[Jugulotympanic paraganglioma: therapy concepts under development]

The operative treatment and radiation therapy of jugulotympanic paragangliomas (JTP) are still a matter of controversial discussion. In spite of various improvements during the last 50 years, selecting the appropriate treatment modality (surgery, radiation, or observation) is still a challenge.

During a 16-year period, 44 patients with 45 JTP (10 at level A/B and 35 at level C/D according to Fisch) were seen at the ENT-department in Fulda. Forty-one cases were treated surgically.

Complete resection was possible for level A/B in 100% of the patients (n = 10). Residual tumor was demonstrated for level C in 23% of the patients (5/22) and for level D in 40% (4/10) with a median follow-up time of 69 months. In two cases residual tumor was treated by radiation. Six patients with residual paraganglioma tissue were maintained under observation without any evidence of tumor progression (median follow-up time 39 months). We report one death after the attempt to resect a large residual paraganglioma that had already caused brain stem compression. A sufficient duraplasty could not be achieved following radiation therapy.

Complete tumor resection of jugulotympanic paragangliomas of levels A and B is often possible without injury to the cranial nerves. Extensive tumors present difficulties in complete tumor resection and increase the risk of cranial nerve injuries. Advanced paragangliomas therefore require an individualized therapeutic regime including surgery, radiation therapy, and observation of tumor growth.