Unveiling the Unexpected: Tongue Bleeding As the First Sign of Extranodal NK-T Cell Lymphoma

Abstract: Natural Killer/T-cell lymphoma (NKTCL) is a rare and aggressive form of non-Hodgkin lymphoma involving natural killer (NK) cells or T lymphocytes, commonly affecting the nasal cavity and upper aerodigestive tract. However, it can also present in extranasal locations like the skin, lungs, and gastrointestinal tract. Here, we describe a rare case of primary extranodal NK/T-cell lymphoma, nasal type (ENKTCL), with an unusual extranasal presentation in the tongue. This led to significant buccal bleeding and hemorrhagic shock due to a lingual artery pseudoaneurysm, an unprecedented complication of ENKTCL.

Case Presentation: A 25-year-old male from Guatemala, residing in the United States, presented with a week-long history of tongue pain, swelling, and a muffled voice following an accidental tongue bite. Examination revealed a large, foul-smelling puncture wound on the tongue with fibrinous exudate, and initial labs showed anemia with a hemoglobin level of 8.8 g/dl. Flexible laryngoscopy revealed erythematous nasal mucosa without masses, and a CT scan of the head and neck showed submandibular lymphadenopathy. Despite treatment with antibiotics and steroids, the tongue lesion did not improve. A biopsy revealed extranodal NK/T-cell lymphoma, nasal type, with necrosis, confirmed by immunostaining positive for CD2, CD7, CD25, TIA-1, granzyme B, and other markers. Before planned radiation therapy, the patient returned to the emergency department with severe tongue bleeding, initially managed with silver nitrate. However, a subsequent episode of profuse bleeding caused hemorrhagic shock. CT angiography identified a lingual artery pseudoaneurysm, necessitating emergent embolization to achieve hemostasis. Further biopsies confirmed NK/T-cell lymphoma with extensive necrosis, and a liver biopsy revealed chronic hepatitis with mild lobular activity and portal fibrosis. The patient was started on chemotherapy with etoposide, steroid, high dose ara-C and cisplatin (ESHAP), however developed further complications and passed away within the following two months.

Conclusion: This case highlights a rare and aggressive presentation of ENKTCL, nasal type, manifesting unusually in the tongue and complicated by a lingual artery pseudoaneurysm. Prompt recognition and management of such atypical presentations are crucial for improving patient outcomes. This case underscores the importance of considering ENKTCL in the differential diagnosis of unusual oral lesions, especially when significant bleeding complications arise.

No relevant conflicts of interest to declare.