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

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Contributors: Gaurav Singh MD, MPH, Susan Burgin MD
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Synopsis

Extranodal NK/T-cell lymphoma, nasal type : Oral ulcers, Hard palate, Nasal septum ulceration, Nose, Rhinorrhea, Epistaxis, Serosanguineous nasal discharge
Nasal type extranodal natural killer (NK)/T-cell lymphoma, previously referred to as lethal midline granuloma, is a lymphoma characterized by Epstein-Barr virus (EBV) positivity and NK cells or, less commonly, cytotoxic T cells. The nasopharynx is most commonly involved (80% of cases), followed by the skin. Lesions are composed of firm papules or nodules that rapidly expand, ulcerate, and destroy surrounding tissue. Skin disease may be primary or secondary. Multiple sites can be involved, including the gastrointestinal (GI) tract, testes, salivary gland, uterus, and muscle. Hemophagocytic syndrome with pancytopenia can further aggravate the clinical course.

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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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Cutaneous findings:
    Nasal septal disease:

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    Therapy

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    References

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    Last Reviewed:02/13/2019
    Last Updated:10/28/2021
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    Extranodal NK/T-cell lymphoma, nasal type - Oral Mucosal Lesion
    See also in: Overview
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    Extranodal NK/T-cell lymphoma, nasal type : Oral ulcers, Hard palate, Nasal septum ulceration, Nose, Rhinorrhea, Epistaxis, Serosanguineous nasal discharge
    Clinical image of Extranodal NK/T-cell lymphoma, nasal type
    Ulceration and crusting on the palate.

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