Clinical spectrum and outcome of functional extraadrenal paraganglioma.
Functioning extraadrenal paragangliomas represent more than 10% of all pheochromocytomas, occur at diverse anatomic locations, and are said to have a higher malignancy rate than intraadrenal pheochromocytomas. Sixty-six patients had surgery for catecholamine-producing paragangliomas between 1952 and 1992. Median follow-up was 8.8 years. Median age was 40 years (11-67 years); the male/female ratio was 29:37. Familial disease occurred in 9 patients (13.6%), and 10 patients (15.2%) also developed adrenal pheochromocytoma. Solitary paragangliomas occurred in 52 patients: 46 abdominal, 4 thoracic, and 2 head and neck. Fifty-three tumors developed in 14 patients with multiple paragangliomas: 38 abdominal and 15 thoracic. Of 28 patients with solitary tumors undergoing localization studies over the past 10 years accurate localization was achieved in 27. There was one operative death; 15 patients had persistent disease; and 50 were cured postoperatively. Of those cured, nine developed recurrence, disease-free survival being 86%, 80%, and 80% at 5, 10, and 20 years. Metastatic disease was found in 14 patients (21%), 7 of whom have died. An additional 10 patients (15%) had locally invasive disease, of whom 4 have died. Cause-specific survivals at 5, 10, and 20 years were 90%, 83%, and 72%. Risk factors for death from pheochromocytoma were tumor size > 5 cm (p = 0.0002), metastatic disease (p = 0.001), and tumor invasion (p = 0.0023). Cause-specific survival for patients with tumors > 5 cm was 59% at 15 years compared to 100% among patients with tumors </= 5 cm (p = 0.0003). Functional paragangliomas are frequently malignant and are associated with a high incidence of persistent or recurrent disease. Tumor size > 5 cm and the occurrence of invasive or metastatic disease are strong predictors of outcome. Most tumors are abdominal, and imaging is highly successful for localization.
O'Riordain
D S
DS
Department of Surgery, Mayo Clinic and Mayo Foundation, 201 Second Street SW, Rochester, Minnesota 55905, USA.
Young
W F
WF
Grant
C S
CS
Carney
J A
JA
van Heerden
J A
JA
D000008:Abdominal Neoplasms / Q000601:surgery; D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000601:surgery; D000328:Adult; D000368:Aged; D002395:Catecholamines / Q000378:metabolism; D002648:Child; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D005544:Forecasting; D006258:Head and Neck Neoplasms / Q000601:surgery; D006801:Humans; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness; D009364:Neoplasm Recurrence, Local / Q000473:pathology; D010235:Paraganglioma / Q000235:genetics / Q000378:metabolism / Q000473:pathology / Q000556:secondary / Q000601:surgery*; D010673:Pheochromocytoma / Q000601:surgery; D012074:Remission Induction; D012307:Risk Factors; D015996:Survival Rate; D013899:Thoracic Neoplasms / Q000601:surgery; D016896:Treatment Outcome
D002395:Catecholamines