Extranodal NK/T cell lymphoma, nasal type, with retrobulbar extraconal phlegmon and naso-oral fistula

BMJ Case Rep. 2019 Mar 9;12(3):e227160. doi: 10.1136/bcr-2018-227160.

Abstract

Extranodal natural killer (NK-)/T cell lymphoma, nasal type (ENKL), 1 is a rare disease that often mimics rheumatological and infectious conditions and can therefore be difficult to diagnose. The authors present a case of a 55-year-old Vietnamese woman who was misdiagnosed with severe atrophic rhinitis and chronic sinus osteitis. Over a period of 8 months from initial referral, she underwent multiple biopsies and was treated with various antimicrobial regimens until the histopathological diagnosis of ENKL was finally made. Her presentation was complicated by bacterial dacrocystitis, preseptal cellulitis and a retrobulbar extraconal phlegmon requiring surgical drainage. She also subsequently developed a naso-oral fistula on treatment. This case highlights the importance of repeated biopsies, in particular from non-necrotic regions of the sinonasal tract when a patient does not respond to therapy and clinical suspicion of neoplastic pathology remains. This is the first case of ENKL to describe significant orbital complication.

Keywords: ear, nose and throat/otolaryngology; malignant disease and immunosuppression; oncology.

Publication types

  • Case Reports

MeSH terms

  • Asian People / ethnology
  • Cellulitis / diagnosis
  • Cellulitis / etiology*
  • Cellulitis / surgery
  • Chemoradiotherapy
  • Dacryocystitis / diagnosis
  • Dacryocystitis / etiology
  • Diagnosis, Differential
  • Diagnostic Errors
  • Female
  • Humans
  • Lymphoma, Extranodal NK-T-Cell / complications*
  • Lymphoma, Extranodal NK-T-Cell / drug therapy
  • Lymphoma, Extranodal NK-T-Cell / pathology*
  • Lymphoma, Extranodal NK-T-Cell / radiotherapy
  • Middle Aged
  • Nose / diagnostic imaging*
  • Nose / pathology
  • Oral Fistula / chemically induced
  • Oral Fistula / complications*
  • Positron-Emission Tomography
  • Rare Diseases
  • Treatment Outcome