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Synchronous Epstein-Barr virus–associated skull base and adrenal smooth-muscle tumors in an 8-year-old girl with recent Epstein-Barr virus infection

Authors :
Michael J. Link
Colin L. W. Driscoll
Mark E. Jentoft
Desmond A. Brown
Nicholas L. Deep
David J. Daniels
Source :
Journal of Neurosurgery: Pediatrics. 22:283-287
Publication Year :
2018
Publisher :
Journal of Neurosurgery Publishing Group (JNSPG), 2018.

Abstract

Epstein-Barr virus–associated smooth-muscle tumors are rare tumors seen in immunocompromised patients. Most cases occur in the context of AIDS and organ transplantation, and very rarely in the setting of congenital immunodeficiency, with only 5 case reports of the latter published so far in the literature. The authors report the case of a previously healthy 8-year-old girl with headaches and precocious puberty who was found to have a large skull base lesion. There was a synchronous left adrenal lesion. She underwent resection of the skull base lesion and a left adrenalectomy. Thorough evaluation for immunodeficiency was negative for a known congenital immunodeficiency syndrome. She had a short course of intravenous immunoglobulin and has had no recurrence of disease or new lesions in the 17 months since presentation. Continued surveillance for the development of opportunistic infections and new or recurrent lesions is warranted in this case. Repeat surgery for surgically accessible tumors or chemoradiation would be recommended for any additional lesions.

Details

ISSN :
19330715 and 19330707
Volume :
22
Database :
OpenAIRE
Journal :
Journal of Neurosurgery: Pediatrics
Accession number :
edsair.doi.dedup.....f943e239a4b1b204faa6ae1f944d2a3c