Abstract

NK/T cell lymphoma is one of the most unique and rare forms of extranodal non-Hodgkin’s lymphoma, mostly derived from natural killer cell lineages and occasionally cytotoxic T cell lines. Due to the non-specific presentation of NK/T cell lymphoma such as nasal obstruction, nasal discharge, and epistaxis, diagnosis is often an issue and can be misleading. Oronasal fistula following a non-healing ulcer on the soft palate, can be one of the clinical presentations of NK/T cell lymphoma. Here, we are reporting a rare case of NK/T cell lymphoma in a 32-year-old gentleman who presented with an oronasal fistula post tissue biopsy for a non-healing ulcer over the soft palate. The tissue biopsy of the soft palate mass was revealed as NK/T cell lymphoma and was staged as Stage1b after computed tomographic imaging revealed a local tumour invasion without sign of nodal involvement and no metastasis. The patient eventually started with the SMILE Protocol and responded well up to the date. The ulcer healed but the fistula persisted. Primary closure was planned by the oromaxillofacial surgery team after completion of chemotherapy.

Highlights

  • Extranodal NK/T cell lymphoma (NKTCL) nasal type or previously known as lethal midline granuloma, is considered as one of the rarest differential diagnoses to cause an oronasal fistula (ONF)

  • ONF is an internal fistula defined by an abnormal epithelialized track communicating the nasal cavity with the oral cavity in which it may be due to multiple causative factors and can be divided into congenital, infection, iatrogenic, and tumour causes

  • As for this patient, the most common differential diagnosis according to the sign and symptoms is left nasal squamous cell carcinoma (SCC), followed by nasopharyngeal carcinoma (NPC) and olfactory neuroblastoma (ONB)

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Summary

INTRODUCTION

Extranodal NK/T cell lymphoma (NKTCL) nasal type or previously known as lethal midline granuloma, is considered as one of the rarest differential diagnoses to cause an oronasal fistula (ONF). A 32-year-old man with no known medical illness, presented with a two-day history of left epistaxis It was associated with worsening bilateral nasal blockage, rhinorrhea and hyposmia for six months. He complained of a painless ‘hole’ on the left side of his soft palate which started as an ulcer and has subsequently increased in size for the past three months. An intraoral examination revealed an irregular edge fistula over the left side of the soft palate connecting the oral and nasal cavity, measuring 3 x 3 cm (Figure 1). A maxillary obturator prosthesis may be applied

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