NK-Cell Lymphomas of the Head and Neck Clinical Presentation

  • Author: Benjamin Daniel Liess, MD; Chief Editor: Arlen D Meyers, MD, MBA   more...
 
Updated: Oct 26, 2011
 

History

  • General manifestations (< 20% of patients having B symptoms on presentation)
    • Weight loss
    • Fever
    • Fatigue
    • Night sweats
    • Bone pain
  • Head and neck manifestations
    • 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
    • Cough
    • Breathing restriction
  • Gastrointestinal manifestations
    • Pain
    • Abdominal fullness
    • Early satiety
    • Perforation
    • Hemorrhage
  • Genitourinary manifestations
    • Testicular pain
  • Skin
    • Non-healing sores
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Physical

  • General
    • Ulcerations involving the skin
    • Pallor
  • Head and neck
    • 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
    • Cough
    • Restricted breathing
  • Gastrointestinal
    • Hepatosplenomegaly
    • Abdominal mass
  • Genitourinary
    • Testicular mass
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Causes

Risk factors are as follows:

  • Inherited immunodeficiencies
    • Klinefelter syndrome
    • Chédiak-Higashi syndrome
    • Ataxia telangiectasia syndrome
    • Wiskott-Aldrich syndrome
    • Common variable immunodeficiency disease
  • Acquired immunodeficiency diseases
    • Iatrogenic immunosuppression
    • HIV-1 infection
    • Acquired hypogammaglobulinemia
  • Autoimmune disease
    • Sjögren syndrome
    • Celiac sprue
    • Rheumatoid arthritis and systemic lupus erythematosus
  • Chemical or drug exposures
    • Phenytoin
    • Dioxin, phenoxyl herbicides
    • Radiation
    • Prior chemotherapy and radiation therapy
  • Infectious agent association
    • EBV
    • Human T-cell leukemia/lymphoma virus-I
    • Ataxia telangiectasia syndrome
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Contributor Information and Disclosures
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, American Laryngological Rhinological and Otological Society, American Medical Association, and Missouri State Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Jerry W Templer, MD  Professor of Otolaryngology, University of Missouri Medical Center at Columbia

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, and Society of University Otolaryngologists-Head and Neck Surgeons

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

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

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.

Christopher L Slack, MD  Private Practice in Otolaryngology and Facial Plastic Surgery, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders

Christopher L Slack, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Medical Association

Disclosure: Nothing to disclose.

Chief Editor

Arlen D Meyers, MD, MBA  Professor, Department of Otolaryngology-Head and Neck Surgery, 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, and American Head and Neck Society

Disclosure: Covidien Corp Consulting fee Consulting; US Tobacco Corporation Unrestricted gift Unknown; Axis Three Corporation Ownership interest Consulting; Omni Biosciences Ownership interest Consulting; Sentegra Ownership interest Board membership; Syndicom Ownership interest Consulting; Oxlo Consulting; Medvoy Ownership interest Management position; Cerescan Imaging Honoraria Consulting; GYRUS ACMI Honoraria Consulting

Additional Contributors

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

References

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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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