Clinical outcome of 50 patients with malignant abdominal paragangliomas and malignant pheochromocytomas

Abstract

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. 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 extraadrenal, 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 aggresEndocrine-Related Cancer (1998) 5 59-68 sive disease.

Sequelae and survivorship in patients treated with 131I-MIBG therapy

“…Other studies have shown 5-year survival of between 30% and 80% (Sclafani et al , 1990; Vassilopoulou-Sellin, 1998). Sclafani et al (1990) reported a 5-year survival of 36%.…”

“…Sclafani et al (1990) reported a 5-year survival of 36%. Vassilopoulou-Sellin's (1998) study had a very long follow-up of 50 patients and showed a 5-year survival of paragangliomas of 30% and phaeochromocytoma of 80%. The advances in the genetics of CCTs being identified may explain the difference between Sclafani's study and ours, with patients identified much earlier and treated, hence showing a better survival.…”

Liver transplantation for the treatment of nonresectable metastatic duodenal paraganglioma

“…There are no reliable morphologic criteria to differentiate microscopically benign from malignant paraganglioma. Its malignant potential is defined after a metastatic lesion or direct invasion is found in a site with no residual embryonic paraganglionic tissue [3,4].…”

“…The sample size and follow‐up after OLT have been limited in these series [5,6]. The Hannover group published their results in 12 patients who underwent OLT for metastases from NET [4]. They had one operative mortality and two patients developed recurrent disease and died secondary to their malignancy.…”