Malignant pheochromocytoma is a rare disease with a high mortality. Surgical resection is the only effective treatment if extensive metastatic disease is not present. However, differentiating between benign and malignant pheochromocytoma is impossible in the absence of locoregional invasion or distant metastasis. This diagnostic dilemma has several drawbacks, including later detection and treatment of recurrence than if malignancy is determined at the original operation. With emerging molecular markers of malignant disease, optimal extent and approach for surgical treatment and appropriate extent of follow up could be established based on specific tumor behavior and the need for additional systemic therapy.
