MPNST after Radiosurgery: A Report and Review of the Literature

Objective: To report an unusual case of malignant degeneration in a ganglioneuroma of the jugular foramen post stereotactic radiosurgery as well as to systematically review the pertinent literature involving radiosurgery associated malignancies. Background: Ganglioneuromas are uncommon lesions of the peripheral nervous system and infrequently reported in the head and neck region. There have been no previously described cases of ganglioneuromas occurring in the jugular foramen. Malignant degeneration of these lesions is rarely described, especially in the context of radiosurgery. Radiosurgery associated malignancies overall are exceptionally rare and only a limited number of cases have been reported. Results/Conclusions: We describe a case of a ganglioneuroma occurring in the jugular foramen and mimicking the appearance of a jugular foramen schwannoma. This lesion underwent stereotactic radiosurgery and developed a delayed degeneration into a malignant nerve sheath tumor. The occurrence of ganglioneuroma in this region has not been previously described and presumed malignant degeneration is even rarer. This case highlights a previously understated risk associated with stereotactic radiation treatments. To date, 33 previous cases of stereotactic radiosurgery associated malignancies have been reported. We review the pertinent details of these cases. We caution that as follow up periods and awareness increases, reports such as this may become increasingly common.