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

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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.

ABBREVIATIONS CVID = common variable immunodeficiency; EBNA = Epstein-Barr nuclear antigen; EBV = Epstein-Barr virus; EBV-SMT = EBV-associated smooth-muscle tumor; NK = natural killer; VCA = viral capsid antigen.
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Contributor Notes

Correspondence David J. Daniels: Mayo Clinic, Rochester, MN. daniels.david@mayo.edu.INCLUDE WHEN CITING Published online June 15, 2018; DOI: 10.3171/2018.3.PEDS17609.Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.
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References
  • 1

    Arva NCSchafernak KT: Rare presentations of Epstein-Barr virus–associated smooth muscle tumor in children. Pediatr Dev Pathol 19:1321382016

  • 2

    Camargo JFLobo SAHsu APZerbe CSWormser GPHolland SM: MonoMAC syndrome in a patient with a GATA2 mutation: case report and review of the literature. Clin Infect Dis 57:6976992013 (Erratum in Clin Infect Dis 59:323 2014)

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 3

    Cela IShah NBBradly DLoew JLeslie W: An Epstein-Barr virus-associated smooth muscle tumor successfully treated with surgical resection: a case report and literature review. Clin Adv Hematol Oncol 8:4234262010

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 4

    Cohen JIDropulic LHsu APZerbe CSKrogmann TDowdell K: Association of GATA2 deficiency with severe primary Epstein-Barr virus (EBV) infection and EBV-associated cancers. Clin Infect Dis 63:41472016

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 5

    Dekate JChetty R: Epstein-Barr virus–associated smooth muscle tumor. Arch Pathol Lab Med 140:7187222016

  • 6

    Deyrup ATLee VKHill CECheuk WToh HCKesavan S: Epstein-Barr virus-associated smooth muscle tumors are distinctive mesenchymal tumors reflecting multiple infection events: a clinicopathologic and molecular analysis of 29 tumors from 19 patients. Am J Surg Pathol 30:75822006

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 7

    Kumar SSanti MVezina GRosser TChandra RSKeating R: Epstein-Barr virus-associated smooth muscle tumor of the basal ganglia in an HIV+ child: case report and review of the literature. Pediatr Dev Pathol 7:1982032004

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 8

    McClain KLLeach CTJenson HBJoshi VVPollock BHParmley RT: Association of Epstein-Barr virus with leiomyosarcomas in young people with AIDS. N Engl J Med 332:12181995

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 9

    Mierau GWGreffe BSWeeks DA: Primary leiomyosarcoma of brain in an adolescent with common variable immunodeficiency syndrome. Ultrastruct Pathol 21:3013051997

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 10

    Petrilli GLorenzi LParacchini RUbiali ASchumacher RFCabassa P: Epstein-Barr virus-associated adrenal smooth muscle tumors and disseminated diffuse large B-cell lymphoma in a child with common variable immunodeficiency: a case report and review of the literature. Int J Surg Pathol 22:7127212014

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 11

    Prévot SNéris Jde Saint Maur PP: Detection of Epstein Barr virus in an hepatic leiomyomatous neoplasm in an adult human immunodeficiency virus 1-infected patient. Virchows Arch 425:3213251994

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 12

    Purgina BRao UNMMiettinen MPantanowitz L: AIDS-related EBV-associated smooth muscle tumors: a review of 64 published cases. Pathol Res Int 2011:5615482011

    • Search Google Scholar
    • Export Citation
  • 13

    Shaw RKIssekutz ACFraser RSchmit PMorash BMonaco-Shawver L: Bilateral adrenal EBV-associated smooth muscle tumors in a child with a natural killer cell deficiency. Blood 119:400940122012

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 14

    Takei HPowell SRivera A: Concurrent occurrence of primary intracranial Epstein-Barr virus-associated leiomyosarcoma and Hodgkin lymphoma in a young adult. J Neurosurg 119:4995032013

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 15

    Timens WBoes AVos HPoppema S: Tissue distribution of the C3d/EBV-receptor: CD21 monoclonal antibodies reactive with a variety of epithelial cells, medullary thymocytes, and peripheral T-cells. Histochemistry 95:6056111991

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 16

    Tulbah AAl-Dayel FFawaz IRosai J: Epstein-Barr virus-associated leiomyosarcoma of the thyroid in a child with congenital immunodeficiency: a case report. Am J Surg Pathol 23:4734761999

    • Crossref
    • Search Google Scholar
    • Export Citation
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