Transcript Lymphomas of the Head and Neck

Lymphomas of the
Head and Neck
Tal Marom, MD
Lymphoma
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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↑)
Hodgkin vs. Non-Hodgkin Ly.
Hodgkin's
Lymphoma
Non-Hodgkin's
Lymphomas
Age
Average age is 27.7
with two age peaks, the
major one between 15
and 24 with a lesser
peak after age 55.
Average age is about 67.
Lifetime
prevalence
Men 0.23%
Women 0.20%
Men 2.12%
Women 1.79%
Occurrence
About 15% of all
lymphomas
About 85% of all
lymphomas
Hodgkin vs. Non-Hodgkin Ly.
Hodgkin's
Lymphoma
Location
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.
Non-Hodgkin's
Lymphomas
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.
Hodgkin vs. Non-Hodgkin Ly.
Hodgkin's
Lymphoma
Non-Hodgkin's Lymphomas
Affected
Lymph Cells
B-Lymphocytes
characterized by the ReedSternberg Cell
B-Lymphocytes (>90%),
TLymphocytes or Natural Killer
(NK) Cells
Symptoms
More likely (40%) to have
systemic ("B") symptoms at the
time of diagnosis.
Less likely than HL to have
systemic ("B") symptoms
(27%) at the time of diagnosis.
Progression
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.
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
Reed Sternberg Cell
H&N Lymphoma
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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.
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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
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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
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History – cough, fever, GI,
abdominal masses, pruritus
Full PE, look for peripheral
adenopathy
Differential diagnosis
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infectious etiologies
Bacteria
Viruses (eg, infectious mononucleosis, cytomegalovirus, HIV)
Parasites (eg, toxoplasmosis)
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Nasal granulomatous disease
Wegener granulomatosis
Lymphomatoid granulomatosis
Infections (eg, leishmaniasis, syphilis, TB)
Mediastinal presentation
Infections (eg, histoplasmosis, tuberculosis)
Sarcoidosis
Other neoplasms
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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
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Other neoplasms
Squamous cell carcinoma
Nasopharyngeal carcinoma
Thyroid carcinoma
Lab studies
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CBC
Chemistry, liver, LDH
ESR
Urine
Serum β2 microglobulin (worse prognosis)
CXR
Total body CT
Galium scanning
Genetic studies
Procedures
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Fiberoscopy- NPH, larynx
FNA-C from neck mass
Excisional/Incisional Bx
Diagnostic TE
(Bone marrow, LP, liver,
explorative laparotomy…)
Ann Arbor Lymphoma staging
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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
modality
 Radiotherapy
 Surgery ???
 Relapse !!!
– major
Chemotherapy
Hodgkin disease –
- MOPP (mechlorethamine [nitrogen mustard],
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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
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prednisolone)
Purine analogues
Other procedures
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Stem cell transplantation
IL-2
Recomninat INF-α
Bone marrow transplantation?
Radiotherapy
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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
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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