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