Paragangliomatosis with numerous catecholamine-producing tumors

Catecholamine-producing tumors may be found wherever paraganglion tissue exists, and they may be associated with a variety of conditions (multiple endocrine neoplasia types 1, 2a and 2b). The patient described in this report had 21 paragangliomas removed between the ages of 13 and 17 years, and she has evidence of additional tumors. This is the greatest number of paragangliomas reported, and the tumors involved numerous sites. In addition, the patient has multiple congenital anomalies that have not been reported previously in association with paraganglioma. Unifying theories of pathogenesis and several features in the management of this patient are discussed.