National Cancer Data Base report on malignant paragangliomas of the head and neck.
BACKGROUND
Malignant paragangliomas of the head and neck are rare, with previous reports limited to nine or fewer patients. The current review included 59 cases extracted from the National Cancer Data Base that were diagnosed between 1985-1996.


METHODS
The primary criterion for inclusion in the current study was verified metastatic spread from a paraganglioma of the head and neck. Patterns of presentation and treatment as well as clinically relevant associations were demonstrated in contingency tables. Relative survival was used for analysis of outcome.


RESULTS
The average patient age at presentation was 44 years, and gender distribution was equivalent. Metastases were confined to regional lymph nodes in the majority of cases (68.6%), with carotid body tumors found to have an even higher rate of regional confinement (93.8%). Surgery was the most common treatment (76.3%). The use of adjuvant irradiation for regionally confined disease increased across time, from 27% (1985-1990) to 46% (1991-1996). The 5-year relative survival rate was 59.5% (76.8% for regionally confined carcinoma and 11.8% for distant metastasis). Among patients who were followed until death, those treated with adjuvant irradiation had a longer median survival (45 months) compared with those patients who were treated with surgery alone (12 months).


CONCLUSIONS
Malignant paraganglioma represents metastatic spread of a tumor type that, when restricted to the site of origin, is considered benign. Metastases from malignant paragangliomas of the head and neck usually are regionally confined. The primary management of a recognized malignancy should be directed toward complete surgical removal of the primary tumor and regional lymph nodes. Postoperative irradiation may be beneficial in slowing the progression of residual disease.
Lee
John H
JH
Department of Otolaryngology-Head and Neck Surgery, University of Iowa Hospitals and Clinics, Iowa City, Iowa 52242, USA.
Barich
Frank
F
Karnell
Lucy Hynds
LH
Robinson
Robert A
RA
Zhen
Weining K
WK
Gantz
Bruce J
BJ
Hoffman
Henry T
HT
D000328:Adult; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D016208:Databases, Factual; D005260:Female; D006258:Head and Neck Neoplasms / Q000453:epidemiology / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008297:Male; D008875:Middle Aged; D009362:Neoplasm Metastasis; D010235:Paraganglioma / Q000453:epidemiology / Q000473:pathology* / Q000601:surgery; D011379:Prognosis; D018714:Radiotherapy, Adjuvant; D012737:Sex Factors; D016019:Survival Analysis