History

General manifestations of natural killer (NK)/T-cell lymphoma (NKTCL) include the following:

Weight loss
Fever
Fatigue
Night sweats
Bone pain

Fewer than 20% of patients have B symptoms on presentation.

Head and neck manifestations include the following:

Facial pain and swelling
Diplopia
Decreased visual acuity
Orbital swelling
Nasal obstruction
Nasal septal perforation
Purulent nasal discharge
Refractory sinusitis
Epistaxis
Impaired hearing
Palatal ulceration
Odynophagia
Dysphagia
Velopharyngeal incompetence (VPI)
Trismus
Halitosis
Otalgia
Facial swelling
Dyspnea
Hoarseness

Pulmonary manifestations include the following:

Cough
Breathing restriction

Gastrointestinal (GI) manifestations include the following:

Pain
Abdominal fullness
Early satiety
Perforation
Hemorrhage

Testicular pain may also be reported, as may nonhealing sores on the skin.

Physical Examination

General findings on physical examination include the following:

Ulcerations involving the skin
Pallor

Head and neck findings include the following:

Cranial neuropathies
Visual impairment
Orbital mass
Proptosis
Facial swelling and erythema (midline facial features)
Serous otitis media
Ulcerations of the palate, tonsil, nasopharynx, and larynx
Nasal mass
Neck mass

Pulmonary findings include the following:

Cough
Restricted breathing

GI findings include the following:

Hepatosplenomegaly
Abdominal mass

A testicular mass may be detected

Differential Diagnoses

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Ooi GC, Chim CS, Liang R, Tsang KW, Kwong YL. Nasal T-cell/natural killer cell lymphoma: CT and MR imaging features of a new clinicopathologic entity. AJR Am J Roentgenol. 2000 Apr. 174(4):1141-5. [QxMD MEDLINE Link].
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Park S, Ko YH. Epstein-Barr virus-associated T/natural killer-cell lymphoproliferative disorders. J Dermatol. 2014 Jan. 41(1):29-39. [QxMD MEDLINE Link].
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Swerdlow E, Campo E, Harris NE, et al. IARC, Lyon. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissue. 4th. 2008.
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Zhang H, Li ZL, Ye SB, et al. Myeloid-derived suppressor cells inhibit T cell proliferation in human extranodal NK/T cell lymphoma: a novel prognostic indicator. Cancer Immunol Immunother. 2015 Oct 23. [QxMD MEDLINE Link]. [Full Text].
Chen Z, Guan P, Shan T, et al. CD30 expression and survival in extranodal NK/T-cell lymphoma: a systematic review and meta-analysis. Oncotarget. 2018 Mar 27. 9 (23):16547-56. [QxMD MEDLINE Link]. [Full Text].
Wang ZY, Liu QF, Wang H, Jin J, Wang WH, Wang SL, et al. Clinical implications of plasma Epstein-Barr virus DNA in early-stage extranodal nasal-type NK/T-cell lymphoma patients receiving primary radiotherapy. Blood. 2012 Jul 23. [QxMD MEDLINE Link].
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[Guideline] Vega F, Amador C, Chadburn A, et al. American Registry of Pathology Expert Opinions: Recommendations for the diagnostic workup of mature T cell neoplasms. Ann Diagn Pathol. 2020 Dec. 49:151623. [QxMD MEDLINE Link]. [Full Text].
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Chim CS, Ma SY, Au WY, et al. Primary nasal natural killer cell lymphoma: long-term treatment outcome and relationship with the International Prognostic Index. Blood. 2004 Jan 1. 103(1):216-21. [QxMD MEDLINE Link].
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Rodrigo JP, Suarez C, Rinaldo A, et al. Idiopathic midline destructive disease: fact or fiction. Oral Oncol. 2005 Apr. 41(4):340-8. [QxMD MEDLINE Link].
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Kwong YL, Kim WS, Lim ST, et al. SMILE for natural killer (NK)/T-cell lymphoma: analysis of safety and efficacy from the Asia Lymphoma Study Group. Blood. 2012 Aug 23. [QxMD MEDLINE Link].
Kim SJ, Yoon SE, Kim WS. Treatment of localized extranodal NK/T cell lymphoma, nasal type: a systematic review. J Hematol Oncol. 2018 Dec 20. 11 (1):140. [QxMD MEDLINE Link]. [Full Text].
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Media Gallery

Coronal (left) and axial (right) CT scans of the sinus reveal severe pansinusitis with abnormal nasopharyngeal thickening, right facial edema and right temporal bone opacification.
MRI revealed a low, non enhancing T1 signal in the right maxillary, ethmoid and sphenoid sinuses. (left) A high and inhomogeneous T2 signal suggested tumor involvement and destruction of the middle and inferior turbinates. (right)
High-power photomicrograph of a nasopharyngeal mass that was diagnosed as natural killer (NK)–/T-cell lymphoma, nasal type. In this section stained with hematoxylin and eosin, a diffuse infiltrate of variably sized cells with irregularly shaped nuclei that contain coarsely granular chromatin is visible. In other areas of this tumor, necrosis and angiocentrism could be appreciated.
In this photomicrograph, immunohistochemical staining shows neoplastic cells to be positive for the pan T-cell antigen CD3 (positive cells have a brown tinge).
In this photomicrograph, immunohistochemical staining shows neoplastic cells to be positive for the natural killer (NK)–cell antigen CD56 (positive cells have a brown tinge).
In this photomicrograph, immunohistochemical staining shows neoplastic cells to be focally positive for granzyme B (positive cells have a brown tinge).
In this photomicrograph, in situ hybridization for Epstein-Barr virus RNA (EBER) shows positivity in neoplastic cells (positive cells have black nuclei).

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Author

Benjamin Daniel Liess, MD Assistant Professor, Department of Otolaryngology, University of Missouri-Columbia School of Medicine

Benjamin Daniel Liess, MD is a member of the following medical societies: American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, The Triological Society, American Medical Association, Missouri State Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Jerry W Templer, MD Professor, Department of Otolaryngology, University of Missouri-Columbia School of Medicine

Jerry W Templer, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Otolaryngology-Head and Neck Surgery, Missouri State Medical Association, Society of University Otolaryngologists-Head and Neck Surgeons

Disclosure: Nothing to disclose.

Chief Editor

Arlen D Meyers, MD, MBA Emeritus Professor of Otolaryngology, Dentistry, and Engineering, University of Colorado School of Medicine

Arlen D Meyers, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, American Head and Neck Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Cerescan; Neosoma; MI10;<br/>Received income in an amount equal to or greater than $250 from: Neosoma; Cyberionix (CYBX)<br/>Received ownership interest from Cerescan for consulting for: Neosoma, MI10 advisor.

Acknowledgements

Benoit J Gosselin, MD, FRCSC Associate Professor of Surgery, Dartmouth Medical School; Director, Comprehensive Head and Neck Oncology Program, Norris Cotton Cancer Center; Staff Otolaryngologist, Division of Otolaryngology-Head and Neck Surgery, Dartmouth-Hitchcock Medical Center

Benoit J Gosselin, MD, FRCSC is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, American Head and Neck Society, American Medical Association, American Rhinologic Society, Canadian Medical Association, Canadian Society of Otolaryngology-Head & Neck Surgery, College of Physicians and Surgeons of Ontario, New Hampshire Medical Society,North American Skull Base Society, and Ontario Medical Association

Disclosure: Nothing to disclose.

M Sherif Said, MD, PhD Associate Professor of Pathology, Director of Head and Neck Pathology, Department of Pathology, University of Colorado School of Medicine

M Sherif Said, MD, PhD is a member of the following medical societies: American Society for Clinical Pathology and College of American Pathologists

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Acknowledgments

The authors would like to acknowledge Young S Paik, MD, for his assistance in the preparation and review of this manuscript.