Tumors: Clinical Behavior

Abstract

Nonchromaffin paragangliomas have been reported arising in all age groups from the neonatal period to advanced age, with a peak incidence between the ages of 30 and 50 years. The average age also varies with the site of origin. Tumors were reported arising at the carotid bifurcation (Wosnessenski, 1923; Helpap and Helpap, 1966) and at ectopic carotid (Akkary, 1964) and temporal (Helpap and Helpap, 1966; Busby and Hepp, 1974) sites in infants several months of age. Elders and DeBoer (1961) also described aortic body tumors in a neonate and in a 6-month-old infant. Review of approximately 2000 reported cases reveals about 30 cases occurring in persons under 20 years of age. On review of the literature between 1919 and 1969, Helpap and Grouls (1971) found 36 cases of nonchromaffin paraganglioma in which the first symptom appeared before the age of 15 years. However, their series included 4 “paraganglioma femorale” lesions, which probably are alveolar soft-part sarcomas, and a carotid body tumor (Balboni and Pandolfini, 1955) and a glomus jugulare tumor (Bilancioni, 1919), the nature of both being controversial.