Erythrocytosis complicated by multiple paraganglioma.
We report the case of a 22-year-old woman with onset of erythrocytosis at the age of 9 years. Endocrinological and radiological examinations revealed an elevated catecholamine level and the presence of multiple abdominal tumors. After the removal of the tumors, the catecholamine level normalized, whereas erythropoietin remained at the same level and erythrocytosis persisted. The tumor lysate contained considerable amounts of catecholamine but not erythropoietin. Moreover, no erythropoietin mRNA was detected in the tumor by in situ hybridization. These data suggest that this paraganglioma did not produce erythropoietin. A review of the literature showed the existence of patients with early-onset erythrocytosis complicated with paraganglioma, whose erythrocytosis was not relieved even after the resection of paraganglioma.
Second Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Japan.
N
N
Terada
K
K
Kitano
S
S
Tokunaga
S
S
Yamada
H
H
Saito
S
S
Ohno
S
S
Furuta
D000008:Abdominal Neoplasms / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D000328:Adult; D002908:Chronic Disease; D000242:Cyclic AMP / Q000032:analysis; D004837:Epinephrine / Q000032:analysis; D004921:Erythropoietin / Q000032:analysis; D005260:Female; D006801:Humans; D009638:Norepinephrine / Q000032:analysis; D010235:Paraganglioma / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D011086:Polycythemia / Q000150:complications* / Q000175:diagnosis / Q000628:therapy
D004921:Erythropoietin; D000242:Cyclic AMP; D009638:Norepinephrine; D004837:Epinephrine