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Learn about lymphomas affecting the head and neck region, including clinical presentation, diagnosis, staging, treatment options, and relevant differential diagnoses. Gain insights into available lab tests, diagnostic procedures, staging methods, and treatment modalities.
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Lymphomas of the Head and Neck Tal Marom, MD
Lymphoma • Cancer of the lymphatic system • Lymphoma is differentiated by the type of cell that multiplies and how the cancer presents itself • Two main groups: Hodkgin’s disease and NHL • US prevalence (HD) = 3 cases/100,000 (incidence ↓) • US prevalence (NHL)=16 cases/100,000 (incidence↑)
H&N Lymphoma • Lymphoma is the second most common primary malignancy occurring in the head and neck, and incidence of aggressive non-Hodgkin lymphoma is rising in young and middle-aged patients. • 25% of all extra-nodal lymphomas occur in the head and neck, and 8% of supraclavicular fine-needle aspirates are diagnosed as lymphoma. • REAL classification (Revised European American Lymphoid neoplasm) : indolent, aggressive and Hodgkin disease
Clinical presentation • Neck- lymphadenopathy, ulcerated mass • Oropharynx- enlarged tonsil, tongue base thickening • Nasopharynx – mass, SOM • Nose & paranasal sinuses- ulcerated destructive lesion –susp. NK/T cell Lymphoma (“midline lethal granuloma”), associated with EBV • Thyroid - neck swelling, hoarseness, dysphagia, or neck pressure/tenderness • Salivary glands- masses • Base skull – cranial neuropathy, facial pain, hearing loss, vertigo, proptosis, or visual symptoms
Physical examination • History – cough, fever, GI, abdominal masses, pruritus • Full PE, look for peripheral adenopathy
Differential diagnosis • infectious etiologiesBacteriaViruses (eg, infectious mononucleosis, cytomegalovirus, HIV)Parasites (eg, toxoplasmosis) • Nasal granulomatous diseaseWegener granulomatosisLymphomatoid granulomatosisInfections (eg, leishmaniasis, syphilis, TB) • Mediastinal presentationInfections (eg, histoplasmosis, tuberculosis)SarcoidosisOther neoplasms • Benign lymphoid hyperplasiasB-cell predominant - Cutaneous lymphoid hyperplasia (CLH), angiolymphoid hyperplasia with eosinophilia, Kimura disease, and Castleman diseaseT-cell predominant - T-cell CLH, lymphomatoid contact dermatitis, and lymphomatoid drug eruption • Other neoplasmsSquamous cell carcinomaNasopharyngeal carcinomaThyroid carcinoma
Lab studies • CBC • Chemistry, liver, LDH • ESR • Urine • Serum β2 microglobulin (worse prognosis) • CXR • Total body CT • Galium scanning • Genetic studies
Procedures • Fiberoscopy- NPH, larynx • FNA-C from neck mass • Excisional/Incisional Bx • Diagnostic TE • (Bone marrow, LP, liver, explorative laparotomy…)
Ann Arbor Lymphoma staging • Stage I - Involvement of a single LN region/ lymphoid structure • Stage II - Involvement of 2 or more LN regions on the same side of the diaphragm or localized contiguous involvement of only one extra-lymphatic site and LN region • Stage III - Involvement of LN regions or lymphoid structures on both sides of the diaphragm • Stage IV - Disseminated involvement of one or more extra- lymphatic organs with or without LN involvement and/or involvement of the bone marrow or liver
Treatment • Chemotherapy – major modality • Radiotherapy • Surgery ??? • Relapse !!!
Chemotherapy • Hodgkin disease – - MOPP(mechlorethamine [nitrogen mustard], vincristine, procarbazine, and prednisolone) [2% post-Rx ALL, infertility] - ABVD(doxorubicin (Adriamycin), bleomycin, vinblastine, and dacarbazine) - For advanced disease : BEACOPP(cyclophosphamide, doxorubicin, etoposide, procarbazine, prednisolone, vincristine, and bleomycin with granulocyte colony-stimulating factor)
Chemotherapy • Non-Hodgkin Lymphoma: CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisolone) Purine analogues
Other procedures • Stem cell transplantation • IL-2 • Recomninat INF-α • Bone marrow transplantation?
Radiotherapy • Mantle field includes the submandibular, cervical, supraclavicular, infraclavicular, axillary, mediastinal, and hilar lymph nodes • It can be extended to cover the Waldeyer ring and the skull base with lateral portals for lymphomas involving the skull base • Combined= Chemo + Radio
Surgical treatment • Excision of necrotic tissue may be necessary in nasal NK/T-cell lymphoma • MALT lymphomas are often treated surgically with or without local radiotherapy • Lymphomas of the CNS and skull base are treated with surgical decompression when necessary