EBV-Associated Non-keratinizing Nasopharyngeal Carcinoma with Prominent Spindled Cell and Whorling Patterns: A Previously Unreported Histological Variant in a Patient Presenting with Dermatomyositis

Abstract

A case of non-keratinizing, EBV-positive (chromogenic EBER-in situ hybridization), carcinoma with a hitherto undescribed nodular whorling architecture is presented. The patient is a 55 year old male with 2 months history of dermatomyositis who was diagnosed with T1N0M0 non-keratinizing nasopharyngeal carcinoma. The patient received radiotherapy with complete response. The tumor cells predominantly displayed spindle cell morphology, strongly expressed low-molecular weight cytokeratins (AE1-3), p63 and p40. There was no evidence of recurrence or disease progression on follow-up after 16 months which included post treatment biopsy, MRI and PET-CT scans.

This is a preview of subscription content, log in to check access.

Fig. 1
Fig. 2

References

  1. 1.

    Petersson F. Nasopharyngeal carcinoma: a review. Semin Diagn Pathol. 2015;32(1):54–73. https://doi.org/10.1053/j.semdp.2015.02.021.

    Article  PubMed  Google Scholar 

  2. 2.

    Petersson F, Vijayadwaja D, Loh KS, Tan KB. Reticular and myxoid non-keratinizing nasopharyngeal carcinoma: an unusual case mimicking a salivary gland carcinoma. Head Neck Pathol. 2014;8(3):364–8. https://doi.org/10.1007/s12105-013-0512-6.

    Article  PubMed  Google Scholar 

  3. 3.

    Petersson F. Non-keratinizing nasopharyngeal carcinoma with adenomatous differentiation. Head Neck Pathol. 2018. https://doi.org/10.1007/s12105-018-0991-6.

    Article  PubMed  PubMed Central  Google Scholar 

  4. 4.

    Petersson F. al. WHO classifiction of head and neck tumours. Lyon: IARC Press; 2017.

    Google Scholar 

  5. 5.

    Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts). N Engl J Med. 1975;292(8):403–7. https://doi.org/10.1056/NEJM197502202920807.

    CAS  Article  PubMed  Google Scholar 

  6. 6.

    Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med. 1975;292(7):344–7. https://doi.org/10.1056/NEJM197502132920706.

    CAS  Article  PubMed  Google Scholar 

  7. 7.

    Lundberg IE, Tjarnlund A, Bottai M, Werth VP, Pilkington C, Visser M, et al. 2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. Ann Rheum Dis. 2017;76(12):1955–64. https://doi.org/10.1136/annrheumdis-2017-211468.

    Article  PubMed  PubMed Central  Google Scholar 

  8. 8.

    Di Rollo D, Abeni D, Tracanna M, Capo A, Amerio P. Cancer risk in dermatomyositis: a systematic review of the literature. G Ital Dermatol Venereol. 2014;149(5):525–37.

    PubMed  Google Scholar 

  9. 9.

    Hill CL, Zhang Y, Sigurgeirsson B, Pukkala E, Mellemkjaer L, Airio A, et al. Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study. Lancet. 2001;357(9250):96–100. https://doi.org/10.1016/S0140-6736(00)03540-6.

    CAS  Article  PubMed  Google Scholar 

  10. 10.

    Yosipovitch G, Tan A, LoSicco K, Manabat CG, Kannagra A, Carroll C, et al. A comparative study of clinical characteristics, work-up, treatment, and association to malignancy in dermatomyositis between two tertiary skin centers in the USA and Singapore. Int J Dermatol. 2013;52(7):813–9. https://doi.org/10.1111/j.1365-4632.2011.05449.x.

    Article  PubMed  Google Scholar 

  11. 11.

    Ang P, Sugeng MW, Chua SH. Classical and amyopathic dermatomyositis seen at the National Skin Centre of Singapore: a 3-year retrospective review of their clinical characteristics and association with malignancy. Ann Acad Med Singapore. 2000;29(2):219–23.

    CAS  PubMed  Google Scholar 

  12. 12.

    Teoh JW, Yunus RM, Hassan F, Ghazali N, Abidin ZA. Nasopharyngeal carcinoma in dermatomyositis patients: a 10-year retrospective review in Hospital Selayang, Malaysia. Rep Pract Oncol Radiother. 2014;19(5):332–6. https://doi.org/10.1016/j.rpor.2014.02.005.

    CAS  Article  PubMed  PubMed Central  Google Scholar 

  13. 13.

    Tang MM, Thevarajah S. Paraneoplastic Dermatomyositis. A 12-year retrospective review in the department of dermatology hospital Kuala Lumpur. Med J Malaysia. 2010;65(2):138–42.

    CAS  PubMed  Google Scholar 

  14. 14.

    Wong KO. Dermatomyositis: a clinical investigation of twenty-three cases in Hong Kong. Br J Dermatol. 1969;81(7):544–7.

    CAS  Article  Google Scholar 

  15. 15.

    Hu WH. [Nasopharyngeal carcinoma (NPC) with dermatomyositis–an analysis of 30 cases]. Zhonghua Zhong Liu Za Zhi. 1986;8(2):133–5.

    CAS  PubMed  Google Scholar 

  16. 16.

    Peng JC, Sheen TS, Hsu MM. Nasopharyngeal carcinoma with dermatomyositis. Analysis of 12 cases. Arch Otolaryngol Head Neck Surg. 1995;121(11):1298–301.

    CAS  Article  Google Scholar 

  17. 17.

    Ee HL, Ng PP, Tan SH. Exacerbation of amyopathic dermatomyositis in Orientals: a high alert for nasopharyngeal carcinoma. Australas J Dermatol. 2004;45(1):77–8.

    Article  Google Scholar 

  18. 18.

    Loh KS, Petersson F. Nonexophytic nasopharyngeal carcinoma: high frequency of advanced lymph node and distant metastasis. Otolaryngol Head Neck Surg. 2011;145(4):594–8. https://doi.org/10.1177/0194599811411141.

    Article  PubMed  Google Scholar 

  19. 19.

    Chang YS, Tyan YS, Liu ST, Tsai MS, Pao CC. Detection of Epstein-Barr virus DNA sequences in nasopharyngeal carcinoma cells by enzymatic DNA amplification. J Clin Microbiol. 1990;28(11):2398–402.

    CAS  Article  Google Scholar 

  20. 20.

    Mutirangura A, Pornthanakasem W, Theamboonlers A, Sriuranpong V, Lertsanguansinchi P, Yenrudi S, et al. Epstein-Barr viral DNA in serum of patients with nasopharyngeal carcinoma. Clin Cancer Res. 1998;4(3):665–9.

    CAS  PubMed  Google Scholar 

Download references

Author information

Affiliations

Authors

Corresponding author

Correspondence to Fredrik Petersson.

Ethics declarations

Conflict of interest

The author has no conflict of interest. No funding was received.

Ethical Approval

It is my institution’s policy not to require formal ethical approval for reports on up to two patients.

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Petersson, F. EBV-Associated Non-keratinizing Nasopharyngeal Carcinoma with Prominent Spindled Cell and Whorling Patterns: A Previously Unreported Histological Variant in a Patient Presenting with Dermatomyositis. Head and Neck Pathol 14, 203–207 (2020). https://doi.org/10.1007/s12105-019-01019-z

Download citation

Keywords

  • Nasopharynx
  • Carcinoma
  • Whorling
  • Epstein–Barr virus