CLINICAL OUTCOME OF 50 PATIENTS WITH MALIGNANT ABDOMINAL PARAGANGLIOMAS AND MALIGNANT PHEOCHROMOCYTOMAS

Background: Tumors of the paraganglionic system represent a distinct, albeit uncommon, clinical entity characterized by catecholamine hypers ecretion and hemodynamic instability; initial pathologic examination o ften cannot predict benign vs malignant behavior. An analysis of the c linical outcome of patients with known malignant tumors may serve to e nhance the initial evaluation and therapeutic plan of all patients pre senting with pheochromocytoma or paraganglioma. Methods: At the Univer sity of Texas M D Anderson Cancer Center, 30 patients with malignant a bdominal paraganglioma and 20 patients with malignant pheochromocytoma were diagnosed between 1971 and 1995. Their medical records were revi ewed 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. Sixt een patients have remained alive with persistent disease 0.2 to 25 yea rs after initial diagnosis while eight patients died of their disease within 0.8 to 32 years. Regional recurrence and skeletal metastases we re the most prominent events. Among the 20 patients with pheochromocyt oma, 60% were men and 70% were younger than 50 years at the time of di agnosis. 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 presentin g feature and regional recurrence was the most frequent pattern of tre atment failure. Conclusions: Important clinical differences distinguis h adrenal pheochromocytomas from extraadrenal, abdominal paraganglioma s. Patients with paragangliomas are, as a group, younger men, more lik ely to have malignant lesions and a more aggressive clinical course. P atients with malignant pheochromocytomas usually present with hyperten sion, are somewhat older, and have less aggressive disease.