world cancer report - iarc

Fig. 5.103 Classical Hodgkin disease. Hodgkin
(arrow) and Reed-Sternberg cells (arrowhead)
infected by the Epstein-Barr virus strongly express
the virus-encoded latent membrane protein LMP1.
Fig. 5.104 Burkitt lymphoma presenting as a large
tumour of the jaw in an African child.
Fig. 5.105 Microarray technology can be used to
identify two major patterns of gene expression
among diffuse large B-cell lymphomas (DLBCL).
One displays a germinal centre T-cell signature,
the other an activated B-cell signature. The analysis
is based on the expression of about 12,000
genes.
240 Human cancers by organ site
Management
The treatment of non-Hodgkin lymphomas
depends on the pathological classification,
the stage of the disease, the biological
behaviour of the disease, the age of the
patient and their general health [6,7]. In general,
it is convenient to classify the pathological
entities into indolent, aggressive or highly
aggressive non-Hodgkin lymphomas,
which parallels the IWF classification.
Indolent non-Hodgkin lymphomas
About two-thirds of indolent lymphomas in
developed countries are follicular lymphomas
and often present as advanced
stage disease in patients over 50 years of
age. This disease usually runs a prolonged
course and is rarely cured (except in a few
cases of early stage disease). The median
survival is eight to ten years, and therapy is
often palliative. Local radiotherapy is useful
for early stage localized disease, and other
options include alkylating agents, purine
analogues, combination chemotherapy,
interferon, monoclonal antibodies and high
dose therapy with autologous stem cell support.
Lymphoplasmacytoid lymphoma is
often associated with a monoclonal paraprotein
and, like small lymphocytic lymphoma/chronic
lymphocytic leukaemia, will
often respond to alkylating agent therapy.
Marginal zone lymphomas can be divided
into those at nodal sites (monocytoid B-cell
lymphomas) and those at extra nodal sites,
usually mucosal (gastrointestinal, lung, salivary
gland etc.) when they are termed MALT
(mucosa associated lymphoid tissue) lymphomas.
Gastric MALT lymphomas are often
associated with H. pylori infection and
appropriate antibiotic treatment often
results in resolution of the lymphoma, albeit
over six to twelve months [8]. Splenic marginal
zone lymphoma, often called splenic
lymphoma with villous lymphocytes, presents
with splenomegaly and usually
responds to splenectomy.
Aggressive non-Hodgkin lymphomas
Diffuse large cell lymphoma is the most
common of these types. Biologically
these tumours are more aggressive than
the indolent lymphomas, although remission
and even cure may be obtained with
appropriate therapy in a significant
proportion of cases. The factors associated
with prognosis in these patients
are age, stage, performance status, the
presence of extranodal disease, and lactic
dehydrogenase levels, which can be
summed to form the International
Prognostic Index. Using this model, four
risk groups can be identified with a predicted
five-year survival of 73%, 51%, 43%
and 26% when treated with conventional
anthracycline based chemotherapy (e.g.
cyclophosphamide, doxorubicin, vincristine,
prednisone). Attempts to improve
outcome with more aggressive chemo-
Histology Translocations
Small cleaved cell, follicular t(14;18)(q32;q21.3)
Small non-cleaved cell (Burkitt and non-Burkitt) t(8;14)(q24;q32)
t(2;8)(p12;q24)
t(8;22)(q24;q11)
Centrocytic/mantle cell t(11;14)(q13;q32)
Large cell, diffuse, B-cell t(3;14)(q27;q32)
t(3;22)(q27;q11)
t(2;3)(p12;q27)
Small lymphocytic/extranodal (MALT) t(11;18)(q21;q21.1)
Large cell, anaplastic t(2;5)(p23;q35)
Table 5.11 Some common chromosomal translocations found in non-Hodgkin lymphomas.