Head and Neck Pathology

, Volume 13, Issue 4, pp 722–726 | Cite as

Laryngeal Epstein–Barr Virus-Associated Smooth Muscle Tumor in an Undernourished Child

  • Ciro Dantas SoaresEmail author
  • Roman Carlos
  • Juan Pablo Díaz Molina
  • Thayná Melo de Lima Morais
  • Oslei Paes de Almeida
Case Report


Smooth muscle tumors associated with Epstein–Barr virus infections (EBV–SMT) of laryngeal origin are exceedingly rare and have been reported in few adult patients, but not in children. This reported case describes a lesion found in the larynx of an 8-year-old Guatemalan undernourished girl. Microscopically, the lesion showed a highly cellular mesenchymal spindle cell tumor, containing frequent lymphocytes. The immunohistochemical analysis revealed positivity for α-smooth muscle actin and h-caldesmon. In addition, most of the tumor cells were positive for EBV by in situ hybridization. To the best of the author’s knowledge, this is the first literature-reported case of laryngeal EBV–SMT occurring in an undernourished child.


Epstein–Barr virus infections/pathology Smooth muscle tumor/pathology Pediatric tumors 



This work was supported by São Paulo Research Foundation (FAPESP), Grant Number #2015/25905-1.

Compliance with Ethical Standards

Conflict of interest

The authors declare no potential conflicts of interest related to this case report, to this study’s authorship, and/or to its publication.


  1. 1.
    Grywalska E, Rolinski J. Epstein–Barr virus-associated lymphomas. Semin Oncol. 2015;42(2):291–303. Scholar
  2. 2.
    Jha HC, Pei Y, Robertson ES. Epstein–Barr virus: diseases linked to infection and transformation. Front Microbiol. 2016;7(OCT):1602. Scholar
  3. 3.
    Rumayor Piña A, dos Santos HT, Carlos R, Altemani A, de Almeida OP. Epstein–Barr virus in nasopharyngeal carcinoma of Guatemalan and Brazilian patients. Int J Surg Pathol. 2017;25(4):304–9. Scholar
  4. 4.
    Kazmi SAJ, Aizenberg MR, Harper JL, McComb RD. Multifocal histologically malignant Epstein–Barr virus-associated smooth muscle tumor in a pediatric transplant patient with an indolent course. Int J Surg Pathol. 2014;22(2):186–9. Scholar
  5. 5.
    Jossen J, Chu J, Hotchkiss H, et al. Epstein–Barr virus-associated smooth muscle tumors in children following solid organ transplantation: a review. Pediatr Transpl. 2015;19(2):235–43. Scholar
  6. 6.
    Deyrup AT, Lee VK, Hill CE, et al. 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. 2006;30(1):75–82. Scholar
  7. 7.
    Gan EC, Lau DPC, Chuah KL. Epstein–Barr virus-associated smooth muscle tumour mimicking bilateral vocal process granuloma. J Laryngol Otol. 2008;122(1):100–4. Scholar
  8. 8.
    Huang J, Loh KS, Petersson F. Epstein–Barr virus-associated smooth muscle tumor of the larynx: report of a rare case mimicking leiomyosarcoma. Head Neck Pathol. 2010;4(4):300–4. Scholar
  9. 9.
    Hatton OL, Harris-Arnold A, Schaffert S, Krams SM, Martinez OM. The interplay between Epstein–Barr virus and B lymphocytes: implications for infection, immunity, and disease. Immunol Res. 2014;58(0):268–76. Scholar
  10. 10.
    Hutt-Fletcher LM. Epstein–Barr virus replicating in epithelial cells. Proc Natl Acad Sci USA. 2014;111(46):16242–3. Scholar
  11. 11.
    Ha C, Haller JO, Rollins NK. Smooth muscle tumors in immunocompromised (HIV negative) children. Pediatr Radiol. 1993;23(5):413–4. Scholar
  12. 12.
    Hussein K, Rath B, Ludewig B, Kreipe H, Jonigk D. Clinico-pathological characteristics of different types of immunodeficiency-associated smooth muscle tumours. Eur J Cancer. 2014;50(14):2417–24. Scholar
  13. 13.
    Dekate J, Chetty R. Epstein–Barr virus-associated smooth muscle tumor. Arch Pathol Lab Med. 2016;140(7):718–22. Scholar
  14. 14.
    Arva NC, Schafernak KT. Rare presentations of Epstein–Barr Virus-associated smooth muscle tumor in children. Pediatr Dev Pathol. 2016;19(2):132–8. Scholar
  15. 15.
    Garg I, Baladron Zanetti MJ, Yasir S, Goenka AH, Kendi AT. PET/CT in an 8-year-old girl with Epstein–Barr virus-associated smooth muscle tumor. Clin Nucl Med. 2017;42(10):770–2. Scholar
  16. 16.
    Bryce J, Boschi-Pinto C, Shibuya K, Black RE. WHO estimates of the causes of death in children. Lancet. 2005;365(9465):1147–52. Scholar
  17. 17.
    Rytter MJH, Kolte L, Briend A, Friis H, Christensen VB. The immune system in children with malnutrition—a systematic review. PLoS ONE. 2014;9(8):e105017. Scholar
  18. 18.
    Bourke CD, Berkley JA, Prendergast AJ. Immune dysfunction as a cause and consequence of malnutrition. Trends Immunol. 2016;37(6):386–98. Scholar
  19. 19.
    Völker H-U, Scheich M, Zettl A, Hagen R, Müller-Hermelink HK, Gattenlöhner S. Laryngeal inflammatory myofibroblastic tumors: different clinical appearance and histomorphologic presentation of one entity. Head Neck. 2010;32(11):1573–1578. Scholar
  20. 20.
    Oliva E, Young RH, Amin MB, Clement PB. An immunohistochemical analysis of endometrial stromal and smooth muscle tumors of the uterus: a study of 54 cases emphasizing the importance of using a panel because of overlap in immunoreactivity for individual antibodies. Am J Surg Pathol. 2002;26(4):403–412.CrossRefGoogle Scholar
  21. 21.
    Rogatsch H, Bonatti H, Menet A, Larcher C, Feichtinger H, Dirnhofer S. Epstein–Barr virus-associated multicentric leiomyosarcoma in an adult patient after heart transplantation: case report and review of the literature. Am J Surg Pathol. 2000;24(4):614–621.CrossRefGoogle Scholar
  22. 22.
    Suwansirikul S, Sukpan K, Sittitrai P, Suwiwat S, Khunamornpong S. Epstein–Barr virus-associated smooth muscle tumor of the tonsil. Auris Nasus Larynx. 2012;39(3):329–32. Scholar

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© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Oral Pathology, Piracicaba Dental SchoolUniversity of Campinas (UNICAMP)PiracicabaBrazil
  2. 2.Centro Clínico de Cabeza y Cuello/Hospital Herrera LlerandiCiudad de GuatemalaGuatemala

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