ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferencesView all Images (6)
Extranodal NK/T-cell lymphoma, nasal type - Oral Mucosal Lesion
See also in: Overview
Print
Other Resources UpToDate PubMed

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

See also in: Overview
Print Images (6)
Contributors: Gaurav Singh MD, MPH, Susan Burgin MD
Other Resources UpToDate PubMed

Alerts and Notices

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 comprised of NK cells or, less commonly, cytotoxic T cells. The nasopharynx is most commonly involved (80% of cases), followed by the skin. Lesions are comprised 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

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Cutaneous findings:
    Nasal septal disease:

    Best Tests

    Subscription Required

    Management Pearls

    Subscription Required

    Therapy

    Subscription Required

    References

    Subscription Required

    Last Reviewed:02/14/2019
    Last Updated:09/11/2019
    Copyright © 2021 VisualDx®. All rights reserved.
    Extranodal NK/T-cell lymphoma, nasal type - Oral Mucosal Lesion
    See also in: Overview
    Print   View all Images (6)
    (with subscription)
    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

    +4 More images of Extranodal NK/T-cell lymphoma, nasal type with subscription

    Copyright © 2021 VisualDx®. All rights reserved.