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

Area of Science:

• Endocrinology
• Oncology
• Surgical Pathology

Background:

• Paragangliomas are rare neuroendocrine tumors originating from extra-adrenal paraganglia.
• They possess the capacity to secrete catecholamines, leading to various clinical manifestations.
• While mostly benign, a small subset exhibits malignant and metastatic potential.

Observation:

• This report details a case of a young male with a large retroperitoneal mass.
• The patient was diagnosed with a functional malignant paraganglioma with liver metastasis.
• Malignant paraganglioma diagnosis is confirmed by metastatic chromaffin cells in ectopic locations.

Findings:

• Functional malignant paraganglioma can present with symptoms like hypertension, headache, sweating, and palpitations due to excessive catecholamine secretion.
• The incidence of malignant paraganglioma is approximately 93 per 400 million people.
• The 5-year survival rate for metastatic malignant paraganglioma varies significantly (12-84%).

Implications:

• Surgical resection of both the primary tumor and metastatic sites is crucial for managing resectable malignant paraganglioma.
• Metastasectomy alongside primary tumor resection can alleviate symptoms, decrease complications, and potentially prolong survival.
• Currently, no curative treatment exists for malignant metastatic paraganglioma, emphasizing the importance of surgical intervention when feasible.