Background: Tumors of the paraganglionic system represent a distinct, albeit uncommon, clinical entity characterized by catecholamine hypersecretion and hemodynamic instability; initial pathologic examination often cannot predict benign vs malignant behavior. An analysis of the clinical outcome of patients with known malignant tumors may serve to enhance the initial evaluation and therapeutic plan of all patients presenting with pheochromocytoma or paraganglioma.
Methods: At the University of Texas M D Anderson Cancer Center, 30 patients with malignant abdominal paraganglioma and 20 patients with malignant pheochromocytoma were diagnosed between 1971 and 1995. Their medical records were reviewed with particular attention to clinical characteristics and disease outcome.
Results: Among the 30 patients with paraganglioma, 73% were men, and 90% were younger than 50 years at the time of diagnosis. Sixteen patients have remained alive with persistent disease 0.2 to 25 years after initial diagnosis while eight patients died of their disease within 0.8 to 32 years. Regional recurrence and skeletal metastases were the most prominent events. Among the 20 patients with pheochromocytoma, 60% were men and 70% were younger than 50 years at the time of diagnosis. Ten patients have remained alive with persistent disease 0.8 to 20 years after initial diagnosis while five patients died of their disease within 1.5 to 39 years. Hypertension was a prominent presenting feature and regional recurrence was the most frequent pattern of treatment failure.
Conclusions: Important clinical differences distinguish adrenal pheochromocytomas from extra-adrenal, abdominal paragangliomas. Patients with paragangliomas are, as a group, younger men, more likely to have malignant lesions and a more aggressive clinical course. Patients with malignant pheochromocytomas usually present with hypertension, are somewhat older, and have less aggressive disease.
We thank the staff of the Department of Medical Informatics for database retrieval and the clinical faculty who participated in the patients' care. We thank Teo Spear for expert preparation of the manuscript. We thank Terry Smith, biostatistician, for her critical review and suggestions.