Lymphomas of the Head and Neck 

  • Author: Kieron M Dunleavy, MD; Chief Editor: Arlen D Meyers, MD, MBA   more...
 
Updated: Apr 1, 2010
 

Background

Otolaryngologists are frequently involved in the diagnosis of lymphoma. A quarter of all extranodal lymphomas occur in the head and neck, and 8% of findings on supraclavicular fine-needle aspirate biopsy yield a diagnosis of lymphoma. In white populations, lymphoma is a more common cause of cervical lymphadenopathy than metastatic disease. Lymphoma is the second most common primary malignancy occurring in the head and neck and importantly, the incidence of aggressive non-Hodgkin lymphoma has risen steadily over recent decades.

The image below depicts a lymphoma of the head and neck.

CT scan of a patient with a natural killer (NK)/T-CT scan of a patient with a natural killer (NK)/T-cell lymphoma of the right nasal cavity and maxillary sinus.
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Pathophysiology

Although a variety of histologic classification schemes have been used for lymphoma in the past, the World Health Organization (WHO) classification is currently used.

The WHO classification of lymphomas is as follows:

  • Hodgkin lymphoma (HL)
    • Nodular lymphocyte predominant
    • Classical
    • Nodular sclerosis classical
    • Mixed cellularity classical
    • Lymphocyte-rich classical
    • Lymphocyte-depleted classical
  • Non-Hodgkin lymphoma (NHL) - B-cell neoplasms
    • Precursor B-cell neoplasms
    • Precursor B lymphoblastic leukemia/lymphoma
    • Mature B-cell neoplasms
      • Chronic lymphocytic leukemia, small lymphocytic lymphoma
      • Lymphoplasmacytic lymphoma
      • Splenic marginal-zone lymphoma
      • Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT)
      • Nodal marginal-zone lymphoma
      • Follicular lymphoma
      • Primary cutaneous follicle center lymphoma
      • Mantle-cell lymphoma
      • Diffuse large B-cell lymphoma (DLBCL)
      • Primary mediastinal (thymic) large B-cell lymphoma
      • Intravascular large B-cell lymphoma
      • ALK positive large B-cell lymphoma
      • Plasmablastic lymphoma
      • Large B-cell lymphoma arising in HHV8-associated multicentric Castleman disease
      • Primary effusion lymphoma
      • Burkitt lymphoma
      • Lymphomatoid granulomatosis
  • NHL - T-cell and natural killer (NK)–cell neoplasms
    • Precursor T-cell neoplasms
      • Precursor T lymphoblastic leukemia or lymphoma
      • Blastic NK-cell lymphoma
    • Mature T- and NK-cell neoplasms
      • Adult T-cell leukemia or lymphoma
      • Extranodal NK- or T-cell lymphoma, nasal type
      • Enteropathy-associated T cell lymphoma
      • Hepatosplenic T-cell lymphoma
      • Subcutaneous panniculitis-like T cell lymphoma
      • Mycosis fungoides
      • Sezary syndrome
      • Primary cutaneous CD30 positive T-cell lymphoproliferative disorders
      • Primary cutaneous peripheral T-cell lymphomas, rare subtypes
      • Peripheral T cell lymphoma, not otherwise specified (NOS)
      • Angioimmunoblastic T cell lymphoma
      • Anaplastic large cell lymphoma
  • Immunodeficiency-associated lymphoproliferative disorders
    • Lymphomas associated with HIV infection
    • Post-transplant lymphoproliferative disorders (PTLD)
    • Lymphoproliferative diseases associated with primary immune disorders
    • Other iatrogenic immunodeficiency-associated lymphoproliferative disorders

HL is characterized by the presence of Reed-Sternberg (RS) cells, and the subtype diagnosis depends on the cytoarchitectural milieu in which the RS cells or their variants are found. Nodular sclerosis, mixed cellularity, lymphocyte-rich and lymphocyte-depleted subtypes are collectively termed classic HL. Nodular sclerosis is the most common subtype, especially in patients younger than 40 years, followed by mixed cellularity. Lymphocyte-predominant HL, more common in young men than in others, behaves more like a low-grade B-cell lymphoma than other tumors. In general, patients who are elderly, those who live in developing countries, and those infected with HIV are most likely to have widespread disease with systemic symptoms at diagnosis.

Approximately 85% of NHLs are B-cell lymphomas. The most common indolent NHL is follicular lymphoma, which is derived from germinal center B cells. Other indolent histologies are lymphoplasmacytoid lymphoma, which has characteristics of B cells differentiating toward plasma cells, and marginal-zone lymphoma derived from the memory B-cell compartment, which includes MALT lymphomas. DLBCL is the most common aggressive NHL. On the basis of messenger RNA microarrays, most cases have profiles that indicate an origin from a germinal center B cell or a postgerminal-center activated B cell. Mantle-cell lymphoma and Burkitt lymphoma are aggressive NHLs that have the characteristics of normal B cells residing in the mantle zone or in the germinal center of a lymphoid follicle, respectively.

Cutaneous T-cell lymphomas, such as mycosis fungoides, can be indolent. However, many T-cell NHLs are aggressive malignancies.

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Epidemiology

Frequency

United States

Lymphoma is the fifth most common cancer in the United States, with an annual incidence of 74,490 estimated cases. Approximately 88% of these cancers are NHLs. The incidence of NHL has doubled over the last 20 years because of the increase in AIDS-related lymphoma (ARL);[1] an increase in the detection of lymphoma; and for other, poorly understood reasons.

International

The different histologic subtypes of NHL have various distributions and geographic predilections. The frequency of NK/T-cell lymphoma is increased in China, in Taiwan, in Southeast Asia and in parts of Africa where Burkitt lymphoma is endemic.

Mortality/Morbidity

For HL, overall 5-year survival rates in the United States are 83% for whites and 77% for African Americans. For NHL, the 5-year survival rate is 53% for whites and 42% for African Americans.

Race

HL and, to a lesser extent, NHL are more common in whites than in African Americans or Hispanics. Other races such as Asian/Pacific islanders or American Indians have the lowest incidence and mortality rates.

Sex

The incidence of both HL and NHL are higher in men than in women, especially among older patients.

Age

In the United States, HL has a bimodal age distribution, with a peak incidence in people aged 20-34 years and a second peak in whites aged 75-79 years and in African Americans aged 55-64 years. In Japan, the early peak is absent, and in some developing countries, the early peak shifts into childhood.

The mortality rate increases with age. For example, incidence and mortality rates for NHL increase with age. In addition, Burkitt lymphoma represents 40-50% of all pediatric lymphomas but is uncommon in adults without AIDS.

Lymphoblastic lymphoma most commonly affects men aged 20-40 years who have lymphadenopathy and/or a mediastinal mass.

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Contributor Information and Disclosures
Author

Kieron M Dunleavy, MD  Investigator/Attending Physician, National Cancer Institute

Kieron M Dunleavy, MD is a member of the following medical societies: American Society of Hematology

Disclosure: Nothing to disclose.

Coauthor(s)

Erik Kass, MD  Chief, Department of Clinical Otolaryngology, Associates in Otolaryngology of Northern Virginia

Erik Kass, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Association for Cancer Research, American Medical Association, and American Rhinologic Society

Disclosure: Nothing to disclose.

Wyndham Wilson, MD, PhD  Senior Investigator, Division of Clinical Sciences, National Institutes of Health National Cancer Institute

Disclosure: Nothing to disclose.

Specialty Editor Board

Daniel J Kelley, MD  Consulting Staff, Eastern Shore ENT and Allergy Associates and Peninsula Regional Medical Center

Daniel J Kelley, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Head and Neck Society, American Laryngological Rhinological and Otological Society, and Pennsylvania Medical Society

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

Karen H Calhoun, MD, FACS, FAAOA  Professor, Department of Otolaryngology-Head and Neck Surgery, Ohio State University College of Medicine

Karen H Calhoun, MD, FACS, FAAOA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Head and Neck Society, American Medical Association, American Rhinologic Society, Association for Research in Otolaryngology, Society of University Otolaryngologists-Head and Neck Surgeons, Southern Medical Association, Texas Medical Association, and Texas Medical Association

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

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CT scan of a patient with a natural killer (NK)/T-cell lymphoma of the right nasal cavity and maxillary sinus.
CT scan 6 months after treatment with 4 cycles of DA-EPOCH (ie, infused etoposide, doxorubicin, and vincristine with bolus cyclophosphamide and prednisone).
CT scan of a patient with a recurrence of stage I-AE angiocentric lymphoma of the left maxillary sinus, treated 7 years earlier with 4 cycles of ProMACE-MOPP (ie, prednisone, methotrexate, Adriamycin, cyclophosphamide, etoposide–mechlorethamine [nitrogen mustard], vincristine, procarbazine, and prednisone) and 3960 cGy of radiation.
CT scan 2 years after salvage therapy.
Fiberoptic nasal examination of a patient with natural killer (NK)/T-cell lymphoma of the right nasal cavity and maxillary sinus.
 
 
 
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