraganglioma with Liver Metastasis: a Rare Case Report.

Paragangliomas are rare neoplasms. Their specific annual incidence is still unclear. These are rare neuroendocrine tumors which arise from extra-adrenal paraganglioma and they have the ability to secrete catecholamines. Most of them are diagnosed in the 3rd to 5th decades of life with mean age around 47 years. Majority of them are benign; however, malignant tumors with metastatic behavior are very rare. The incidence of malignant paraganglioma is estimated around 93/400 million people. The clinical course of metastatic malignant disease is variable and the reported 5-year survival is around 12-84%. There is no curative treatment option for malignant metastatic paraganglioma. If resectable, both, primary and metastasis should be resected. The only criteria which defines its malignancy is the presence of metastatic spread of chromaffin cells in tissues that normally do not contain such cells. Functional paraganglioma secretes excessive catecholamines which clinically manifest as paroxysmal hypertension, headache, sweating, and palpitations. We reported a case of young male who presented with huge left retroperitoneal mass and after evaluation found to have a functional malignant paraganglioma with liver metastasis. Surgical resection of the primary malignant paraganglioma with metastatectomy helps in decreasing the complications, improving the symptoms and prolonging the survival. © Indian Association of Surgical Oncology 2021.