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!.