Bone metastases from a paraganglioma - A review of five cases

Paragangliomas are infrequent, usually benign tumors developed from neuroec toderm cells. The neck is the most common location, although some cases ari se within the abdominal cavity, usually in the retroperitoneal space. We re port five cases with bone metastases, In three patients, convincing evidenc e was obtained that the primary was in the retroperitoneal space. Clinical manifestations of metastatic bone disease occurred up to 17 years after the diagnosis of paraganglioma. Useful data were obtained from plain radiograp hs, magnetic resonance imaging, serum and urine catecholamine assays, and a bove all meta(123)iodobenzylguanidine scintigraphy, Histologic and immunohi stochemical studies of the lesion yielded the definite diagnosis. Surgery a nd radiation therapy are the two mainstays of therapy. Although rare, metas tatic forms of paraganglioma should be borne in mind, This diagnosis should be entertained in patients with bone lesions and recent-onset arterial hyp ertension, irrespective of whether they report a history of surgery for a t umor, and even if this tumor was removed many years earlier and labeled ben ign.