hodgkins disease-brain ca
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Hodgkins Disease & Non Hodgkins Lymphoma
2
Background
OverviewThe malignant lymphomas, Hodgkins disease (HD) and non-Hodgkins lymphoma (NHL), comprise a
group o clinically and pathologically diverse malignant lymphomas o largely unknown cause! Their
successul management involves a multidisciplinary approach, proceeding rom an accurate diagnosis to
a comprehensive staging evaluation and appropriate therapeutic recommendation and regimen!
"tiology # $athogenesis
%n &', Thomas Hodgkin presented a paper entitled *On +ome orid .ppearances o the .sorent
/lands and +pleen0! His report was an autopsy description o seven patients with an entity he descried
as a primary process involving the lymph nodes and spleen rather than a reactive inlammatory condition!
%ndependent reports y +ternerg and 1eed descried the giant cells characteristic o Hodgkins disease,
which are known today as 1eed-+ternerg (1+) cells!
The 1+ cell is characteri2ed y its large si2e and classic inucleated structure and large eosinophilic
nucleoli! %t is the tumor cell characteri2ing HD and must e identiied within the appropriate cellular milieu
o lymphocytes, eosinophiles and histiocytes in order or the diagnosis to e estalished! Hodgkins
disease is uni3ue pathologically ecause the tumor cells comprise a minority o the cell population in
which normal inlammatory cells are the ma4or cellular components! The 1+ cell is characteri2ed as oth
a macrophage and lymphocyte and has the aility to phagocytose! 1+ cells are not asolutely speciic or
HD and have een noted in inectious mononucleosis5 and in other malignancies including lymphomas,
carcinomas and sarcomas!
%n the years that have ollowed the initial identiication o Hodgkins disease as a neoplastic entity, a
heterogeneous group o over 6 additional and discrete non-Hodgkin lymphomatous neoplasms have
een descried with characteristic morphologic, immunophentypic, genetic and clinical eatures! This
group has een classiied as non-Hodgkin lymphomas (NHL) and, with an ever-increasing incidence, now
is the si7th most common cause o cancer in the 8nited +tates!
The precise etiology and pathogenesis o oth HD and NHLare unknown! There is, however, convincing
evidence that most types o HD and non-Hodgkins lymphoma are derived rom germinal center or post-
germinal center B-cells (one marrow) and that the B-cell germinal center plays a key role in oth normal
B-cell dierentiation and in the genesis o B-cell neoplasms! 9ymphoid neoplasms o T-cell (thymus) origin
constitute less than &:; o NHLgroup, the vast ma4ority eing o B-cell origin!
The e7act mechanism that triggers the transormation to malignancy is unknown, ut over the years a
variety o inectious agents, congenital and ac3uired immunodeiciency states, autoimmune disorders andphysical and chemical agents have een associated with an increased risk o these disorders!
The arguments or an inectious etiology o HD date ack to its early descriptions! ycoacterium
tuerculosis was the irst organism to e suspected o causing HD! However, once it was reali2ed that HD
is associated with immune deects, ! tuerculosis was recogni2ed as a conse3uence o HD rather than
a cause o the disease! 1eports o clustering o cases tended to support the inectious etiology, ut these
reports were suse3uently recogni2ed as chance occurrence!
http://nursinglink.monster.com/training/articles/332-hodgkins-disease-non-hodgkins-lymphomahttp://nursinglink.monster.com/training/articles/332-hodgkins-disease-non-hodgkins-lymphoma -
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The "pstein-Barr virus (EBV) has een another proposed cause o Hodgkins disease, and the
circumstantial evidence is impressive! The incidence o HD is elevated in patients with a history
o EBVinection! The "-B virus, which inects and immortali2es B-lymphocytes in vitro, has een
implicated in the pathogenesis o .rican Burkitts lymphoma and B-cell malignancies in immuno-
compromised patients such as those that occur ater organ transplantation! 8sing molecular iological
techni3ues, EBVgenome ractions have een ound in 1eed-+ternerg cells!
The possiility that an anormal immune response is related to the development o HD has een given
support y reports that there is as much as a twenty-old increase in HD among HIV-inected individuals!
The data are even more impressive or the incidence o non-Hodgkins lymphoma< patients with long-
standing human immunodeiciency virus (HIV) inection are also estimated to have a &66-old increased
risk or the development o =H9s! %n the early &>'6s, an increase in intermediate and high-
grade NHLwas noticed among young homose7ual men! .nalysis o a series o >6 such cases revealed
that their age distriution was identical to that o AIDS, with the highest percentage seen etween ages
6 and >, in contrast to NHLin the general population where the median age was etween :: and ?6
years! %n this and suse3uent series, 6; to 56; o patients had a lymphoma similar in histology to
Burkitts lymphoma, a tumor that is epidemic in e3uatorial .rica and rare in the 8nited +tates!
Helicoacter pylori inection o the stomach results in chronic gastritis and the development o mucosa-
associated lymphoid tissue (MALT) and associated gastric lymphomas! This link etween H! pylori
inection and primary gastric MALTlymphomas had prompted the development o treatment strategies
ased on the eradication o the H! pylori inection! The human T-cell leukemia virus (HTLV) and the
@aposis sarcoma-associated herpes virus (KSHV) are two rare viruses that have also een associated
with NHL! .dditionally, certain chemical and physical agents have een associated with an increased risk
o NHL, as is the case with the survivors o nuclear e7plosions or reactor accidents and in patients with
other tumors that have een treated with certain chemotherapies and#or radiation!
The idea that HD may represent an uncommon host response to a common agent has received support
rom a study o mono2ygotic and di2ygotic twins! ono2ygotic twins, who would e e7pected to have
similar immune responses, had a >>-old increased risk o eing concordant or having HD, supporting a
role or genetic susceptiility and#or an anormal immune response in the etiology o HD!
.ccurate classiication o the malignant lymphoid neoplasms is essential or the correct diagnosis and or
the evaluation o prognosis and treatment o patients! Hodgkins disease has een classiied into our
histological sutypes! "ach sutype classiication is ased on the numer and appearance o 1eed-
+ternerg cells as well as the ackground histological milieu!
+everal classiications have een proposed or the comple7 array o non-Hodgkins lymphomas! These
have een ased on the degree o dierentiation o the tumor and on whether the growth pattern is
nodular or diuse! Other classiications have een ased on the cell o origin o the malignancy! The
%nternational $anel Aorking ormulation o the =ational Cancer %nstitute has separated NHLinto
categories, each incorporating the aove-mentioned items and having therapeutic implications&'! Their
generally accepted NHLclassiication identiies our sutypes and their respective incidences
9ow grade or avorale prognosis lymphomas (';)
Diuse, well dierentiated
=odular, poorly dierentiated
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=odular, mi7ed types
%ntermediate grade or intermediate prognosis lymphomas (56;)
=odular, histiocytic
Diuse, poorly dierentiated
9ymphocytic
Diuse E mi7ed types
High grade or unavorale prognosis lymphoma (6;)
Diuse histiocytic lymphoma (diuse large cell cleaved, noncleaved, and immunolastic types)
Diuse, undierentiated (Burkitts and non-Burkitts type)
iscellaneous lymphomas (;)
Composite lymphoma
ycosis ungoides
True histiocytic, other, and unclassiiale types
The ovious comple7ity o the current classiication o =H9s has ta7ed the aility o hematopathologists!
The diagnosis o a non-Hodgkins lymphoma is un3uestionaly one o the most diicult tasks conronting
a general surgical pathologist! The diiculties involved have resulted in many institutions routinely
reerring new cases oNHLto e7pert hematopathologists or review and consultation!
Demographics # "pidemiology
.ppro7imately F,:66 new cases o Hodgkins disease are diagnosed annually in the 8nited +tates! %n
contrast to the increasing incidence o non-Hodgkins lymphoma, the annual incidence o HD has
remained stale over the last several decades! The male to emale ratio o HD incidence is &! E &!5 to &!
%n most economically developed countries, there is a imodal age distriution with one peak occurring inthe third decade o lie (age range &:-> years) and a second smaller peak occurring ater age :6! The
second peak may e an artiact o histologic misclassiication ecause recent studies have demonstrated
that many o the older-age cases originally diagnosed as HD turned out to e =H9s! The age-speciic
incidence is low, no early incidence peak occurs! %n some developing countries, the peak shits into
childhood, mostly oys!
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%n contrast to the stale and relatively static incidence o Hodgkins disease, non-Hodgkins lymphoma is
emerging as a worldwide epidemic! ore than :6,666 new cases were diagnosed annually in the 8nited
+tates during the &>>6s and there is evidence that the incidence is increasing! +ome o this increase is
undoutedly related to the predilection or the development o NHLin patients with AIDS! However, a
large numer o other actors may e contriuting to the increase in incidence since NHLwas maniesting
a steady increase o ; to 5; per year in the &>F6s prior to the emergence o the AIDScrisis! The rise
in NHLincidence is aster and higher than that o all other malignancies e7cept lung cancer in women and
prostate cancer!
The incidence o NHLincreased '?!?; in males rom &>F to &>>& with an estimated annual change o
!';! .s a result, NHLnow constitutes aout ?; o all malignancies in the 8nited +tates!
The incidence o the various non-Hodgkins lymphomas is age-dependent, has a variale distriution, and
is more common in males than emales! 9ymphomas represent appro7imately &6; o all childhood
cancers in developed countries and are third in relative re3uency ehind acute leukemias and rain
tumors! They are more common in adults than in children and maniest a steady increase in incidence
rom childhood through age '6! The mean age at diagnosis o NHLis 5: to :: years and the median age
is ?6 to ?: years! The greatest rise in new cases o NHLhas een in elderly white males and the least
increase has een seen in young white emales! The increase in incidence o NHLhas een
accompanied y an increase in e7tra and diuses involvement such as involvement o the gastrointestinal
tract and oropharyn7! Other common e7tranodal sites include the rain, skin, one, thyroid, lung, reast
and testis!
Diagnosis
History
It is important that the history include any occurrence of lymphoma in the familyand a history of preious infectious mononucleosis or e!posure to radiation or to!icchemicals "y the patient# Sti$mata su$$estin$ the presence of an immunode%ciencydisorder should "e sou$ht "ecause of the predilection for patients &ith AIDS todeelop non'Hod$(in)s lymphoma#
@ey aspects o the medical history o HD or NHLpatient include the determination o whether he#she has
symptoms that have a prognostic signiicance as well as a possile inluence on the therapeutic regimen!
The physician should ocus on symptoms that suggest a speciic e7tranodal site (e!g!, one pain), as well
as symptoms such as ever, night sweats and a weight loss e3ual or greater than &6; o the patients
ody weight, since these complaints usually signal the presence o diuse disease and have a signiicant
inluence on prognosis and uture therapy! %n lymphomatous malignancies ever may e maniest in any
pattern including a low-grade continuous ever or occasional high spikes o ever! The pattern o recurrent
episodes o daily high ever separated y days without ever is known as $el-"pstein ever! $ain at the
site o Hodgkins disease involvement in association with the ingestion o alcohol has een well
recogni2ed! %ts mechanism is unknown and its presence does not have prognostic signiicance!
$hysical "7amination
A meticulous e!amination of all lymph node'"earin$ sites is mandatory# Inaddition to the fre*uently encountered cerical and supraclaicular sites+ the a!illaeand $roins merit careful palpation# Splenome$aly is another fre*uent physical %ndin$in lymphomatous mali$nancies#
Hodgkins disease almost always presents with lympadenopathy and the involved nodes are usually
reely movale and have a ruery consistency! Cases in which the histology demonstrates irosis or
sclerosis can e associated with hard and irm adenopathy! .lthough any lymph node group can e
involved, cervical and supraclavicular adenopathy are the most common physical indings, although
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a7illary adenopathy is not rare! ediastinal disease is oten present, ut rarely is the only site o
involvement ecause it usually occurs in association with cervical and supraclavicular adenopathy! HD
can also present with iliac, inguinal and emoral adenopathy, and in appro7imately ; o cases, there
may e only sudiaphragmatic disease! +plenomegaly is noted at presentation in appro7imately &6; o
cases o HD! However, splenomegaly may occur as a nonspeciic maniestation o HD and in only hal o
the cases is there actual splenic involvement with HD ound at laparatomy! .dditionally, splenicinvolvement has een reported in 6; o HD cases in the asence o splenomegaly!
"7tranodal HD can occur at any site! 9ung, liver, one and one marrow are the most common sites o
e7tranodal involvement and each o these sites is seen in appro7imately :; to &6; o cases!
Hodgkins disease tends to spread in a contiguous ashion! or e7ample, ecause o contiguity with the
thoracic duct, let supraclavicular nodal involvement is oten seen in association with mediastinal
involvement!
.s is the case with Hodgkins disease, most patients with non-Hodgkins lymphoma present with painless
lymphadenopathy, most commonly in the cervical and supraclavicular regions! +ystemic symptoms occur
in less than :; o patients in most large series! Ahen present, they are usually associated with
advanced disease!
/astrointestinal lymphoma occurs in less than ; o NHL, with stomach and colon the most common
sites o involvement, and with vague adominal pain the most common presentation! Ostruction,
speciically intussusception in children, or peroration, may e complications o intestinal lymphoma!
/enitourinary presentations include renal mass, ureteral ostruction, testicular and ovarian masses and
vaginal leeding! =eurological signs and symptoms include headache, cranial nerve palsies and signs o
leukoencephalopathy! 8nusual metaolic presentations include hyperuricemia, renal ailure,
hypercalcemia and severe hypoglycemia!
Disease +taging
The symptoms and physical indings play key roles in the important process o the staging o Hodgkins
disease and non-Hodgkins lymphoma, a process which is o utmost value in the prognosis o the disease
and in the planning o a therapeutic program! +everal staging classiications have een proposed or oth
HD and NHL! Tale 5 presents the widely used Cotswald classiication or HD!
Tale 5-Cotswald +taging Classiication or Hodgkins Disease
The staging classiication o HD into . (asymptomatic) and B (symptomatic) has a relationship to
prognosis! The presence o symptoms is a suggestive indication o advanced disease! +ymptoms are
ound in ewer than &6; o patients with +tage % disease and in appro7imately ?:; o patients with +tage
%G disease!
The purpose o clinical evaluation is not only to determine the stage o the disease and the therapeutic
regimen, ut also to estalish an appropriate clinical aseline so that success or ailure o therapy can e
assessed!
Tale : shows the classiication o HD cases with respect to +tage and +ymptomatic +tatus!
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Tale :-Distriution o Hodgkins Disease Cases with 1espect to +tage and +ymptomatic +tatus
. numer o staging classiications have een proposed or the non-Hodgkins lymphomas! %n essence,
they parallel those o Hodgkins disease in respect to +tages % and %% with only minor modiications o
+tages IIIand, i present, +tage %G!
Testing $rocedures or HD and NHLOice and 9aoratory +tudies
Hod$(in)s disease and non'Hod$(in)s lymphoma patients are prone to deelop aariety of hematolo$ic and "lood chemistry a"normalities and "lood specimensshould "e &ithdra&n for appropriate "aseline alues#
. slight to moderate polymorphonuclear leukocytosis may e present! 9ymphocytopenia may occur early
and ecome pronounced with advancing disease! "osinophilia is present in aout 6; o patients and
thromocytosis may e oserved! .nemia, oten hypochromic and microcytic, usually develops with
advancing disease! Deective iron utili2ation is characteri2ed y low serum iron levels and low iron-
inding capacity! Hypersplenism may occur producing cytopenias alone or in any comination in the
presence o marked splenomegaly!
9ymphomatous iniltration o ones and liver is characteri2ed y elevations o serum levels o the
respective isoen2ymes o alkaline phosphatase! Other lood chemistry studies should include levels o
lood glucose, urea nitrogen and creatinine! +erum iliruin levels and measurements o hepatic
en2ymes are helpul in assessing liver involvement! +erum uric acid should e monitored especially
during therapy to detect increases that may predict hyperuricemic renal shutdown! +erum electrolytes
should also e checked to monitor luid and electrolyte alance in patients who are oten depleted y the
underlying disease as well as therapy!
+erum levels o lactic dehydrogenase (LDH) and eta- microgloulin (eta -) represent surrogate
3uantitative measures o metastatic potential and tumor urden respectively! They have een used as
indicators and serological staging agents to predict reedom rom relapse and survival! $atients at low risk
or disease recurrence have normal levels o oth markers whereas elevations predict shortened
remission and survival!
1adiologic +tudies
,adiolo$ic studies are particularly alua"le in assessin$ disease e!tent# Thespeci%c studies to "e performed &ill ary &ith the indiidual patient and depend onthe physician)s assessment of the li(elihood of di-use disease inolement#
. chest radiograph is indicated in all patients with HD and NHLprimarily to assess lung status and in
particular, mediastinal disease involvement! ost authorities also recommend computeri2ed tomographic
(CT) studies o the chest, adomen, pelvis and neck to assess the presence o disease not readilyassessale y physical e7amination! Other radiologic procedures are perormed as deemed clinically
appropriate and include gallium scanning, magnetic resonance imaging (M,I), ultrasound and one and
liver scans! 9ymphangiography is occasionally perormed when there is a strong suspicion o periaortic
and retroperitoneal nodal involvement!
%nvasive $rocedures
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The dia$nosis of "oth HD and NHL re*uires a "iopsy that contains su.cient tissueto permit an accurate microscopic dia$nosis "y an e!perienced hematopatholo$ist#Biopsy specimens are usually o"tained from palpa"le lymph nodes "ut mayoccasionally "e o"tained from other tissues# Needle aspirations or "iopsies areinade*uate for the primary histolo$ic dia$nosis#
. one marrow aspiration and iopsy should e perormed as part o the initial staging procedure
although these tests are rarely positive unless other evidence o advanced disease (at least +tage III) ispresent! The ma4ority o patients with one marrow involvement will e symptomatic< nevertheless, a
one marrow iopsy should e otained at aseline in all patients ecause the results may not only
inluence the use o initial therapy ut might aect uture decisions at the time o relapse (e!g!, a decision
on the easiility o an autologous one marrow transplantation)!
. ma4or controversy surround the perormance o a staging laparatomy! . staging laparoscopy can only
e 4ustiied when the results will inluence therapy! +ome physicians recommend the procedure whenever
therapeutic planning includes radiation! Others reserve it or those cases where clinical staging is
e3uivocal! Ahen perormed, it should include inspection o the liver and spleen and liver and nodal
iopsies!
Currently, laparoscopy is eing perormed ar less re3uently ecause chemotherapy is incorporated inmany clinical settings and ecause retrospective studies provide a asis or determining the likelihood o
intraadominal disease with dierent clinical perspectives! +everal studies have demonstrated that
laparotomy had no signiicant impact on survival or relapse rate in patients with a avorale prognosis
who were randomi2ed to staging with laparotomy or clinical staging! The risk o occult sudiaphragmatic
disease can e estimated on the asis o se7, age, erythrocyte sedimentation rate (ES,), numer o
involved lymph node sites, and histologic sutype! . one marrow iopsy is included in the routine
staging o patients who have advanced-stage disease, constitutional symptoms, or oth!
Dierential Diagnosis
The di-erential dia$nosis of "oth Hod$(in)s disease and non'Hod$(in)s lymphomainoles the rulin$ out of o"ious and common conditions as &ell as othermali$nancies# The e!act dia$nosis hin$es on the histolo$ical %ndin$s and the opinionof an e!perienced hematopatholo$ist#
%n patients with cervical adenopathy, inections, including acterial or viral pharyngitis, inectious
mononucleosis and histoplasmosis must e e7cluded! Other malignancies, such as nasopharyngeal and
thyroid cancers, can present with locali2ed cervical adenopathy and a7illary adenopathy is a common
metastatic maniestation o reast cancer! ediastinal lymphomatous involvement must e distinguished
rom inections, sarcoidosis and other thoracic neoplasms!
%n other patients, the dierential diagnosis includes tumors o the lung and mediastinum, speciically o
small cell and non-small cell type! 1eactive mediastinitis and hilar lymphadenopathy rom histoplasmosis
can e conused with lymphoma since it occurs in otherwise asymptomatic patients!
The pathologic diagnosis o HD has traditionally een considered to e easier and saer than that o the
more comple7 non-Hodgkins lymphomas! However, a &>?' review o the histopathologic material o ?66
patients in whom an initial diagnosis o HD had een made revealed a mistaken diagnosis in an
astonishing 5F; o cases! The most common error in pathological diagnosis was the conusion o
inlammatory or other non-neoplastic conditions with HD (;)! The high rate o error was attriuted to
the relative ine7perience o the contriuting general pathologists in the diagnosis o hematopathologic
malignancies! %n a study reported y the +outhwest Oncology /roup (S/01) in &>', the rate o
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misdiagnosis in :F: cases o HD was again high, ut the most common class o error was conusion o
HD with NHL! %n suse3uent similar studies o HD, the conusion with NHLwas always ound to e the
most common diagnostic error! .lthough the incidence o misdiagnosis has decreased, ma4or errors still
e7ist in up to &6; o cases!
The correct diagnosis o oth Hodgkins disease and non-Hodgkins lymphoma is completely dependent
on a correct interpretation o the histopathology o the iopsy material and it is mandatory that assistancein this area e otained rom e7perienced hematopathologists!
Treatment
The currently successul treatment o Hodgkins disease is one o the triumphs o modern cancer
therapeutics! The death rate due to Hodgkins disease has decreased y two-thirds in the last 56 years
ecause o the introduction o eective diagnostic and therapeutic modalities! .ll patients regardless o
the stage o the disease can and should e treated with curative intent!
The therapy o Hodgkins disease and non-Hodgkins lymphoma involves three main modalities
,adiation
2hemotherapy
2om"ination radiotherapy and chemotherapy
%n the case o Hodgkins disease, staging is critical in choosing the therapeutic modality! %n non-Hodgkins
lymphoma, the histologic tissue type, as well as staging, is a ma4or determinant o therapy!
1adiation Therapy
1adiation is the standard approved therapy in Hodgkins disease stages % and %%!
%n disease limited to lymph nodes aove the diaphragm, therapy is usually directed only to the involved
area (limited ield radiation)! Beore the advent o eective salvage chemotherapy, radiotherapy was oten
directed at oth involved and uninvolved lymphoid areas (e7tended ield radiation involving total or
sutotal lymphoid radiation)! The tendency today is to limit the ield o radiation to the *mantle0 o
involvement i the disease is conined to the upper chest (aove the carina) with a resultant cure rate in
e7cess o '6; (reaching as high as >; when causes o death other than Hodgkins disease are
e7cluded!
1adiotherapy alone remains an option in some patients with minimal splenic, splenic hilar or celiac node
involvement (stage IIIA)! %n these patients, it is possile to eect a cure in ?6; to F6; o patients using
total nodal irradiation, ut those with e7tensive splenic involvement will re3uire systemic therapy ecause
o the high rate o recurrence with radiation therapy alone! ost relapses (F:;) occur within the irst
years ater completing therapy< late relapses thereater are uncommon! There is a tendency to oer such
patients comined modality therapy ecause it is associated with the lowest recurrence rate!
.n alternative treatment applicale to early-stage disease is to use oth radiation and chemotherapy in
selected patients such as those with ulky mediastinal disease and signiicant B symptoms! Other
candidates are those at a high risk or sudiaphragmatic involvement, mi7ed cellularity or lymphocytic
depleted Hodgkins Disease histology, or over 56 years-old!
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The radiation dose re3uired to eradicate Hodgkins disease in demonstraly involved nodes is 56 to 5:
/y (& /y&66 rads)! . standard regimen is a total dose o ? /y delivered in 6 daily unctions o &!' /y
over a period o 5 weeks!
Chemotherapy
%t is important to note that more than hal o the patients with Hodgkins disease who e7perience relapse
ater radiation therapy alone have disease that is still curale with chemotherapy!
8ntil recently, M033(mechlorethamine, Oncovin, procara2ine and prednisone) was the
chemotherapeutic comination o choice ut is now used much less re3uently ecause o its side eects
including myelosuppression, susceptiility to inection, inertility and autonomic and peripheral
neuropathy!
The comination chemotherapy o choice today is ABVD(do7oruicin) I.driomycinJ, leomycin,
vincristine, and dacara2ine)! %t has een shown to e oth more eective as well as less to7ic
than M033! . cycle oABVDtherapy includes
.dvanced Hodgkins disease is classically deined as stages IIIBand %G! %n these stages, Hodgkinsdisease is curale only with a systemic approach! ABVDis the standard comination chemotherapy
generally administered or ? cycles! %t remains controversial whether post chemotherapy ad4uvant
radiotherapy improves the outcome in advance Hodgkins disease! % it is to e utili2ed, the radiotherapy
ield should e kept minimi2ed, doses kept low and sites to e eradicated limited to *ulky0 disease!
ABVDis generally well tolerated! %ts primary side eects are nausea, atigue, moderate hair loss, some
increased susceptiility to inections, neuropathy secondary to vincristine and pulmonary irosis
secondary to leomycin! $ulmonary unction should e monitored and leomycin withheld i there is a all
o :; or more in pulmonary diusion capacity! Do7oruicin cardioto7icity is rare< however, it is
recommended that all patients have let ventricular e4ection raction measured eore therapy! %nertility is
not a ma4or prolem with ABVDut all men should e advised to ank sperm! ABVDhad rarely een
administered during the second and third trimesters o pregnancy< no adverse eects have een noted inthe ew such cases where it was administered! .ll women o childearing age should have a pregnancy
test eore therapy and e advised to delay pregnancy or years ater treatment to avoid the
coincidence o pregnancy and relapse!
%n the case o non-Hodgkins lymphoma, the choice o therapy depends not only on the staging o the
disease, ut also on the histological classiication o the tumor!
9ow-grade lymphomas (indolent or avorale) are usually small lymphocytic or ollicular center
lymphomas! /rade % and %% low grade lymphomas are uncommonly diagnosed, ut may e curale with
involved ield radiation! One study reported a relapse-ree survival rate o ?; at : years in all patients
and '6; in &: years or later in patients 56 years o age or younger! /enerally, no eneit rom additional
ad4uvant chemotherapy has een documented!
8sually low-grade lymphomas are diagnosed in stages IIIor %G (F:; to >6;) and there is one marrow
involvement in appro7imately F6;! The median survival o this group is : to F years with an initial
response to radiotherapy or chemotherapy and then relapse eventually leading to death! +ome have
adopted a *watch and wait0 approach to this advanced stage, ut in one study, : year survival was only
&; with this approach compared to :&; or aggressive therapy!
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%ntermediate grade lymphomas are more aggressive than low-grade lymphomas with untreated survival
times measured in months rather than in years! The most common intermediate grade lymphomas are
the large cell lymphomas, which are curale in aout :6; o cases! ost are treated similarly, even those
with apparent stage % and %% disease ecause o the now-recogni2ed systemic nature o these stages!
1ecommended therapy consists o standard anthracycline comination chemotherapy, usually in the orm
o 2H03(cyclophosphamide, do7oruicin, vincristine, prednisone) administered in three cycles (2H03-)
and ollowed y radiotherapy! This regimen has produced a 5-year survival as high as >F; on stage %
patients less than ?6 years o age and with a normal perormance status! %n advanced (stage IIIor %G)
intermediate grade non-Hodgkins lymphoma, 2H03has een shown to produce long-term complete
remission rates o up to F:;!
High-grade lymphoma, including precursor lympholastic lymphoma, Burkitts lymphoma and Burkitts-like
lymphoma, tend to occur in younger age patients and have an untreated survival time usually measured
in weeks! They usually present with advanced stage disease and are always treated with systemic
chemotherapy! any have 2NSinvolvement and re3uire intrathecal therapy! 9ow risk patients, i!e!, those
with a serum LDLlevel less than 66 %8#9 and without one marrow or 2NSinvolvement, may have a :
year remission rate as high as >5;!
onoclonal antiody (.) therapy or non-Hodgkins lymphoma
+tudies have revealed that more than >6; o lymphomas o B-cell origin e7press the CD6 antigen, thus
identiying CD6 as a potential target or monoclonal antiody (.) therapy! %n &>'F, the irst such .,
ritu7ima, was approved y the 4DAor the treatment o patients with relapsed or reractory low-grade or
ollicular, CD-6 positive, B-cell non-Hodgkins lymphoma! This was ollowed y the approval o
tositumoma in &>>'!
1itu7ima is a mouse-human chimeric %g/&-kappa immunogloulin that selectively inds the CD6
antigen on lymphoma cells inducing complement-and antiody-dependent cytoto7icity! %t has also een
shown to induce apoptosis in the tumor cell! +everal phase % and phase %% trials suggest an appro7imate
:6; response rate in the therapy o indolent and relapsed or reractory low-grade or ollicular-B
lymphoma, ut imply no change in survival! +igniicantly lower response rates were seen in patients with
lymphocytic histology or in patients with one marrow involvement and with two or more e7tranodal sites
o disease!
1itu7ima was well tolerated in multiple inusions o F: mg#m! The most common adverse eects were
low-grade ever, chills, nausea and headache! These were not commonly seen ater the initial inusion
and were much less re3uent or asent with suse3uent inusions! The o4ective o multiple clinical trials
in progress is to incorporate . therapy in primary management strategies in comination with other
cytoto7ic therapies! .nti-CD6 therapy has produced avorale results when used in comination with
chemotherapy! %n one trial, patients with newly diagnosed or relapsed#reractory CD6-positive low-grade
or ollicular lymphomas received ? cycles o ADV3(cyclophosphamide, do7oruicin, vincristine and
prednisone) administered every three weeks with ? inusions o ritu7ima! There was a complete
remission response rate o ::; and partial response rate o 56;! +everal studies have evaluated
ritu7ima in the treatment o aggressive non-Hodgkins lymphoma!
The results were signiicantly less avorale than those with indolent lymphoma with overall response
rates o 6; to 56;! The greater the tumor urden, the less was the response 1te with no response
seen in patients with tumors larger than &6 cm!
.nother aspect o . therapy is its in con4ugation with radioactive materials such as the radioisotopes
iodine-&& and yttrium->6! The ollicular lymphomas are e7tremely sensitive to irradiation and these
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agents have the advantage that the radiotherapy is targeted to tumor cells, therey limiting to7ic eects
on normal cells! 1esults have een encouraging!
Developing Therapies
+tem cells
. recent development has een the use o stem cell transplantation! +igniicantly higher response rates to
cytoto7ic agents have een reported in patients who had received prior stem cell transplantation than in
those who had not (F'; vs! 5;, respectively, pK6!6&!
Gaccines
The idiotype, the molecular determinant o the variale region o the surace immunogloulin, o a B-cell
lymphoma can unction as a tumor-speciic marker! %mmune responses against the idiotype have een
induced in patients y tailor-made vaccines! 9ymphoma vaccines are prepared y isolating the idiotypeprotein rom the lymphoma tissue, con4ugating it to a protein carrier and mi7ing it with an immunologic
ad4uvant in vitro!
Complications
"arly complications o Hodgkins disease and non-Hodgkins lymphoma are usually related to therapy
Complications o radiotherapy depend on the radiated volume o tissue, dose administered and the
techni3ue employed! The acute eects are usually mild and transient and may include dryness o the
mouth, changes in taste, pharyngitis, nausea, dry cough, dermatitis, and atigue! +i7 weeks to months
ater radiation therapy, patients may note an electric shock sensation in the lower e7tremities on le7ion othe neck! This so-called 9hermittes syndrome is related to transient demyelini2ation o the cervical spinal
cord! 1adiation pneumonitis and pericarditis are other rare delayed complications o radiotherapy!
.cute and suacute adverse eects to chemotherapy are airly common! %n act, M033therapy, which
was widely used up until recently, has een discontinued in many cancer clinics due to its association with
myelosuppression (particularly thromocytopenia), susceptiility to inections, inertility and autonomic
and peripheral neuropathy! The current *gold standard0 chemotherapy, ABVDis etter tolerated although
associated with hair loss, nausea and vomiting, atigue and myelosuppression (particularly neutropenia)!
Delayed and long-term complications include hypothyroidism secondary to thyroid gland radiation,
pericardial irosis, ocal carcinoma or sarcoma secondary to radiation therapy, sterility, anemia and
hematological complications o chemotherapy such as thromocytopenia! 9ate-appearing inections mayoccur due to the chronic state o immune depression ollowing therapy and patients who have had
splenectomy may have a predilection or acterial inections!
+pecial Considerations
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9ymphomatous malignancies occurring in children, the elderly, patients with HIVinection and during
pregnancy have special therapeutic needs! These patients should e reerred to health providers with
training and e7perience in these speciali2ed areas!
%n patients with low-grade lymphomas, age does not usually make a dierence in type o therapy or
prognosis and nonaggressive therapy is usually roken! However, in the treatment o diuse aggressive
lymphomas, age is an important prognostic actor! The +outhwest Oncology /roup (S/01) has reported
a complete response rate o ?:; and a median survival o &6& months in patients under 56 years-old in
comparison with a response rate o F; and a median survival o only &? months in patients over the age
o ?:! +imilarly, the "uropean Organi2ation or 1esearch and Treatment o Cancer 9ymphoma
Cooperative +tudy /roup reported superior results in elderly patients with the use o aggressive
chemotherapy (2H03) compared to the results otained with a less aggressive anthracycline regimen!
$atients with HIVinection also constitute a special group o lymphoma patients ecause o their
predilection or the development o non-Hodgkins lymphoma, usually o high-grade B-cell type
(immunolastic or small uncleaved cell types)< aout 6; unusual e7tranodal involvement! +tudies have
demonstrated that aggressive chemotherapy induces remissions and prolongs lie! However,
chemotherapy to7icity has een reported to shorten survival in these seriously ill patients!
Ahen to 1eeror the primary physician, determination o the presenting signs and symptoms should e ollowed y
iopsy o the most accessile tissue, usually a peripheral node! ollowing e7amination and the
determination o a deinitive diagnosis y an e7perienced hematopathologist, reerral to an oncologist is
advisale or recommendations or appropriate therapy! 9ymphomatous neoplasms occurring in children,
the elderly, during pregnancy or in patients with HIVinection will re3uire specialists e7perienced in those
ields and who are prepared to anticipate the increased demands that these special populations will
impose!
1adiotherapy re3uires the availaility o modern appropriate acilities and administration y trained
radiotherapists! Chemotherapeutic protocols are est administered y e7perienced medical oncologists!
$rognosis
.ll patients with oth locali2ed and disseminated Hodgkins disease should e treated with curative intent!
The prognosis o patients with stage %. and IIAdisease treated y radiotherapy is e7cellent with &6-year
survivals in e7cess o '6;! $atients with disseminated disease (IIIB, %G) have a :-year survival rates o
:6; to ?6;! $oorer results are seen in patients who are older, those who have ulky disease and those
with lymphocytic depletion or mi7ed cellularity on histologic e7amination! $atients whose disease recurs
ater radiation may still e curale with chemotherapy! The treatment o choice or patients who relapse
ater initial chemotherapy is high-dose chemotherapy with autologous stem cell transplantation! This
oers a :; to :6; chance o cure in patients whose disease is still chemotherapy sensitive! .lthough
early stage non-Hodgkins lymphoma share the same prognosis as Hodgkins disease patients, those with
more comple7 patterns are less well! The median survival o indolent lymphomas is ? to ' years! These
diseases ultimately ecome reractory to chemotherapy, oten associated with histologic progression o
the disease to a more aggressive orm!
The %nternational $rognostic %nde7 is now widely used to categori2e patients with intermediate grade
lymphomas into risk groups! actors that coner an adverse prognosis include
0er 56 years'old
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Eleated serum LDH
Adanced sta$e 7III or IV8 disease
3oor performance status
$atients with no or one risk actor may have a complete ('6;) response to standard chemotherapy and
most responses ('6;) are durale! $atients with two risk actors have a complete F; complete response
and F6; are long-lasting! $atients with higher risk disease have lower response rates and poor survival
with standard regimens o treatment and alternative therapies are needed! "arly treatment with high-dose
therapy and autologous stem cell transplantation improves the outcome! or patients who relapse ater
initial chemotherapy, the prognosis depends on whether the lymphoma is still partially sensitive to
chemotherapy! % so, autologous transplantation oers a :6; chance o long-term salvage!
$atient "ducation
$atients should e inormed o the nature o their disease, its course and prognosis! ost patients with
Hodgkins disease and non-Hodgkins lymphoma have some degree o amiliarity with the nature, course
and prognosis o their disease! .s with other conditions, this knowledge has een reinorced y the ready
availaility o medical inormation supplied y the %nternet and other sources!
The physician should independently provide the patient with accurate and actual inormation, correcting
any misconceptions the patient may have ac3uired rom other sources! The e7cellent prognosis o
Hodgkins disease and non-Hodgkins lymphoma and their actual curaility with todays therapeutic
armamentarium should e emphasi2ed! This will not alone maintain morale on the part o the patient, ut
also will support his#her compliance with the program!
.s with any disease, the physician should alert the patient to potential therapeutic side eects and
signiicant signs and symptoms that may herald a relapse!
ollow up
9ong term survivors o successul therapy should e ollowed or long-term! Complications such as
hypothyroidism, pericardial irosis, ocal carcinoma or sarcoma secondary to radiation therapy, sterility,
anemia and hematological complications o chemotherapy such as thromocytopenia! The chronic state
o immune depression ollowing therapy can result in late-appearing inections such as herpes 2oster!
$atients who have had splenectomy may have a predilection or acterial inections!
About The Brain
The brain is a sof, spongy mass o ssue. t is prote!ted by"
# The bones o the sku$$
# Three thin $ayers o ssue %meninges
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# 'atery (uid %!erebrospina$ (uid that (o)s through spa!es bet)een the meninges and through spa!es
%*entri!$es )ithin the brain
This pi!ture sho)s the brain and nearby stru!tures. %mage !ourtesy o the Naona$ +an!er nstute
This pi!ture sho)s the brain and nearby stru!tures. %mage !ourtesy o the Naona$ +an!er nstute
The brain dire!ts the things )e !hoose to do %$ike )a$king and ta$king and the things our body does
)ithout thinking %$ike breathing. The brain is a$so in !harge o our senses %sight, hearing, tou!h, taste,
and sme$$, memory, emoons, and persona$ity.
A net)ork o ner*es !arries messages ba!k and orth bet)een the brain and the rest o the body. omener*es go dire!t$y rom the brain to the eyes, ears, and other parts o the head. -ther ner*es run
through the spina$ !ord to !onne!t the brain )ith the other parts o the body. 'ithin the brain and
spina$ !ord, g$ia$ !e$$s surround ner*e !e$$s and ho$d them in p$a!e.
The three maor parts o the brain !ontro$ di/erent a!*ies"
# +erebrum" The !erebrum uses inormaon rom our senses to te$$ us )hat is going on around us and
te$$s our body ho) to respond. t !ontro$s reading, thinking, $earning, spee!h, and emoons. The
!erebrum is di*ided into the $ef and right !erebra$ hemispheres. The right hemisphere !ontro$s the
mus!$es on the $ef side o the body. The $ef hemisphere !ontro$s the mus!$es on the right side o the
body.
# +erebe$$um" The !erebe$$um !ontro$s ba$an!e or )a$king and standing, and other !omp$e0 a!ons.
# Brain stem" The brain stem !onne!ts the brain )ith the spina$ !ord. t !ontro$s breathing, body
temperature, b$ood pressure, and other basi! body un!ons.
This pi!ture sho)s the maor parts o the brain. %mage !ourtesy o the Naona$ +an!er nstute
This pi!ture sho)s the maor parts o the brain. %mage !ourtesy o the Naona$ +an!er nstute
Tumor 1rades and Types
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'hen most norma$ !e$$s gro) o$d or get damaged, they die, and ne) !e$$s take their p$a!e. omemes,
this pro!ess goes )rong. Ne) !e$$s orm )hen the body doesn2t need them, and o$d or damaged !e$$s
don2t die as they shou$d. The bui$dup o e0tra !e$$s ofen orms a mass o ssue !a$$ed a gro)th or tumor.
3rimary brain tumors !an be benign or ma$ignant"
Benign brain tumors do not !ontain !an!er !e$$s"
# 4sua$$y, benign tumors !an be remo*ed, and they se$dom gro) ba!k.
# Benign brain tumors usua$$y ha*e an ob*ious border or edge. +e$$s rom benign tumors rare$y in*ade
ssues around them. They don2t spread to other parts o the body. Ho)e*er, benign tumors !an press on
sensi*e areas o the brain and !ause serious hea$th prob$ems.
# 4n$ike benign tumors in most other parts o the body, benign brain tumors are somemes $ie
threatening.
# Benign brain tumors may be!ome ma$ignant.
5a$ignant brain tumors %a$so !a$$ed brain !an!er !ontain !an!er !e$$s"
# 5a$ignant brain tumors are genera$$y more serious and ofen are a threat to $ie.
# They are $ike$y to gro) rapid$y and !ro)d or in*ade the nearby hea$thy brain ssue.
# +an!er !e$$s may break a)ay rom ma$ignant brain tumors and spread to other parts o the brain or to
the spina$ !ord.
# They rare$y spread to other parts o the body.
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Tumor 1rade
Do!tors group brain tumors by grade. The grade o a tumor reers to the )ay the !e$$s $ook under a
mi!ros!ope"
# 1rade " The ssue is benign. The !e$$s $ook near$y $ike norma$ brain !e$$s, and they gro) s$o)$y.
# 1rade " The ssue is ma$ignant. The !e$$s $ook $ess $ike norma$ !e$$s than do the !e$$s in a 1rade
tumor.
# 1rade " The ma$ignant ssue has !e$$s that $ook *ery di/erent rom norma$ !e$$s. The abnorma$ !e$$s
are a!*e$y gro)ing %anap$as!.
# 1rade 6" The ma$ignant ssue has !e$$s that $ook most abnorma$ and tend to gro) 7ui!k$y.
+e$$s rom $o)#grade tumors %grades and $ook more norma$ and genera$$y gro) more s$o)$y than
!e$$s rom high#grade tumors %grades and 6.
-*er me, a $o)#grade tumor may be!ome a highgrade tumor. Ho)e*er, the !hange to a high#grade
tumor happens more ofen among adu$ts than !hi$dren.
Types o 3rimary Brain Tumors
There are many types o primary brain tumors. 3rimary brain tumors are named a!!ording to the type o
!e$$s or the part o the brain in )hi!h they begin. 8or e0amp$e, most primary brain tumors begin in g$ia$
!e$$s. This type o tumor is !a$$ed a g$ioma.
Among adu$ts, the most !ommon types are"
Astro!ytoma" The tumor arises rom star#shaped g$ia$ !e$$s !a$$ed astro!ytes. t !an be any grade. n
adu$ts, an astro!ytoma most ofen arises in the !erebrum.
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# 1rade or astro!ytoma" t may be !a$$ed a $o)#grade g$ioma.
# 1rade astro!ytoma" t2s somemes !a$$ed a high#grade or an anap$as! astro!ytoma.
# 1rade 6 astro!ytoma" t may be !a$$ed a g$iob$astoma or ma$ignant astro!y! g$ioma.
5eningioma" The tumor arises in the meninges. t !an be grade , , or . t2s usua$$y benign %grade
and gro)s s$o)$y.
-$igodendrog$ioma" The tumor arises rom !e$$s that make the a9y substan!e that !o*ers and prote!ts
ner*es. t usua$$y o!!urs in the !erebrum. t2s most !ommon in midd$e#aged adu$ts. t !an be grade or
.
Among !hi$dren, the most !ommon types are"
5edu$$ob$astoma" The tumor usua$$y arises in the !erebe$$um. t2s somemes !a$$ed a primi*e
neuroe!toderma$ tumor. t is grade 6.
1rade or astro!ytoma" n !hi$dren, this $o)grade tumor o!!urs any)here in the brain. The most
!ommon astro!ytoma among !hi$dren is u*eni$e pi$o!y! astro!ytoma. t2s grade .
:pendymoma" The tumor arises rom !e$$s that $ine the *entri!$es or the !entra$ !ana$ o the spina$ !ord.
t2s most !ommon$y ound in !hi$dren and young adu$ts. t !an be grade , , or .
Brain stem g$ioma" The tumor o!!urs in the $o)est part o the brain. t !an be a $o)#grade or high#grade
tumor. The most !ommon type is di/use intrinsi! ponne g$ioma.
;ou !an
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'hen you2re to$d that you ha*e a brain tumor, it2s natura$ to )onder )hat may ha*e !aused your
disease. But no one kno)s the e0a!t !auses o brain tumors. Do!tors se$dom kno) )hy one person
de*e$ops a brain tumor and another doesn2t. esear!hers are studying )hether peop$e )ith !ertain risk
a!tors are more $ike$y than others to de*e$op a brain tumor. A risk a!tor is something that may in!rease
the !han!e o geGng a disease. tudies ha*e ound the o$$o)ing risk a!tors or brain tumors"
oniing radiaon" oniing radiaon rom high dose 0#rays %su!h as radiaon therapy rom a $arge
ma!hine aimed at the head and other sour!es !an !ause !e$$ damage that $eads to a tumor. 3eop$e
e0posed to ioniing radiaon may ha*e an in!reased risk o a brain tumor, su!h as meningioma or
g$ioma.
8ami$y history" t is rare or brain tumors to run in a ami$y. -n$y a *ery sma$$ number o ami$ies ha*e
se*era$ members )ith brain tumors.
esear!hers are studying )hether using !e$$ phones, ha*ing had a head inury, or ha*ing been e0posed to
!ertain !hemi!a$s at )ork or to magne!
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+hanges in mood, persona$ity, or abi$ity to !on!entrate
3rob$ems )ith memory
5us!$e erking or t)it!hing %seiures or !on*u$sions
Numbness or ng$ing in the arms or $egs
5ost ofen, these symptoms are not due to a brain tumor. Another hea$th prob$em !ou$d !ause them.
you ha*e any o these symptoms, you shou$d te$$ your do!tor so that prob$ems !an be diagnosed and
treated.
Diagnosis
you ha*e symptoms that suggest a brain tumor, your do!tor )i$$ gi*e you a physi!a$ e0am and ask
about your persona$ and ami$y hea$th history. ;ou may ha*e one or more o the o$$o)ing tests"
Neuro$ogi! e0am" ;our do!tor !he!ks your *ision, hearing, a$ertness, mus!$e strength, !oordinaon, and
re(e0es. ;our do!tor a$so e0amines your eyes to $ook or s)e$$ing !aused by a tumor pressing on the
ner*e that !onne!ts the eye and the brain.
5" A $arge ma!hine )ith a strong magnet $inked to a !omputer is used to make detai$ed pi!tures o
areas inside your head. omemes a spe!ia$ dye %!ontrast materia$ is ine!ted into a b$ood *esse$ in your
arm or hand to he$p sho) di/eren!es in the ssues o the brain. The pi!tures !an sho) abnorma$ areas,
su!h as a tumor.
+T s!an" An 0#ray ma!hine $inked to a !omputer takes a series o detai$ed pi!tures o your head. ;ou may
re!ei*e !ontrast materia$ by ine!on into a b$ood *esse$ in your arm or hand. The !ontrast materia$makes abnorma$ areas easier to see. ;our do!tor may ask or other tests"
Angiogram" Dye ine!ted into the b$oodstream makes b$ood *esse$s in the brain sho) up on an 0#ray. a
tumor is present, the 0#ray may sho) the tumor or b$ood *esse$s that are eeding into the tumor.
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pina$ tap" ;our do!tor may remo*e a samp$e o !erebrospina$ (uid %the (uid that
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# do ha*e a brain tumor, )ho )i$$ ta$k )ith me about treatmentI 'henI
Treatments
3eop$e )ith brain tumors ha*e se*era$ treatment opons. The opons are surgery, radiaon therapy, and
!hemotherapy. 5any peop$e get a !ombinaon o treatments. The !hoi!e o treatment depends main$y
on the o$$o)ing"
# The type and grade o brain tumor
# ts $o!aon in the brain
# ts sie
# ;our age and genera$ hea$th
8or some types o brain !an!er, the do!tor a$so needs to kno) )hether !an!er !e$$s )ere ound in the
!erebrospina$ (uid.
;our do!tor !an des!ribe your treatment !hoi!es, the e0pe!ted resu$ts, and the possib$e side e/e!ts.
Be!ause !an!er therapy ofen damages hea$thy !e$$s and ssues, side e/e!ts are !ommon. Beore
treatment starts, ask your hea$th !are team about possib$e side e/e!ts and ho) treatment may !hange
your norma$ a!*ies. ;ou and your hea$th !are team !an )ork together to de*e$op a treatment p$an
that meets your medi!a$ and persona$ needs.
;ou may )ant to ta$k )ith your do!tor about taking part in a !$ini!a$ tria$, a resear!h study o ne)
treatment methods.
;our do!tor may reer you to a spe!ia$ist, or you may ask or a reerra$. pe!ia$ists )ho treat brain tumors
in!$ude neuro$ogists, neurosurgeons, neuro#on!o$ogists, medi!a$ on!o$ogists, radiaon on!o$ogists, and
neuroradio$ogists. ;our hea$th !are team may a$so in!$ude an on!o$ogy nurse, a registered diean, a
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menta$ hea$th !ounse$or, a so!ia$ )orker, a physi!a$ therapist, an o!!upaona$ therapist, a spee!h
therapist, and a physi!a$ medi!ine spe!ia$ist.
;ou may )ant to ask your do!tor these 7uesons beore you begin treatment"
# 'hat type o brain tumor do ha*eI
# s it benign or ma$ignantI
# 'hat is the grade o the tumorI
# 'hat are my treatment !hoi!esI 'hi!h do you re!ommend or meI 'hyI
# 'hat are the e0pe!ted bene
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# Ho) ofen shou$d ha*e !he!kupsI
urgery
urgery is the usua$
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prob$ems $essen or disappear )ith me. But somemes damage to the brain is permanent. ;ou may
need physi!a$ therapy, spee!h therapy, or o!!upaona$ therapy.
;ou may )ant to ask your do!tor these 7uesons about surgery"
# Do you suggest surgery or meI
# Ho) )i$$ ee$ afer the operaonI
# 'hat )i$$ you do or me i ha*e painI
# Ho) $ong )i$$ be in the hospita$I
# 'i$$ ha*e any $ong#term e/e!tsI 'i$$ my hair gro) ba!kI Are there any side e/e!ts rom using meta$
or abri! to rep$a!e the bone in the sku$$I
# 'hen !an get ba!k to my norma$ a!*iesI
# 'hat is my !han!e o a u$$ re!o*eryI
adiaon Therapy
adiaon therapy ki$$s brain tumor !e$$s )ith high#energy 0#rays, gamma rays, or protons. adiaon
therapy usua$$y o$$o)s surgery. The radiaon ki$$s tumor !e$$s that may remain in the area. omemes,
peop$e )ho !an2t ha*e surgery ha*e radiaon therapy instead.
Do!tors use e0terna$ and interna$ types o radiaon therapy to treat brain tumors"
:0terna$ radiaon therapy" ;ou2$$ go to a hospita$ or !$ini! or treatment. A $arge ma!hine outside the
body aims beams o radiaon at the head. Be!ause !an!er !e$$s may in*ade norma$ ssue around a
tumor, the radiaon may be aimed at the tumor and nearby brain ssue, or at the enre brain. ome
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peop$e need radiaon aimed at the spina$ !ord a$so. The treatment s!hedu$e depends on your age, and
the type and sie o the tumor. 8ra!onated e0terna$ beam therapy is the most !ommon method o
radiaon therapy used or peop$e )ith brain tumors. 1i*ing the tota$ dose o radiaon o*er se*era$
)eeks he$ps to prote!t hea$thy ssue in the area o the tumor. Treatments are usua$$y J days a )eek or
se*era$ )eeks. A typi!a$ *isit $asts $ess than an hour, and ea!h treatment takes on$y a e) minutes.
ome treatment !enters are studying other )ays o de$i*ering e0terna$ beam radiaon therapy"
ntensity#modu$ated radiaon therapy or E#dimensiona$ !onorma$ radiaon therapy" These types o
treatment use !omputers to more !$ose$y target the brain tumor to $essen the damage to hea$thy ssue.
3roton beam radiaon therapy" The sour!e o radiaon is protons rather than 0#rays. The do!tor aims
the proton beam at the tumor. The dose o radiaon to norma$ ssue rom a proton beam is $ess than
the dose rom an 0#ray beam.
tereota!! radiaon therapy" Narro) beams o 0#rays or gamma rays are dire!ted at the tumor rom
di/erent ang$es. 8or this pro!edure, you )ear a rigid head rame. The therapy may be gi*en during a
sing$e *isit %stereota!! radiosurgery or o*er se*era$ *isits.
nterna$ radiaon therapy %imp$ant radiaon therapy or bra!hytherapy" nterna$ radiaon isn2t
!ommon$y used or treang brain tumors and is under study. The radiaon !omes rom radioa!*e
materia$ usua$$y !ontained in *ery sma$$ imp$ants !a$$ed seeds. The seeds are p$a!ed inside the brain andgi*e o/ radiaon or months. They don2t need to be remo*ed on!e the radiaon is gone.
ome peop$e ha*e no or e) side e/e!ts afer treatment. are$y, peop$e may ha*e nausea or se*era$
hours afer e0terna$ radiaon therapy. The hea$th !are team !an suggest )ays to he$p you !ope )ith this
prob$em. adiaon therapy a$so may !ause you to be!ome *ery red )ith ea!h radiaon treatment.
esng is important, but do!tors usua$$y ad*ise peop$e to try to stay as a!*e as they !an.
A$so, e0terna$ radiaon therapy !ommon$y !auses hair $oss rom the part o the head that )as treated.Hair usua$$y gro)s ba!k )ithin a e) months. adiaon therapy a$so may make the skin on the s!a$p and
ears red, dry, and tender. The hea$th !are team !an suggest )ays to re$ie*e these prob$ems. omemes
radiaon therapy !auses brain ssue to s)e$$. ;ou may get a heada!he or ee$ pressure. The hea$th !are
team )at!hes or signs o this prob$em. They !an pro*ide medi!ine to redu!e the dis!omort. adiaon
somemes ki$$s hea$thy brain ssue.
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A$though rare, this side e/e!t !an !ause heada!hes, seiures, or e*en death.
adiaon may harm the pituitary g$and and other areas o the brain. 8or !hi$dren, this damage !ou$d
!ause $earning prob$ems or s$o) do)n gro)th and de*e$opment. n addion, radiaon in!reases the risk
o se!ondary tumors $ater in $ie.
;ou may )ant to ask your do!tor these 7uesons about radiaon therapy"
# 'hy do need this treatmentI
# 'hen )i$$ the treatments beginI 'hen )i$$ they endI
# Ho) )i$$ ee$ during therapyI Are there side e/e!tsI
# 'hat !an do to take !are o myse$ during therapyI
# Ho) )i$$ )e kno) i the radiaon is )orkingI
# 'i$$ be ab$e to !onnue my norma$ a!*ies during treatmentI
+hemotherapy
+hemotherapy, the use o drugs to ki$$ !an!er !e$$s, is somemes used to treat brain tumors. Drugs may
be gi*en in the o$$o)ing )ays"
By mouth or *ein %intra*enous" +hemotherapy may be gi*en during and afer radiaon therapy. The
drugs enter the b$oodstream and tra*e$ throughout the body. They may be gi*en in an outpaent part o
the hospita$, at the do!tor2s oK!e, or at home. are$y, you may need to stay in the hospita$. The side
e/e!ts o !hemotherapy depend main$y on )hi!h drugs are gi*en and ho) mu!h. +ommon side e/e!ts
in!$ude nausea and *oming, $oss o appete, heada!he, e*er and !hi$$s, and )eakness. the drugs
$o)er the $e*e$s o hea$thy b$ood !e$$s, you2re more $ike$y to get ine!ons, bruise or b$eed easi$y, and ee$
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*ery )eak and red. ;our hea$th !are team )i$$ !he!k or $o) $e*e$s o b$ood !e$$s. ome side e/e!ts may
be re$ie*ed )ith medi!ine.
n )aers that are put into the brain" 8or some adu$ts )ith high#grade g$ioma, the surgeon imp$ants
se*era$ )aers into the brain. :a!h )aer is about the sie o a dime. -*er se*era$ )eeks, the )aersdisso$*e, re$easing the drug into the brain. The drug ki$$s !an!er !e$$s. t may he$p pre*ent the tumor rom
returning in the brain afer surgery to remo*e the tumor. 3eop$e )ho re!ei*e an imp$ant %a )aer that
!ontains a drug are monitored by the hea$th !are team or signs o ine!on afer surgery. An ine!on
!an be treated )ith an anbio!.
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