Pheochromocytoma

Abstract

Objective: Pheochromocytomas are catecholamine-producing neuroendocrine tumors arising from chromaffin cells of the adrenal medulla or extra-adrenal paraganglia. There is no large-scale study in Taiwan at present. Our retrospective analysis was focused on clinical characteristics of Taiwanese patients with pheochromocytoma. Method: We retrospectively reviewed 48 patients who were pathologically proved as pheochromocytoma at the Kaohsiung Veterans General Hospital between Jan. 1990 and Jan. 2014. Parameters including patient and tumor characteristics, clinical presentation, treatment and long-term followup results were collected from the medical records. Metastasis was confirmed by pathologic proof using surgical removal or percutaneous biopsy. Result: Thirty-one patients (64.5%) had adrenal and 17 (35.4%) had extra-adrenal pheochromocytoma. The locations of extra-adrenal pheochromocytoma included the retroperitoneum in 6 patients (35%), head and neck in 4 (23%), scrotum in 2 (12%), urinary bladder in 2 patients (12%), multiple organs in 2 (12%), and mediastinum in 1 (6%). The most common symptom was sustained hypertension (52%). Five patients were diagnosed as malignant pheochromocytoma due to pathologically proved metastasis. Conclusion: Pheochromocytomas are rare neuro-endocrine tumors with a highly variable clinical presentation. Prompt surgical removal of pheochromocytoma ensures a better prognosis. Radical surgical removal of tumor with vigorous control of blood pressure is the mainstay to improve symptoms and survival for malignant pheochromocytoma. Multi-center and large-scale studies were warranted and Lifelong followup is strongly recommended for all patients with pheochromocytoma.