# Therapeutic Strategy for Functional Metastatic Malignant Paraganglioma: A Case Report and Review of the Literature

## TL;DR

This case report describes the treatment journey of a young patient with a rare and aggressive neuroendocrine tumor called functional metastatic paraganglioma.

## Contribution

The paper presents the first case of metastatic functional paraganglioma managed in their department, highlighting treatment challenges and outcomes.

## Key findings

- Tri-chemotherapy showed poor hematological tolerance and limited progression-free survival.
- Sunitinib treatment also failed to produce a significant therapeutic response.
- The patient's overall survival was two years despite multiple treatment lines.

## Abstract

Paraganglioma is a rare neuroendocrine tumor that arises outside of the adrenal gland, typically originating from the chromaffin tissue of the sympathetic or parasympathetic ganglia. It can manifest at any age, with a peak incidence occurring between 40 and 50 years old. When the tumor secretes catecholamines, it is referred to as "functional." Currently, there is no standardized therapeutic approach. However, the management of metastatic forms is based on a systemic treatment with tri-chemotherapy.

Herein, we present the case of a young male patient with heavily metastatic functional malignant paraganglioma, which represents the first case managed in our department. After seven months of Somatuline treatment, our patient experienced disease progression. Subsequently, he received tri-chemotherapy comprising cyclophosphamide, vincristine, and dacarbazine, which proved to be suboptimal due to poor hematological tolerance and a progression-free survival of less than three months. In the third line of treatment, Sunitinib was administered, but the therapeutic response was poor, with clinical progression observed within two months, ultimately leading to the patient's demise at home. The overall survival was two years.