Approach to the Patient with Metastatic Pheochromocytoma and Paraganglioma

Abstract Pheochromocytomas and paragangliomas (PPGLs) are rare neural crest-derived tumors with malignant potential and a highly variable natural history, where some patients achieve a cure through surgical resection, while others experience an aggressive and protracted disease course characterized by recurrence and metastasis. While currently no definitive curative treatment exists for metastatic PPGL, ongoing trials and advances in biology of the disease present a beacon of hope. We present a case that illustrates a 15-year treatment journey, illustrating the complexity of metastatic PPGL treatment with different modalities, each with distinct efficacy and toxicity profiles. The choice of treatment is often an art, as much as it is based on evidence, as the clinician must balance among several factors, including tumor-related (pace of progression, tumor burden) and patient-related (functional status, symptoms, general health) ones. Through a stepwise approach, this discussion aims to provide insights into the evolving landscape of metastatic PPGL management.