caa a22 4500 477035116 CHVBK 20180405111303.0 cr unu---uuuuu 170330e19960701xx s 000 0 eng 10.1007/s002689900139 doi (NATIONALLICENCE)springer-10.1007/s002689900139 Clinical Spectrum and Outcome of Functional Extraadrenal Paraganglioma [Elektronische Daten] [Diarmuid S. O'Riordain, Jr., William F. Young, Clive S. Grant, J. Aidan Carney, Jon A. van Heerden] Abstract. 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. 1996 by the Société Internationale de Chir, ugie O'Riordain Diarmuid S. Department of Surgery, Mayo Clinic and Mayo Foundation, 201 Second Street SW, Rochester, Minnesota 55905, U.S.A., US aut Young Jr., William F. Department of Internal Medicine, Mayo Clinic and Mayo Foundation, 201 Second Street SW, Rochester, Minnesota 55905, U.S.A., US aut Grant Clive S. Department of Surgery, Mayo Clinic and Mayo Foundation, 201 Second Street SW, Rochester, Minnesota 55905, U.S.A., US aut Carney J. Aidan Department of Pathology, Mayo Clinic and Mayo Foundation, 201 Second Street SW, Rochester, Minnesota 55905, U.S.A., US aut van Heerden Jon A. Department of Surgery, Mayo Clinic and Mayo Foundation, 201 Second Street SW, Rochester, Minnesota 55905, U.S.A., US aut https://doi.org/10.1007/s002689900139 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s002689900139 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- O'Riordain Diarmuid S. Department of Surgery, Mayo Clinic and Mayo Foundation, 201 Second Street SW, Rochester, Minnesota 55905, U.S.A., US aut NATIONALLICENCE 700 1- Young Jr., William F. Department of Internal Medicine, Mayo Clinic and Mayo Foundation, 201 Second Street SW, Rochester, Minnesota 55905, U.S.A., US aut NATIONALLICENCE 700 1- Grant Clive S. Department of Surgery, Mayo Clinic and Mayo Foundation, 201 Second Street SW, Rochester, Minnesota 55905, U.S.A., US aut NATIONALLICENCE 700 1- Carney J. Aidan Department of Pathology, Mayo Clinic and Mayo Foundation, 201 Second Street SW, Rochester, Minnesota 55905, U.S.A., US aut NATIONALLICENCE 700 1- van Heerden Jon A. Department of Surgery, Mayo Clinic and Mayo Foundation, 201 Second Street SW, Rochester, Minnesota 55905, U.S.A., US aut Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer