Extra-adrenal paragangliomas of the retroperitoneum

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Abstract
The clinical and pathologic features of 12 extraadrenal paragangliomas of the retroperitoneum are presented. The patients (8 men, 4 women) had an average age of 43 yr at the time of diagnosis; 3 had symptoms related to excess catecholamine secretion. Complete surgical resection was attempted in each case but was possible for only 4 patients. Five tumors were malignant; 4 patients died from regional and/or distant metastases 17 mo.-10 yr after surgery. One patient was alive with metastatic paraganglioma 25 yr after initial treatment. Each patient with metastatic or locally recurrent paragangloma had incomplete resection of the primary tumor. An important microscopic feature of each paraganglioma was the presence of cytoplasmic argyrophilia. Three of the 5 metastasizing paragangliomas showed readily identifiable mitoses or evidence of vascular invasion; the 2 other cases and all of the benign tumors lacked these features. In some cases histology may prove helpful in evaluating the malignant potential of these tumors.