Extranodal NK/T-cell lymphoma, nasal type - Oral Mucosal Lesion
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Synopsis

The disease is exceedingly rare in the United States and Europe but more common in Central / South America and Asia. Adults are most frequently affected, but cases in children have been reported.
EBV exposure is a key risk factor, and detection of the virus in lymphoma cells is required for diagnosis. Genetic aberrations such as deletion of chromosome 6q and possible abnormalities in the Janus kinase (JAK)-signal transducer and activator of transcription (STAT) pathway have been implicated.
Treatment is difficult and includes chemotherapy and/or radiation therapy.
Related topic: Non-Hodgkin lymphoma
Codes
ICD10CM:M31.2 – Lethal midline granuloma
SNOMEDCT:
414166008 – Extranodal natural killer/T-cell lymphoma, nasal type
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Differential Diagnosis & Pitfalls
Cutaneous findings:- Lymphomatoid papulosis
- Primary cutaneous anaplastic large cell lymphoma
- Leukemia cutis
- Kaposi sarcoma
- Plasmacytoid dendritic cell neoplasm
- Syphilis
- Sarcoidosis
- Pseudolymphoma
- Leprosy
- Invasive fungal disease such as mucormycosis
- Mucocutaneous leishmaniasis
- Rhinoscleroma
- Granulomatosis with polyangiitis
- Yaws
- Nasal polyp
- Angiosarcoma
- Rhinosporidiosis
- Paracoccidioidomycosis
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Last Reviewed:02/13/2019
Last Updated:10/28/2021
Last Updated:10/28/2021
Extranodal NK/T-cell lymphoma, nasal type - Oral Mucosal Lesion
See also in: Overview