Intrathoracic paravertebral malignant paraganglioma.

This article reviews the clinical and pathologic features of intrathoracic paravertebral paragangliomas. Including the present case, there have been 31 tumors reported in the English literature; the average age of patients was 29 years, with a sex distribution of 20 men and 11 women. Fifteen patients (48%) had symptoms related to excess secretion of catecholamines; the remaining 16 tumors were clinically nonfunctional. Seven patients (22%) had multiple paragangliomas. Complete surgical resection was attempted in 25 patients, 13 were alive with no evidence of tumor an average of 2.2 years later. Tumor was locally invasive in five patients, with involvement of the vertebral canal and symptoms of spinal cord compression. Malignant behavior with distant metastases was observed in two patients. As shown by the present case, the Grimelius stain is a useful diagnostic technique for demonstrating cytoplasmic argyrophilia of neoplastic chief cells. Electron microscopy demonstrated neurosecretory granules (average core diameter, 100 nm). "Light" and "dark" chief cell types were inconspicuous. Due to important clinical and pathologic differences, paravertebral paragangliomas should be distinguished from similar tumors occurring in the anterosuperior mediastinum (aorticopulmonary paragangliomas).