1. Lymphomas of the Head and Neck
Tal Marom, MD

3. 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↑)

4. Hodgkin vs. Non-Hodgkin Ly.
Non-Hodgkin's Lymphomas
Hodgkin's  Lymphoma
Average age is about 67. 
Average age is 27.7 with two age peaks, the major one between 15 and 24 with a lesser peak after age 55.
Age
Men 2.12% Women 1.79%
Men 0.23% Women 0.20%
Lifetime prevalence
About 85% of all lymphomas
About 15% of all lymphomas
Occurrence

5. Hodgkin vs. Non-Hodgkin Ly.
Non-Hodgkin's Lymphomas
Hodgkin's  Lymphoma
LN above the collar bone. In NHL it is also more likely to appear in the mesenteric nodes in the abdomen.
The disease occurs in the chest cavity in less than 40% of patients. (An exception, lymphoblastic lymphoma, which is seen most often in young people, is likely to first appear in the chest.) Extra-nodular disease in about 23% of patients. Slow-growing lymphomas are common in the liver and bone marrow.
LN above the collar bone. In Hodgkin's it is also more likely to appear in the chest cavity (mediastinum), particularly in younger patients. Only about 15% to 20% of cases are below the diaphragm.
Extra-nodular disease in about 4% of cases.
Location

6. Hodgkin vs. Non-Hodgkin Ly.
Non-Hodgkin's Lymphomas
Hodgkin's  Lymphoma
B-Lymphocytes (>90%), T-Lymphocytes or Natural Killer (NK) Cells
B-Lymphocytes characterized by the Reed-Sternberg Cell
Affected Lymph Cells
Less likely than HL to have systemic ("B") symptoms (27%) at the time of diagnosis.
More likely (40%) to have systemic ("B") symptoms at the time of diagnosis.
Symptoms
More likely than HD to be diagnosed in stage IV (36%) but this will vary by NHL subtype. 
The Non-Hodgkin's lymphomas are less predictable in their course than Hodgkin's and they are more apt to spread
Less likely to be diagnosed in stage IV (10%).
Hodgkin's disease usually progresses slowly (or aggressively) in an orderly way from one lymph node region to the next.
If it spreads below the diaphragm, it usually reaches the spleen first; the disease then may spread to the liver and bone marrow. If the disease starts in the nodes in the middle of the chest, it may spread outward to the chest wall and areas around the heart and lungs.
Progression

7. Reed Sternberg Cell

8. 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

9. 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

10. Physical examination
History – cough, fever, GI, abdominal masses, pruritus
Full PE, look for peripheral adenopathy

11. Differential diagnosis
infectious etiologies Bacteria Viruses (eg, infectious mononucleosis, cytomegalovirus, HIV) Parasites (eg, toxoplasmosis)
Nasal granulomatous disease Wegener granulomatosis Lymphomatoid granulomatosis Infections (eg, leishmaniasis, syphilis, TB)
Mediastinal presentation Infections (eg, histoplasmosis, tuberculosis) Sarcoidosis Other neoplasms
Benign lymphoid hyperplasias B-cell predominant - Cutaneous lymphoid hyperplasia (CLH), angiolymphoid hyperplasia with eosinophilia, Kimura disease, and Castleman disease T-cell predominant - T-cell CLH, lymphomatoid contact dermatitis, and lymphomatoid drug eruption
Other neoplasms Squamous cell carcinoma Nasopharyngeal carcinoma Thyroid carcinoma

12. Lab studies CBC Chemistry, liver, LDH ESR Urine
Serum β2 microglobulin (worse prognosis)
CXR
Total body CT
Galium scanning
Genetic studies

13. Procedures Fiberoscopy- NPH, larynx FNA-C from neck mass
Excisional/Incisional Bx
Diagnostic TE
(Bone marrow, LP, liver, explorative laparotomy…)

14. 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

15. Chemotherapy – major modality Radiotherapy Surgery ??? Relapse !!!
Treatment
Chemotherapy – major modality
Radiotherapy
Surgery ???
Relapse !!!

16. 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)

17. Chemotherapy Non-Hodgkin Lymphoma:
CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisolone)
Purine analogues

18. Other procedures Stem cell transplantation IL-2 Recomninat INF-α
Bone marrow transplantation?

19. 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

20. 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