CA & Benign Tumors - 2

8/3/2019 CA & Benign Tumors - 2

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Principles of

Treatment


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Treatment of children with canceris one of the most complexendeavors in pediatrics.

a. correct diagnosis (including subtype)

b. accurate and thorough staging of the extent ofdisease

c. determination of prognostic subgroup


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Team:

a. pediatric oncologists

b. Pathologists

c. Radiologists

d. Surgeons

e. Radiotherapists

f. Nurses


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g.various support staff :

nutritionists

social workers

psychologists,

pharmacistsother medical specialists

teachers trained to work withseriously ill children.


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DIAGNOSIS AND STAGING

Diagnostic imaging:

a. Evaluation pre and post treatment

b. Determine extent of diseasec. Appropriate therapy

d. Follow-up


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Example:

a. MRIb. CT

c. Ultrasonography

d. scintigraphy (nuclear medicine scans)

e. positron emission tomography

f. spectroscopy,


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DRUG MECHANIS

M OFACTION

INDICATION(

S)

ADVERSE REACTIONS MONITORY DRUG

LEVEL

DRUG LEVEL

COMMENTS

Methotrexate

Folic acidantagonist; inhibitsdihydrofol

atereductase

ALL non-Hodgkinlymphoma,osteosarco

ma,Hodgkinlymphoma,medulloblas toma

Myelosuppression,mucositis, stomatitis,dermatitis, hepatitis

Long Term:

Osteopenia and bonefractures

High dose:

Renal & CNS toxicity

IntrathecalAdministration:

Arachnoiditis,leukoencephalopathy,leukomyelopathy

Plasma levelsmust bemonitored withhigh-dose

therapy andwhen low dosesare administeredto patients withrenal dysfunctionand leucovorin

rescue appliedaccordingly

Systemicadministration may bePO, IM, or

IV;also maybe adminis-teredintrathe-cally

Common Chemotherapeutic Agents Used in Children


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DRUG MECHANISMOF ACTION

INDICATION(S)

ADVERSEREACTIONS

MONITORYDRUG LEVEL

DRUG LEVELCOMMENTS

6-Mercaptopurine(Purinethol)

Purineanalog;inhibitspurinesynthesis

ALL Myelosuppression, hepaticnecrosis,mucositis;allopurinolincreases toxicity

Therapeuticdrugmonitoringnot availableor indicated

Allopurinolinhibitsmetabolism

Cytarabine(Ara-C)

Pyrimidineanalog;inhibitsDNApolymerase

ALL, AML,non-Hodgkinlymphoma, Hodgkin

lymphoma

Nausea,vomiting,myelosuppression, conjunctivitis,mucositis,

central nervoussystemdysfunction


Systemicadministrationmay be Po, IM,or IV;may alsobe

administeredintrathecally


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DRUG MECHANISM OF

ACTION

INDICATION(S)

ADVERSE REACTIONS MONITORY DRUGLEVEL

DRUGLEVELCOMMENTS

Cyclophosphamide(Cytoxan)

Alkylatesguanine;inhibitsDNAsynthesis

ALL, non-Hodgkinlymphoma,Hodgkinlymphoma, softtissue sarcoma,Ewing sarcoma

Nausea, vomiting,myelosuppression,hemorrhagic cystitis,pulmonary fibrosis,inappropriate ADHsecretion, bladder cancer,anaphylaxis

Therapeuticdrugmonitoringnotavailable orindicated

Requireshepaticactivationand thuslesseffective inpresence of

liverdysfunction

Ifosfamide (Ifex)

Alkylatesguanine;inhibits

DNAsynthesis

Non-Hodgkinlymphoma,Wilms tumor,

sarcoma, germcell andtesticulartumors

myelosuppression, Nausea,vomiting hemorrhagiccystitis, pulmonary fibrosis,

inappropriate ADHsecretion, bladder cancer,central nervous systemdysfunction, cardiac toxicity,anaphylaxis

Therapeuticdrugmonitoring

notavailable orindicated


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DRUG MECHANISM OF

ACTION

INDICATION(S) ADVERSE REACTIONS MONITORYDRUG LEVEL

DRUGLEVELCOMMENTS

Doxorubicin(Adriamycin)anddaunorubicin(Cerubidine)

Binds toDNA,intercalation

ALL, AML,osteosarcoma, Ewingsarcoma,Hodgkinlymphoma,non-Hodgkin

lymphoma,neuroblastoma

Nausea, vomiting,cardiomyopathy, redurine, tissuenecrosis onextravasation,myelosuppression,conjunctivitis,

radiation dermatitis,arrhythmia


Dactinomycin

Binds toDNA,inhibitstranscription

Wilms tumor,rhabdomyosarcoma, Ewingsarcoma

Nausea, vomitingtissue necrosis onextravasation,myelosuppression,radiosensitizer,mucosal ulceration



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DRUG MECHANISM OFACTION

INDICATION(S)

ADVERSE REACTIONS MONITORYDRUG LEVEL

DRUGLEVELCOMMENTS

Bleomycin(Blenoxane)

Binds toDNAcleavesDNAstrands

Hodgkindisease, non-Hodgkinlymphoma,germ cell

tumors

Nausea, vomiting,pneumonitis,stomatitis, Raynaudphenomenon,pulmonary fibrosis,

dermatitis


L-Asparagin

ase

Depletionof L-

asparagine

ALL;AML,when used in

combinationwithasparaginase

Allergic reactionpancreatitis,

hyperglycemia,platelet dysfunctionand coagulopathy,encephalopathy

Therapeuticdrug

monitoringnot availableor indicated


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DRUG MECHANISM OF

ACTION

INDICATION(S) ADVERSEREACTIONS

MONITORYDRUG LEVEL

DRUGLEVELCOMMENTS

Vincristine(Oncovin)

Inhibitsmicrotubuleformation

ALL, non-Hodgkinlymphoma,Hodgkin disease,

Wilms tumor,Ewing sarcoma,

neuroblastomarhabdomyosarcoma

Local cellulitis,peripheralneuropathy,constipation, ileus,

jaw pain,inappropriate ADH

secretion, seizures,ptosis, minimalmyelosuppression


IVadministrationonly;must notbe

allowed toextravasate

Vinblastine

(Velban)

Inhibitsmicrotub

uleformation

Hodgkin disease;Langerhans'cell

histiocytosis

Local cellulitis,leukopenia


IVadministration only;must notbe allowedtoextravasate


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DRUG MECHANISM OFACTION

INDICATION(S)

ADVERSE REACTIONS MONITORYDRUG LEVEL

DRUGLEVELCOMMENTS

Etoposide(VePesid) Topoisomeraseinhibitor

ALL, non-Hodgkinlymphoma,germ celltumor

Nausea, vomiting,myelosuppression,secondary eukemia


Etretinate(Tegison)(vitamin Aanalog) andtretinoin

Enhancesnormaldifferentiation

Acuteprogranuloc

yticleukemia;neuroblastoma

Dry mouth, hair loss,pseudotumor cerebri,premature epiphysealclosure



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DRUG MECHANISM OF

ACTION

INDICATION(S)

ADVERSEREACTIONS

MONITORYDRUG LEVEL

DRUGLEVELCOMMENTS

Pegaspargase(Pegaspar)

Polyethylene glycolconjugateof L-asparagin

e

ALL Indicated forprolongedasparaginedepletion and forpatients with

allergy to L-asparaginase


Carmustine(nitrosourea)

Carbamylation ofDNA;inhibitsDNAsynthesis

CNStumors,non-Hodgkinlymphoma,Hodgkindisease

Nausea, vomiting,delayedmyelosuppression(46 wk);pulmonary fibrosis,carcinogenicstomatitis


Phenobarbitalincreasesmetabolism,decreases activity


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Infectious Complications of Malignancy

PREDISPOSINGFACTOR

ETIOLOGY SITE OFINFECTION

INFECTIOUS AGENTS

Splenectomy Staging of

Hodgkindisease

Sepsis,

shock,meningitis

Pneumococcus, Haemophilus

influenzae, meningococcus

Indwellingcentral venous

catheter

Nutrition,administration

ofchemotherapy

Line sepsis,tract of

tunnel, exitsite

S. epidermidis, S. aureus,Candida albicans, P. aeruginosa,

Aspergillus, CorynebacteriumJK, Streptococcus faecalis,Mycobacterium fortuitum,Propionibacterium acnes


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The Leukemias


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group of malignant diseases in which

genetic abnormalities in a

hematopoietic cell give rise to an

unregulated clonal proliferation of

cells.


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The progeny of these cells have a growth

advantage over normal cellular elements,because of their increased rate ofproliferation, and a decreased rate ofspontaneous apoptosis.

The result is a disruption of normal

marrow function and, ultimately, marrowfailure.


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Leukemias: Most common malignant neoplasms inchildhood,

41% of all malignancies that occur in children


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Acute Lymphoblastic

Leukemia


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EPIDEMIOLOGY

USA: Approx 2,000 children


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Among identical twins:

risk to the second twin if one develops leukemiais greater than that in the general population

risk is >70% if the first twin is diagnosed duringthe first year of life and the twins shared the same

(monochorionic) placenta


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ETIOLOGY

In virtually all cases, the etiology of ALL isunknown

several genetic and environmental factorsare associated with childhood leukemia


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Factors Predisposing to Childhood

LeukemiaGENETIC CONDITIONS

Down syndrome

Fanconi syndrome

Bloom syndrome

Diamond-Blackfan anemia

Schwachman syndrome

Klinefelter syndrome

Turner syndrome

Neurofibromatosis type 1 Ataxia-telangiectasia Severe combined immune deficiency

Paroxysmal nocturnal hemoglobinuria

Li-Fraumeni syndrome


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ENVIRONMENTAL FACTORS Ionizing radiation

Drugs

Alkylating agents

Nitrosourea

Epipodophyllotoxin Benzene exposure

Advanced maternal age


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CLINICAL MANIFESTATIONS

Initially: nonspecific and relatively brief.

Anorexia

FatigueIrritability

intermittent, low-grade fever

joint pain:lower extremities

URTI in the preceding 12 mo.


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As the disease progresses:

signs and symptoms of bone marrowfailure:

pallor

fatiguebruising

epistaxis

fever


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Physical examination

Bone Marrow Failure: Pallor

Listlessness

purpuric and petechial skin lesions

mucous membrane hemorrhage


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The proliferative nature:

Lymphadenopathy Splenomegaly

Hepatomegaly less common

bone or joint pain-

tenderness over the bone or objective evidence ofjoint swelling and effusion

with marrow involvement--deep bone pain may be

present but tenderness will not be elicited


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DIAGNOSIS

The diagnosis of ALL is stronglysuggested by peripheral bloodfindings indicative of bonemarrow failure.


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Anemia and thrombocytopenia are seen inmost patients.

Leukemic cells often are not observed in theperipheral blood in routine laboratory

examinations.Many patients with ALL present with total

leukocyte counts of


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Bone marrow aspiration alone usually is

sufficient But sometimes a bone marrow biopsy

---is needed to provide adequate tissue

for study or to exclude other possiblecauses of bone marrow failure


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ALL is diagnosed by a bone marrowevaluation that demonstrates >25% of thebone marrow cells as a homogeneouspopulation of lymphoblasts.


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Staging of ALL

- based partly on a cerebrospinal fluid

(CSF) examination

If lymphoblasts are found and the CSFleukocyte count is elevated, overt CNS ormeningeal leukemia is present.

This finding reflects a worse stage and

indicates the need for additional CNS andsystemic therapies


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TREATMENT

single most important prognostic

factor in ALL is the treatment:without effective therapy, the

disease is fatal


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Predictive Factors

age of the patient at the time ofdiagnosis

initial leukocyte count

speed of response to treatment

(i.e., how rapidly the leukemic cells can be clearedfrom the marrow or peripheral blood).


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Average Risk: age between 110 yr

leukocyte count of 10 yr of age

initial leukocyte count of >50,000/L


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Therapy

Remission Induction:therapy designed to eradicate the

leukemic cells from the bone marrow

therapy usually is given for 4 wk:a. vincristine weeklyb. corticosteroid such as dexamethasone or prednisone

c. repeated doses of native L-asparaginaseor single dose of a long-acting, pegylated asparaginasepreparation.

d. Intrathecal cytarabine or methotrexate, or both, also may begiven.


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Patients with higher risk:

daunomycin at weekly intervalsWith this approach, 98% of patients are in

remission

Remission:


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CNS therapy

to prevent later CNS relapses

Intrathecal chemotherapy is given repeatedly bylumbar puncture in conjunction with intensivesystemic chemotherapy.

CNS relapse is thereby reduced to


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Features that predict a high risk of CNS

relapse: patients who, at the time of diagnosis, have lymphoblastsin the CSF

either an elevated CSF leukocyte count or physical signs

of CNS leukemia, such as cranial nerve palsy.

CNS relapse may receive irradiation to the brain andspinal cord irradiation.


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Testicular relapse occurs in 12% of boys with ALL, usually after

completion of therapy. relapse presents as painless swelling of one or

both testes.

diagnosis is confirmed by biopsy of the affectedtestis

Treatment

a. systemic chemotherapyb. local irradiation.


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maintenance phase therapy: lasts for 23 yr,

depending on the protocol used.

After remission has been induced

many regimens provide 1428 wk of multi-agenttherapy

with the drugs and schedules used varying dependingon the risk group of the patient

daily mercaptopurineweekly methotrexate, usually with intermittent doses

of vincristine

corticosteroid.

Maintenance phase


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delayed intensification

approximately 57 mo after the beginning of therapy,and after a relatively nontoxic phase of treatment(interim maintenance) to allow recovery from the initial

intensive therapy.


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PROGNOSIS

Most children with ALL can now beexpected to have long-term survival

survival rate >80% at 5 yr fromdiagnosis


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Type of treatment chosen according

a. type of ALL

b. stage of diseasec. age of the patient

d. rate of response to initial therapy

Favorable: patient responds in


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Unfavorable: age 10 yr at diagnosis

leukocyte count of >100,000/L at diagnosis

slow response to initial therapy

Chromosomal abnormalities

Hypodiploidy

Philadelphia chromosome

MLL gene rearrangements and translocations[t(1:19) or t(4;11)], portend a poorer outcome.


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Acute Myelogenous

Leukemia


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EPIDEMIOLOGY

accounts for 11% of the cases of childhoodleukemia in the USA

approximately 370 children diagnosedwith AML annually.


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Several chromosomal abnormalitiesassociated with AML have beenidentified, but no predisposing genetic

or environmental factors can beidentified in most patients


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PATHOGENESIS

Characteristic feature of AML >30% of bone marrow cells on bone marrow aspiration or

biopsy touch preparations constitute a fairly

homogeneous population of blast cells

with features similar to those that characterize earlydifferentiation states of the myeloid-monocyte-

megakaryocyte series of blood cells..


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French-American-British (FAB) Classification ofAcute Myelogenous Leukemia

SUBTYPECOMMON NAME

M1 Acute myeloblastic leukemia without maturation

M2 Acute myeloblastic leukemia with maturation

M3 Acute promyeloblastic leukemia

M4 Acute myelomonocytic leukemia

M5 Acute monocytic leukemia

M6 Erythroleukemia

M7 Acute megakaryocytic leukemia


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subcutaneous nodules or blueberry muffinlesions

infiltration of the gingiva

disseminated intravascular coagulation

discrete masses

chloromas or granulocytic sarcomas

CNS symptoms


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DIAGNOSIS

Analysis of bone marrow aspiration and biopsy

specimen--

features of a hypercellular marrow consisting of a

rather monotonous pattern of cells with features

that permit FAB subclassification of disease


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Special stains assist in identification ofmyeloperoxidase-containing cells-

confirming the origin of the leukemia and

the diagnosis.


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TREATMENT

Matched-sibling bone marrow or stem celltransplantation after remission has been shown toachieve long-term disease-free survival in 6070%of patients.

Continued chemotherapy for patients who do nothave a matched donor is less effective than

marrow transplantation but, nevertheless, iscurative in some patients.

CHROM ALT GENES USUAL PROGNOSI RECOMD TREATMENT


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ABN MORPH S

t(8;21) AML1-ETO FAB AML-M2 Favorable Intensive chemotherapy, includinghigh-dose cytarabine

inv(16),t(16;16)

CBFB-MYHIIFAB AML-M4Eo Favorable Intensive chemotherapy, includinghigh-dose cytarabine

t(15;17) PML-RARA FAB AML-M3 Favorable Intensive chemotherapy, includingATRA and anthracyclines

t(11;17) PLZF-RARA FAB AML-M3 Favorable Intensive chemotherapy, includingATRA and anthracyclines

11q23 abn. MLLrearrangements

FAB AML-M4or AML-M5

Unavorable Intensive chemotherapy, with high-

dose cytarabine and MRD HSCT

t(3;v) EVI1 MDS/AML Unavorable Intensive chemotherapy with orwithout HSCT

t(3;5) NPM-MLF MDS/AMS Unavorable Intensive chemotherapy with orwithout HSCT

del(7q), -7

Unknown MDS/FABAML-M0

Unavorable Intensive chemotherapy with or

without HSCT

del(5q), -

5

Unknown MDS/FAB

AML-M0

Unavorable Intensive chemotherapy with or

without HSCT


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Lymphoma


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Lymphoma is the third most commoncancer among children in the USA

annual incidence of 15 per million children

14 yr of age

Two categories of lymphoma

a. hodgkin disease (HD)

b. non-Hodgkin lymphoma (NHL)


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Hodgkin Disease

malignant process of the lymphoreticularsystem that constitutes 6% of childhood cancers

USA:about 5% of cancers in persons 14 yr of age

about 15% in persons 1519 yr of age

rare in children


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EPIDEMIOLOGY bimodal with regard to age early peak occurs in the middle to late 20s

second peak after 50 yr of age

developing countriesearly peak occurs before adolescence

male: female ratio

4 : 1 for children 37 yr of age

3 : 1 for children 79 yr of age

1.3 : 1 for children >10 yr of age


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infectious agents may be involved

a. human herpesvirus 6b. cytomegalovirus

c. Epstein-Barr virus (EBV)

Immunodeficiency

congenital- ataxia-telangiectasia,

acquired- HIV infection


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ETIOLOGY

Reed-Sternberg cell

large cell (1545 m in diameter) with multiple

or multilobulated nuclei hallmark of HD

arises from the germinal center B cells.


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RYE CLASSIFICATION

Lymphocyte predominanceaffects 1015% of patients

common among male and younger patients

presents as localized disease

Mixed cellularity

observed in 30% of patients

more common among children 10 yr of age

often presents as advanced disease with extranodalextension


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Nodular sclerosis

NS is the most common subtype

affecting 40% of younger patients and 70% ofadolescents

Lymphocyte depletionrare in children

common in patients with HIV infection


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NEW WHO/REAL CLASSIFICATION

Nodular lymphocyte predominance Classical Hodgkin lymphoma

Lymphocyte rich

Mixed cellularity Nodular sclerosis

Lymphocyte depletion

Anaplastic large cell lymphoma Hodgkin-like


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HD appears to arise in lymphoid tissue and

spreads to adjacent lymph node areas in arelatively orderly fashion

Hematogenous spread also occurs, leadingto involvement of the liver, spleen, bone,bone marrow, or brain, and usually is

associated with systemic symptoms


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painless, non-tender, firm, rubbery, cervical orsupraclavicular lymphadenopathy

affected lymph nodes are firmer than inflammatorynodes

Signs & symptoms of airway obstruction

(dyspnea, hypoxia, cough)

pleural or pericardial effusion

hepatocellular dysfunction bone marrow infiltration

(anemia, neutropenia, or thrombocytopenia)


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Systemic symptoms classified as B symptoms:

a. unexplained fever >39C

b. weight loss >10% total body weight over 3 mo

c. drenching night sweatsd. some present as a fever of unknown origin.


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Less common and not considered of

prognostic significance: Pruritus

Lethargy

Anorexia pain that worsens after ingestion of alcohol.


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Immune system abnormalities

a. anergy to delayed-hypersensitivity skin tests

b. abnormal cellular immune response

c. slightly decreased CD4:CD8 ratiod. reduced natural killer cell cytotoxicity


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DIAGNOSIS

Any patient with persistent, unexplainedlymphadenopathy unassociated with anobvious underlying inflammatory orinfectious process should have a chestradiograph to identify the presence of amediastinal mass before undergoing node

biopsy


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Evaluation includes History

physical examination

imaging studies

chest radiograph

CT scans of the chestabdomen and pelvis

gallium scan

positron emission tomography (PET) scan.

L b t t di


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Laboratory studies

complete blood cell count (CBC

- to identify abnormalities that might suggestmarrow involvement

erythrocyte sedimentation rate (ESR)

serum copper and serum ferritin levels

-if abnormal at diagnosis, serve as a baseline toevaluate the effects of treatment

Li f ti t t


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Liver function tests

-influence treatment and treatment complications

chest radiograph

- for measuring the size of the mediastinal mass inrelation to the maximal diameter of the thorax

Chest CT

-defines the extent of a mediastinal mass if present

- identifies hilar nodes and pulmonary parenchymalinvolvement, which may not be evident on chestradiographs

Abdominal CT or MRI


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Abdominal CT or MRI

-identify gross subdiaphragmatic involvement of nodesand enlargement and defects in the liver and spleen

Bone marrow aspiration and biopsy

performed with advanced disease (stage III or IV) or B

symptoms (fever, weight loss, night sweats)

Bone scans

performed in patients with bone pain and/or elevatedalkaline phosphatase

ll


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Gallium-67 scan

- helpful in identifying areas of increased uptake, which

can then be re-evaluated at the end of treatment- especially in patients with mediastinal masses that donot resolve completely on chest radiographs or CT

Fluorodeoxyglucose (FDG)-PET

- has advantages over gallium-67 scanning because thescan is a 1-day procedure

- higher resolution, better dosimetry, and less intestinalactivity

-greater quantitation potential

Ann Arbor Staging Classification for Hodgkin


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Ann Arbor Staging Classification for Hodgkin

Disease

STAGE DEFINITIONI Involvement of a single lymph node (1) or of a

single extralymphatic organ or site (IE)II Involvement of two or more lymph node regions

on the same side of the diaphragm (II) or localizedinvolvement of an extralymphatic organ or site and one or morelymph node regions on the same side of the diaphragm (IIE)

III Involvement of lymph node regions on both sides ofthe diaphragm (III), which may be accompanied by involvementof the spleen (IIIS) or by localized involvement of an

extralymphatic organ or site (IIIE) or both (IIISE)

IV Diffuse or disseminated involvement of one or moreextralymphatic organs or tissues with or without associatedlymph node involvement


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STAGE DEFINITION

I Involvement of a single lymph node (1) or of a singleextralymphatic organ or site (IE)

II Involvement of two or more lymph node regions on the same sideof the diaphragm (II) or localized involvement of anextralymphatic organ or site and one or more lymph node regions

on the same side of the diaphragm (IIE)

III Involvement of lymph node regions on both sides of thediaphragm (III), which may be accompanied by involvement ofthe spleen (IIIS) or by localized involvement of an extralymphaticorgan or site (IIIE) or both (IIISE)

IV Diffuse or disseminated involvement of one or moreextralymphatic organs or tissues with or without associated lymphnode involvement

TREATMENT


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TREATMENT Chemotherapy and radiation therapy are effective in the

treatment of HD.

Pediatric patients

- combined chemotherapy with or without low-dose

involved field radiation therapy. determined largely by:

disease stage

age at diagnosis

presence or absence of B symptoms

presence of hilar lymphadenopathy or bulky nodaldisease.

Radiation therapy alone


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Radiation therapy alone

standard doses of 3,5004,000 cGy, initially

resulted in prolonged remissioncure rates of 4095% in patients with surgically low-staged HD

This treatment approach resulted in significant long-term morbidity in pediatric patients:

growth retardation

thyroid dysfunction cardiac and pulmonary toxicity


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The MOPP regimen(mechlorethamine [nitrogen mustard], vincristine,procarbazine, and prednisone)

introduced in 1964 was the first combinationchemotherapy

complete response rate of 7080% cure rate of 4050% at 10 yr in patients with advanced

stage disease

Associated with significant acute and long-term

toxicity.


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COPP/ABV

(cyclophosphamide, vincristine, procarbazine,prednisone/doxorubicin, bleomycin andvinblastine)

minimum of six cycles of chemotherapy

cumulative toxicity

second malignancies

sterility

cardiac and pulmonary dysfunction


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RELAPSE

occur within the first 3 yr from diagnosis

as late as 10 yr have been reported

Poor prognostic features

tumor bulk

stage at diagnosis

presence of B symptoms.


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Patients who never achieve remission or relapse


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PROGNOSIS

patients with favorable prognostic factors andearly-stage disease have

a. event-free survival (EFS) of 8590%

b. overall survival (OS) at 5 yr of 95%.

Patients with advanced stage disease have an EFS

and OS of 8085% and 90%, respectively


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Prognosis after relapse depends

a. time from completion of treatment to recurrenceb. site of relapse (nodal vs. extranodal)

c. presence of B symptoms at relapse

Patients who relapse >12 mo after chemotherapy aloneor combined modality therapy

a. best prognosis

b. usually respond to additional standard therapyc. resulting in a long-term survival of 6070%


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myeloablative autologous stem celltransplant in patients with refractorydisease or relapse within 12 mo of therapy

results in a long-term survival rate of 4050%.


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Non-Hodgkin

Lymphoma


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Non-Hodgkin Lymphoma approx. 60% of all lymphomas in children and

adolescents

represents 810% of all malignancies in children between519 yr of age

annual incidence in the USA of 750800 cases per year inchildren 19 yr of age

survival rates

9095% for localized disease6090% with advanced disease.


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EPIDEMIOLOGY

most children and adolescents with NHLpresent with de novo disease

small number of patients develop NHLsecondary to specific etiologies


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Inherited or acquired immune deficiencies

(e.g., severe combined immunodeficiencysyndrome, Wiskott-Aldrich syndrome)

viral etiologies (e.g., HIV, EBV)

genetic syndromes (e.g., ataxia-telangiectasia,

Bloom syndrome)


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Documents

CA & Benign Tumors - 2