Lymphomas of the Head and Neck Clinical Presentation

Updated: May 28, 2019
  • Author: Kieron M Dunleavy, MD; Chief Editor: Arlen D Meyers, MD, MBA  more...
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Presentation

History

Lymphoma may be nodal or extranodal. Extranodal lymphoma is usually NHL and worsens the patient's prognosis. HL extends by means of contiguous nodal spread; therefore, it is often localized and frequently occurs in the mediastinum. NHL tends to spread hematogenously and is often systemic at diagnosis.

  • A nodal presentation of HL is typically with 1 or more small-to-medium, rubbery lymph nodes in the neck, which may wax or wane in size but grow over time.

    • Biopsy should be considered when a firm lymph node larger than 1 cm that is not associated with infection and that persists longer than 4 weeks.

    • In 2-5% of patients with HL, involved nodes can become painful after they drink alcoholic beverages.

  • NHL may manifest in the cervical region and lymphoid tissue of the Waldeyer ring. It appears as a mass in the oropharynx or nasopharynx.

    • Unilateral tonsillar enlargement is highly suggestive of malignancy.

    • Extranodal disease, such as primary lymphoma, of the oral cavity is less common than other findings; this disease usually arises in the tongue base.

    • In contrast to squamous cell carcinoma, NHL is bulky, fleshy, and nonulcerating.

  • Extranodal NK/T-cell lymphoma, nasal type (formerly called lethal midline granuloma), can manifest as an ulcerative destructive lesion of the nose, sinuses, and face.

    • This lesion is usually associated with Epstein-Barr virus and worsens the patient's prognosis compared with that of patients with B-cell lymphoma.

    • The disease often manifests with a nasal mass, epistaxis, and/or nasal obstruction. (See the video below of a fiberoptic examination of an NK/T-cell lymphoma in the right nasal cavity and maxillary sinus.)

      Fiberoptic nasal examination of a patient with natural killer (NK)/T-cell lymphoma of the right nasal cavity and maxillary sinus.
  • Lymphoma in the thyroid is more common than anaplastic carcinoma of the thyroid. It manifests as a neck swelling, hoarseness, dysphagia, or neck pressure/tenderness.

    • Advanced disease can cause facial edema and Horner syndrome.

    • Extranodal marginal-zone B-cell lymphoma of MALT lymphoma occurs in the head and neck, in the ocular adnexa, and in thyroid areas.

    • Patients with Sjögren syndrome are at increased risk for MALT lymphomas in the oral mucosa.

    • Lymphoma involving the base of the skull or CNS can cause cranial nerve palsies, facial pain, hearing loss, vertigo, proptosis, or visual symptoms.

  • Constitutional symptoms, designated B symptoms, may occur in up to one third of patients with lymphoma.

    • B symptoms include weight loss greater than 10% of the patient's previous body weight, temperatures higher than 38°C without evidence of infection, and/or drenching night sweats.

    • Symptoms such as bony pain, headaches, or GI discomfort may indicate generalized disease.

    • A persistent cough can be the presenting feature of mediastinal lymphadenopathy, as can chest pain, shortness of breath, or hypertrophic osteoarthropathy.

    • Pruritus, malaise, fatigue, and weakness are nonspecific symptoms that occur in 10% of patients.

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Physical

Full otorhinolaryngologic and neck examination including fiberoptic examination, in addition to complete physical examination, is indicated.

  • Examine patients for painless or mildly tender peripheral adenopathy in cervical, axillary, inguinal, and femoral regions. Less typically, enlargement of epitrochlear nodes is associated with enlargement of the Waldeyer ring.

  • Superior vena cava (SVC) syndrome and pleural effusions may be due to a mediastinal mass.

  • Some patients with indolent NHLs may have large, asymptomatic abdominal masses.

    • Splenic or hepatic enlargement may be detected.

    • The endemic (African) form of Burkitt lymphoma manifests as a jaw or abdominal tumor that spreads to extranodal sites, but the nonendemic (American) form has an abdominal presentation with massive disease.

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Causes

Incidence of HL is increased 10-fold in same-sex siblings and by as much as 100-fold in identical twins. This observation implies genetic factors in the etiology.

  • Chromosomal abnormalities are common in lymphomas.

  • Aneuploidy occurs in HL, and translocations and deletions mark NHL, such as the c-myc translocation of Burkitt lymphoma and the bcl-2 translocation found in most follicular lymphomas.

Infectious agents implicated in the pathogenesis of some lymphomas include Epstein-Barr virus, HIV-1 (aggressive NHL occurs in 10-30% of patients with AIDS), Helicobacter pylori, human t-cell lymphotropic virus-1 (HTLV-1), hepatitis B and C viruses, human herpes virus 8, Borrelia burgdorferi, Chlamydia psittaci, and Campylobacter jejuni.

Chronic inflammation increases the risk of lymphoma, such as a MALT lymphoma arising in the salivary gland in a patient with Sjögren syndrome.

  • Hashimoto disease increases a patient's risk of thyroid lymphoma, usually aggressive NHL, about 70-fold.

  • In general, patients with autoimmune diseases, such as rheumatoid arthritis, have an increased risk of lymphoma.

Immunosuppressive medications, for example those used following organ allotransplantation, increase the risk of Epstein-Barr virus–associated NHL. A percentage of these lymphomas regress spontaneously when the immunosuppressive medication is discontinued.

Farming, welding, and work in the lumber industry are associated with an increased risk of lymphoma.

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