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Soft Palate Ulcer—Benign or Malignant?

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 71-year-old man with a history of rheumatoid arthritis and type 2 diabetes presented to the otolaryngology team with 6 weeks’ history of pain and ulcers in the pharynx. He also complained of nasal regurgitation. He denied having any systemic symptoms. He took linagliptin, valsartan, and 15 mg of methotrexate weekly. He was a nonsmoker and consumed very little alcohol.

Clinical examination including flexible nasendoscopy revealed multiple shallow ulcers on the posterior wall of the nasopharynx. There was a large defect involving the entire soft palate on the left. There was no obvious mass or any exophytic lesion. The edges of the soft palate defect were smooth and clean (Figure 1). There were no cranial nerve palsies. There was no cervical, axillary, or inguinal lymphadenopathy. Findings of the remaining head and neck examinations were normal.

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D. EBV-positive mucocutaneous ulcer

Histologic findings from the biopsy showed stratified squamous mucosa and respiratory-type mucosa with large areas of surface ulceration. There were dense underlying lymphoid infiltrates composed of lymphocytes, transformed lymphoid cells, and plasma cells. The transformed cells resembled atypical immunoblastic or Hodgkin and Reed-Sternberg–like cells (Figure 2A) dispersed between lymphocytes with no evidence of palisaded lymphocytes around those. These cells showed strong positivity for CD20, Pax5, MUM1, CD30, and CD15 on immunohistochemical staining (Figure 2B). More than 90% of the cells’ nuclear expression also showed EBV positivity on EBV-encoded RNA testing, but the results of B-cell clonality analysis using polymerase chain reaction were negative for any monoclonal B-cell population. No lymphoepithelial lesion, dysplasia, or epithelial malignant neoplasm was seen.

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Article Information

Corresponding Author: Billy L. K. Wong, MBBS, Department of Otolaryngology–Head and Neck Surgery, Broomfield Hospital, CM1 7ET, Essex, United Kingdom (bwonglk@doctors.org.uk).

Published Online: August 12, 2021. doi:10.1001/jamaoto.2021.1927

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Gaulard  P , Swerdlow  SH , Harris  NL , Sundstrom  C , Jaffe  ES . EBV-positive mucocutaneous ulcer. In: Swerdlow  SH , Campo  E , Harris  NL ,  et al, eds.  WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Revised 4th ed. International Agency for Research on Cancer; 2016:307-308.
2.
Ikeda  T , Gion  Y , Nishimura  Y , Nishimura  MF , Yoshino  T , Sato  Y .  Epstein-Barr virus-positive mucocutaneous ulcer: a unique and curious disease entity.   Int J Mol Sci. 2021;22(3):1053. doi:10.3390/ijms22031053PubMedGoogle ScholarCrossref
3.
Ikeda  T , Gion  Y , Yoshino  T , Sato  Y .  A review of EBV-positive mucocutaneous ulcers focusing on clinical and pathological aspects.   J Clin Exp Hematop. 2019;59(2):64-71. doi:10.3960/jslrt.18039PubMedGoogle ScholarCrossref
4.
Dojcinov  SD , Venkataraman  G , Raffeld  M , Pittaluga  S , Jaffe  ES .  EBV positive mucocutaneous ulcer—a study of 26 cases associated with various sources of immunosuppression.   Am J Surg Pathol. 2010;34(3):405-417. doi:10.1097/PAS.0b013e3181cf8622PubMedGoogle ScholarCrossref
5.
Bunn  B , van Heerden  W .  EBV-positive mucocutaneous ulcer of the oral cavity associated with HIV/AIDS.   Oral Surg Oral Med Oral Pathol Oral Radiol. 2015;120(6):725-732. doi:10.1016/j.oooo.2015.06.028PubMedGoogle ScholarCrossref
AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
  • 1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
  • 1.00 CME points in the American Board of Surgery’s (ABS) Continuing Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

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