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

Desmond A. Brown, Nicholas L. Deep, Colin L. Driscoll, Michael J. Link, Mark E. Jentoft, David J. Daniels

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish (US)
Pages (from-to)283-287
Number of pages5
JournalJournal of Neurosurgery: Pediatrics
Volume22
Issue number3
DOIs
StatePublished - Sep 2018

Keywords

  • Epstein-Barr virus
  • Immunodeficiency
  • Oncology
  • Smooth-muscle tumor

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

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