Oncology Assessment

Prepared by: Dr.Hamda Abdirahman

MD,Pediatrician
• Childhood cancer is a rare
• Hematopoietic tumors (leukemia, lymphoma)
are the most common childhood cancers,
followed by brain/central nervous system
(CNS) tumors and sarcomas of soft tissue and
bone
• There is wide variability in the age-specific
incidence of childhood cancers.
• Embryonal tumors, such as neuroblastoma and
retinoblastoma peak during the first 2 years of
life
• Acute lymphoblastic leukemia (ALL) peaks
during early childhood ages 2-5 years
• Osteosarcoma peaks during adolescence
• Hodgkin disease peaks during late adolescence
 Clinical features
History
• Many signs and symptoms of childhood cancer
are nonspecific.
• Although most children with fever, fatigue, weight
loss, or limp do not have cancer, each of these
symptoms may be a manifestation of an
underlying malignancy
• A prominent lymph node that does not resolve
(with or without antibiotics) may warrant a biopsy
• A limp that does not improve within weeks
should prompt a CBC and a radiograph.
• Fever, night sweats, or weight loss should raise
the concern for lymphoma
PHYSICAL EXAMINATION:
• Growth parameters and vital signs are
important to obtain in all patients.
• Pulse oximetry should be obtained if
respiratory symptoms are present.
• The overall appearance of the patient should
be noted, particularly general appearance,
pain, cachexia, pallor, and respiratory distress.
• Palpable masses should be measured
• Lymphadenopathy and organomegaly should
be quantified, if present
• A thorough examination of the skin may reveal
rashes, bruises, and petechiae.
• Careful neurological and ophthalmological
examinations are crucial if headache or
vomiting is present because the vast majority
of patients with CNS tumors have abnormal
neurological examinations
 COMMON MANIFESTATIONS:
 Visual Impairment and Leukocoria
 Vomiting
 Hepatomegaly
 Splenomegaly
 Headaches
 Lymphadenopathy
 Anemia
 Petechiae/Purpura
 Pancytopenia
 Fever of Unknown Origin
• Lymphadenopathy and organomegaly are
common in leukemia, particularly with T-cell
ALL or non-Hodgkin lymphoma
• Patients with solid tumors usually have a
palpable or measurable mass
• Other signs and symptoms may include limp,
cough, dyspnea, cranial nerve palsies, and
papilledema
• In general, malignant masses are firm, fixed,
and nontender, whereas masses that are
infectious or inflammatory in nature are
relatively softer, mobile, and tender to
palpation
 INITIAL DIAGNOSTIC EVALUATION
Screening Tests:
• CBC with differential and review of the peripheral
blood smear is the best screening test for many
pediatric malignancies
– Leukopenia (with or without neutropenia),
– Anemia
– Thrombocytopenia
• may be present in leukemia or any cancer that
invades the bone marrow (e.g., neuroblastoma,
rhabdomyosarcoma, and Ewing sarcoma)
• Leukemia may also produce leukocytosis,
usually with blasts present on the peripheral
blood smear
• Lactate dehydrogenase and uric acid are often
elevated in fast-growing tumors (leukemia or
lymphoma) and occasionally in sarcomas or
neuroblastoma
• Electrolytes
• Renal function test
• Hepatic function test
• Elevated blood pressure, if confirmed by
repeat measurements, should prompt a
urinalysis, as should the palpation of an
abdominal mass
• Serum (and/or cerebrospinal fluid) alpha-
fetoprotein (AFP) and beta-human chorionic
gonadotropin (β-HCG) are screening tests
(and tumor markers) for germ cell tumors
– Serum AFP is also a tumor marker for
hepatoblastoma.
• Urine homovanillic acid (HVA) and
vanillylmandelic acid (VMA) are tumor
markers for neuroblastoma
 Diagnostic Imaging
• A chest x-ray ( PA and lateral) is the best
screening radiographic study for a patient with
– Suspicious cervical lymphadenopathy fever, and
weight loss
– Mediastinal masses and pleural effusions can
often be detected on CXR
• Abdominal signs or symptoms warrant an
ultrasound or CT scan
• Persistent headaches or morning vomiting
should prompt a CT or magnetic resonance
imaging (MRI) scan of the head
• If bone tumors are suspected, plain
radiographs are indicated and will usually
reveal the lesion(s), if present
 Principles of Cancer Treatment
• The overall goal of pediatric oncology is to
cure all patients with minimal toxicity.
• Treatment for children with cancer is often
multimodal and may involve:
– Surgery
– Radiation therapy
– Chemotherapy
– Immunotherapy
• Surgery and radiation are generally local
treatment modalities (an exception is total
body irradiation as part of a bone marrow or
stem cell transplant), whereas chemotherapy
and immunotherapy have both local and
systemic effects
 ONCOLOGICAL EMERGENCIES
• Adverse effects of tumors and treatments may
result in oncological emergencies in children
and adolescents
• Mediastinal masses from lymphoma can
cause life-threatening airway obstruction.
• A common metabolic emergency is tumor lysis
syndrome, often seen in treatment of leukemia
and lymphoma.
• Large amounts of phosphate, potassium, and uric
acid are released into the circulation from lysed
cells resulting in acute renal failure by tubular
deposition of uric acid crystals.
• Overwhelming infection and spinal cord
compression with neurological compromise are
other oncological emergencies
Leukemia
• Leukemia is a type of cancer of blood bone
marrow
• Characterized by an abnormal increase of
immature white blood cells called blasts
• The leukemia are the most common malignant
neoplasms in childhood, accounting for
approximately 31% of all malignancies that
occur in children younger than 15 yr
 Blast cell
• Blast cells are immature precursors of either
lymphocytes( lymphoblast), or
granulocytes( Myoblasts)
• They do not normally appear in peripheral blood.
• They can be recognized by their large size, and
primitive nuclei ( i.e. the nucleus contain nucleoli)
• Presence of BS in blood, signify Acute Leukemia
• Acute lymphoblastic leukemia (ALL) 77%
• Acute myelogenous leukemia (AML) 11%
• chronic myelogenous leukemia (CML) for 2–
3%
• juvenile myelomonocytic leukemia (JMML)
1–2%.
 Acute Lymphoblastic Leukemia
• Childhood acute lymphoblastic leukemia (ALL )
was the first disseminated cancer shown to be
curable
• It actually is a heterogeneous group of
malignancies with a number of distinctive
genetic abnormalities that result in varying
clinical behaviors and responses to therapy.
 Epidemiology
• ALL has a striking peak incidence at 2-3 yr of age
• Occurs more in boys than in girls at all ages
• The disease is more common in children with certain
chromosomal abnormalities, such as:
– Down syndrome
– Bloom syndrome
– Ataxia-telangiectasia
– Fanconi anemia
• Among identical twins, the risk to the second twin if one
twin develops leukemia is greater than that in the
general population
• The risk is >70% if ALL is diagnosed in the first
twin during the 1st yr of life and the twins
shared the same (monochorionic) placenta

• If the first twin develops ALL by 5-7 yr of age,

the risk to the second twin is at least twice
that of the general population, regardless of
zygosity.
 Etiology
• In virtually all cases, the etiology of ALL is
unknown
• Although several genetic and environmental
factors are associated with childhood
leukemia
 Factors Predisposing to Childhood
Leukemia
GENETIC CONDITIONS ENVIRONMENTAL
• Down syndrome FACTORS
• Fanconi anemia
• Bloom syndrome
• Ionizing radiation
• Diamond-Blackfan anemia • Drugs
• Shwachman-Diamond syndrome • Alkylating agents
• Kostmann syndrome
• Neurofibromatosis type 1 • Benzene exposure
• Ataxia-telangiectasia
• Severe combined immune
deficiency
• Paroxysmal nocturnal
hemoglobinuria
• Li-Fraumeni syndrome
 Cellular Classification
• FAB classification
• WHO classification
• Cyto-genetic classification
 FAB classification of ALL
Cytologic features L1 L2 L3

Cell size Small cells Large heterogeneous Large homogenous

predominate, in size
homogenous

Cytoplasm Scanty Variable often Moderately abundant

moderately
abundant

Nucleoli Small prominent One or more,

prominent

Nuclear size Homogenous Variable, Stippled,

heterogeneous homogenous

Nuclear shape regular Irregular clefts Regular

Cyt.basophilia Variable Variable Intensely basophilic
Cyt vacuolation variable Variable Prominent
 Classification of ALL (WHO)
Immunologic % of cases FAB subtype Cytogenetic
subtype abnormalities

Pre B ALL 85 L1.L2 T(9:22),t(4;11)

t(1:19))

T cell ALL 15 L1,L2 14q11 or 7q34

Mature B cell 5 L3 T(8:14)

ALL (Burkett
leukemia)
 Cytogenetic classification
Translocation ALL Prognosis

T (12;21) Good

T(1;19) Poor

T(4;11) MLL fusion Poor

JAK – 2 Mtation Poor

T (9;22) BCR – ABL Very poor

 Clinical Manifestations
• The initial presentation of ALL usually is
nonspecific and relatively brief
• Anorexia, fatigue, malaise, irritability, and
intermittent low-grade fever are often present
• Bone or joint pain, particularly in the lower
extremities, may be present
• Bone pain is severe and can wake the patient
at night
• As the disease progresses, signs and
symptoms of bone marrow failure become
more obvious with the occurrence
– Pallor, fatigue, exercise intolerance, bruising, oral
mucosal bleeding or epistaxis
– Fever, which may be caused by infection or the
disease
• Organ infiltration can cause
– Lymphadenopathy
– Hepatosplenomegaly
– Testicular enlargement
– Central nervous system (CNS) involvement
(cranial neuropathies, headache, seizures).
• Respiratory distress may be caused by severe
anemia or mediastinal node compression of
the airways
Physical examination:
• findings of
– Pallor
– Listlessness
– Purpuric and petechial skin lesions, or mucous membrane
hemorrhage can reflect bone marrow failure
• The proliferative nature of the disease may be
manifested as:
– Lymphadenopathy
– Splenomegaly
– Hepatomegaly
• Rarely, patients show signs of increased
intracranial pressure that indicate leukemic
involvement of the CNS. These include
– Papilledema
– Retinal hemorrhages
– Cranial nerve palsies
• B-lymphoblastic leukemia is the most common
immuno-phenotype, with onset at 1-10 yr of
age.
• The median leukocyte count at presentation
is 33,000/μL, although 75% of patients have
counts <20,000/μL
• Thrombocytopenia is seen in 75%
• Hepatosplenomegaly in 30–40% of patients
• In all types of leukemia, CNS symptoms are
seen at presentation in 5% of patients (10–
15% have blasts in cerebrospinal fluid [CSF]).
• Testicular involvement is rarely evident at
diagnosis
 Diagnosis
• Confirmative tests
• Supportive tests
 Supportive test
• LDH, serum uric acid
• Coagulation profile
• LFT,RFT
• Chest x-ray
• Ct scan of chest and brain
• Blood culture
• Baseline Echo, ECG
 Investigation ( confirmative)
• CBC
• Bone marrow aspiration/biopsy
• Cyto genetics
CBC
– Anemia
– Thrombocytopenia
– Leucopenia or leucocytosis
• Peripheral smear study – circulating blast can
be seen
– ALL is diagnosed by a bone marrow evaluation
that demonstrates >25% of the bone marrow cells
as a homogeneous population of lymphoblasts.
 Treatment
• Supportive care
• Chemotherapy
– Induction
– consolidation
– Maintenance therapy
• Allogeneic stem cell transplantation
• Treatment of relapse
 Supportive management
• Patients with high WBC counts are especially
prone to tumor lysis syndrome as therapy is
initiated. The kidney failure associated with
very high levels of serum uric acid can be
prevented or treated with allopurinol or urate
oxidase
• Chemotherapy often produces severe
myelosuppression, which can require
erythrocyte and platelet transfusion and
always requires a high index of suspicion and
aggressive empirical antimicrobial therapy for
sepsis in febrile children with neutropenia
• Patients must receive prophylactic treatment
for Pneumocystis jiroveci pneumonia during
chemotherapy and for several months after
completing treatment
 chemotherapy
• Standard treatment involves chemotherapy
for 2-3 yr, and most achieve remission at the
end of the induction phase.
Remission induction
• Initial therapy, termed remission induction
– It is designed to eradicate the leukemic cells from the
bone marrow.
• During this phase, therapy is given for 4 wk and
consists of:
– vincristine weekly
– A corticosteroid such as dexamethasone or
prednisone, and
– A single dose of a long-acting, pegylated asparaginase
preparation
– Patients at higher risk also receive daunomycin at
weekly intervals
• With this approach, 98% of patients are in
remission, as defined:
– by <5% blasts in the marrow
– A return of neutrophil and platelet counts to near-
normal levels after 4-5 wk of treatment
– Intrathecal chemotherapy is always given at the
start of treatment and at least once more during
induction
Consolidation:
• The second phase of treatment, consolidation
, focuses on intensive CNS therapy in
combination with continued intensive
systemic therapy in an effort to prevent later
CNS relapses
• Intrathecal chemotherapy is given repeatedly
by LP
• A small percentage of patients with features that
predict a high risk of CNS relapse may receive
irradiation to the brain in later phases of therapy.
• This includes:
– Patients who at diagnosis have lymphoblasts in the
CSF
– Either an elevated CSF leukocyte count
– Physical signs of CNS leukemia, such as cranial nerve
palsy
• Subsequently, many regimens provide 14-28
wk of therapy, with the drugs and schedules
used varying depending on the risk group of
the patient.
• This period of treatment is often termed
intensification and includes
– Delayed intensification phases of aggressive
treatment
– Interim maintenance nontoxic phases of treatment
• During these phases multi-agent
chemotherapy medications is used to
eradicate residual disease, such as:
– Cytarabine
– Methotrexate
– Asparaginase
– Vincristine
maintenance
• Finally, patients enter the maintenance phase of
therapy, which lasts for 2-3 yr
• Patients are given
– Daily mercaptopurine
– Weekly oral methotrexate
– Intermittent doses of vincristine and a corticosteroid
• A small number of patients with particularly poor
prognostic features, such as those with induction
failure or extreme hypodiploidy, may undergo
bone marrow transplantation during the first
remission.
 Treatment of Relapse
• Relapse occurs in the bone marrow in 15–20%
of patients with ALL and carries the most
serious implications, especially if it occurs
during or shortly after completion of therapy
• Intensive chemotherapy with agents not
previously used in the patient followed by
allogeneic stem cell transplantation can result
in long-term survival for some patients with
bone marrow relapse
• The incidence of CNS relapse has decreased to
<5% since introduction of preventive CNS
therapy
• The diagnosis is confirmed by demonstrating
the presence of leukemic cells in the CSF.
• The treatment includes:
– Intrathecal medication
– Cranial or craniospinal irradiation
– Systemic chemotherapy also must be used,
because these patients are at high risk for
subsequent bone marrow relapse
• Testicular relapse occurs in <2% of boys with
ALL, usually after completion of therapy.
• Such relapse occurs as painless swelling of 1 or
both testes.
• The diagnosis is confirmed by biopsy of the
affected testis
• Treatment includes systemic chemotherapy
and possibly local irradiation.
 Prognostic factors in ALL
Determinants Favorable Unfavorable
WBC Counts <10.000 > 50,000
Age 2-10 years < 1yr,>10yr
Gender Female Male
Ethnicity White Black
Node,liver,splenomegaly Absent Massive
CNS involvement Absent Present
FAB type L1 L2
Cytogenetic T(12:12) (TEL-AML1) t(9:22)(bcr-abl)
Trisomies 4,10,17
Ploidy Hyperdipoidy Hypodiploidy

Time to remission <14 days > 28 days

Lymphoma
• It is the most common cancer in adolescents,
accounting for >25% of newly diagnosed
cancers in those 15-19 yr old
• The 2 broad categories of lymphoma, Hodgkin
lymphoma and non-Hodgkin lymphoma, have
different clinical manifestations and
treatments
 Hodgkin Lymphoma
• Hodgkin lymphoma (HL ) is a malignant
process involving the lymphoreticular system
that accounts for 6% of childhood cancers
Epidemiology
• The worldwide incidence of HL is
approximately 2-4 new cases/100,000
population/yr.
• There is a bimodal age distribution, with
peaks at 15-35 yr of age and again after 50 yr
• HL is the most common cancer seen in adolescents and
young adults, and the 3rd most common in children <15 yr
old.
• In developing countries, the early peak tends to occur
before adolescence
• A male predominance is found among young children but
lessens with age
• Infectious agents may be involved, such as human
herpesvirus 6, cytomegalovirus, and Epstein-Barr virus (EBV)
• Infection with EBV confers a 4-fold higher risk of developing
HL and may precede the diagnosis by years.
 Etiology
• Reed-Sternberg cell is hallmark of disease
• Large (15-45 mm) multiple/multilobulated
nuclei
• Colonal in origin
• Arises from germinal center B cells
• HL appears to arise in lymphoid tissue and
spread to adjacent lymph node areas in a
relatively orderly manner
• Hematogenous spread also occurs, leading to
involvement of the liver, spleen, bone, bone
marrow, or brain, and is usually associated
with systemic symptoms
 Classification of HL
• The Revised World Health Organization
Classification of Lymphoid Neoplasms includes
2 modifications of the older Rye system
1. Nodular lymphocyte predominance
2. Classic Hodgkin lymphoma
1. Lymphocyte rich
2. Mixed cellularity
3. Nodular sclerosis
4. Lymphocyte depletion
Lymphocyte Predominant
10-15% of patients
More common in male
Younger patients
Localized disease
Mixed cellularity
30% of patients
< 10 yr of age
Advanced disease
Extra-nodal extension
Lymphocyte depletion
Rare in children
Common with HIV
Nodular sclerosis
Most common
40% of younger patients
70% of adolescents
 CLINICAL MANIFESTATIONS
– Lymphadenopathy cervical / supraclavicular
– Painless, non tender, firm and rubbery
– Hepatosplenomegaly
– Cough, dyspnoea, hypoxia
– Pleural or pericardial effusion
– Hepatocellular dysfunction
– B.M infiltration
– (Anemia, neutropenia, thrombocytopenia)
– Disease below diaphragm is rare (only3%)
 Systemic Symptoms (B symptoms)
• Important in staging
• Unexplained fever > 390C
• Weight loss > 10% in 6m
• Drenching night sweats
• Less common and not considered of
prognostic significance are symptoms of
pruritus, lethargy, anorexia, or pain
 Diagnosis
• Excisional Biopsy • Blood CP & ESR
– Light Microscopy • LFT’s
– Immunocytochemistry • Bone Marrow
– Molecular Studies Aspiration
• Chest X – Ray • Serum Copper &
– Mediastinal Mass Ferritin
• CT Scan • Bone Scan
– Chest
– Abdomen
– Pelvis
 ANN ARBOR STAGING CLASSIFICATION FOR
HODGKIN DISEASE
• Stage I
– Involvement of a single lymph node (1)
– or of a single extra lymphatic site or organ(1f)
• Stage II
– Involvement of two or more lymph node regions
on the same side of the diaphragm(II)
– or localized involvement of an extra lymphatic site
or organ and one or more lymph node regions on
the same side of the diaphragm (IIf)
• Stage III
– Involvement of lymph node regions on both sides
of the diaphragm (III)
– which may be accompanied by the involvement of
spleen (IIIS)
– or by localized involvement of an extra lymphatic
site or organ ( IIIf) or both ( IIIsf)
• Stage IV
– Diffuse or disseminated involvement of one or
more extra lymphatic organs or tissues with or
without associated lymph node involvement
• The absence or presence of fever > 38C for
three consecutive days , drenching night
sweats , or unexplained loss of > 10% body
weight in the 6 months preceding admission
are to be denoted in all cases by the suffice
letters A & B respectively
 TREATMENT

Treatment depends on:

• Stage of the disease
• Age at diagnosis
• Presence / absence of B symptoms
• Presence of hilar lymphadenopathy
• Presence of bulky nodal disease
Current Treatment Regimen
• Combined chemotherapy with or without low
dose involved field radiation therapy
Chemotherapy Regimens:
– MOPP (Mechlorethamine , Vincristine ,
Procarbazine , Prednisolone)
– COPP (Cyclophosphamide , Vincristine ,
Procarbazine , Prednisolone)
– ABVD (Adriamycin , Bleomycin , Vinblastine ,
Dacarbazine)
– BEACOPP ( For advanced stage disease )
(Bleomycin , Etoposide , Doxorubicin ,
Cyclophosphamide , Vincristine , Procarbazine ,
Prednisolone)
PROGNOSTIC FACTORS:
• FAVORABLE
– <10, F, favorable subtypes (LP and NS)
– and Stage I non-bulky disease
• UNFAVORABLE
– Persistently elevated ESR
– LD histopathology
– bulky disease--largest dimension >10cm
– B symptoms
LONG TERM COMPLICATIONS
• Secondary malignancy
– Acute Myelogenous Leukemia
– Non Hodgkin lymphoma
– Carcinomas of breast , lungs & thyroid
• Short stature
• Hypothyroidism
• Sterility
• Dental caries
• Sub clinical pulmonary dysfunction
• Ischemic heart disease
 PROGNOSIS
• Early Stage Disease 5 year survival ….95%
Advanced Stage Disease 5 year survival ….90%
• Relapses common within first 3 years from
diagnosis
• Relapses treated with Autologous Stem Cell
Transplantation
Non-Hodgkin Lymphoma
 Introduction
• Non-Hodgkin lymphoma (NHL ) accounts for
approximately 60% of lymphomas in children
and is the 2nd most common malignancy
between ages 15 and 35 yr
• Although >70% of patients present with
advanced disease, the prognosis has improved
dramatically, with survival rates of 90–95% for
localized disease and 80–95% for advanced
disease
 Epidemiology
• Although most children and adolescents with
NHL present with de novo disease
• Secondary cause of NHL include:
– Inherited or acquired immunodeficiencies
• Severe combined immunodeficiency syndrome
• Wiskott-Aldrich syndrome
– Virus-associated malignancy
• HIV, EBV
– Genetic syndromes
• Ataxiatelangiectasia
• Bloom syndrome
 Pathologic sub-type of NHL
• Three most prevalent subtypes of childhood
and adolescent NHL with different treatment
approaches are
1. Lymphoblastic lymphoma (LBL)
2. Mature B-cell lymphoma
I. Burkitt lymphoma (BL)
II. diffuse large B-cell lymphoma (DLBCL)
3. Anaplastic large cell lymphoma (ALCL)
• DLBCL is further divided into several subtypes:
– Germinal center B-cell–like ( which carries a
favorable prognosis and accounts for the vast
majority of pediatric cases of DLBCL)
– Activated B-cell–like and Primary mediastinal B-
cell subtypes(poorer prognosis)
 Clinical Manifestations
• The clinical manifestations of childhood and
adolescent NHL depend primarily on
pathologic subtype and sites of involvement
• The primary site of tumor involvement and
the pattern of metastasis vary by pathologic
subtype
• Lymphoblastic lymphoma (LBL):
– Typically manifests as a symptomatic mediastinal
mass
– A predilection for spreading to the bone marrow,
CNS, and testes in males
Burkitt Lymphoma (BL)
– Commonly manifests as:
• A diffuse leukemia presentation
• Massive abdominal (sporadic type)
• Head and neck (endemic type) tumor
– Can metastasize to the bone marrow or CNS
• Diffuse large B cell lymphoma (DLBCL)
– It usually manifests as either an abdominal or a
mediastinal primary tumor
– Rarely, disseminates to the bone marrow or CNS
• Anaplastic large cell lymphoma (ALCL)
– It manifests either:
– As a primary cutaneous manifestation (10%) or
– As systemic disease (90%) with dissemination to liver,
spleen, lung, or mediastinum
– Bone marrow or CNS disease is rare in ALCL
Site-specific manifestations of NHL include:
– Painless, rapid lymph – Increased abdominal
node enlargement girth or intestinal
– Cough obstruction with an
– Dyspnea with thoracic abdominal mass
involvement – Nasal congestion
– Superior mediastinal – Earache
syndrome – Hearing loss
– Ascites – Tonsil enlargement with
Waldeyer ring
involvement;
– Localized bone pain
• NHL can present as a life-threatening
oncologic emergency including:
• Superior mediastinal syndrome can occur as a
consequence of a large mediastinal mass
causing obstruction of blood flow or
respiratory airways
• Spinal cord tumors can cause cord
compression and acute paraplegias requiring
emergent radiation therapy
• Tumor lysis syndrome (TLS) can occur from rapid
cell turnover, which is especially common in BL
• TLS can result in severe metabolic abnormalities,
including hyperuricemia, hyperphosphatemia,
hyperkalemia, and hypocalcemia
• This can rapidly lead to renal insufficiency/failure,
as well as cardiac abnormalities, if not
aggressively treated
 Laboratory Findings
• Tissue biopsy for
– Flow cytometry
– Karyotyping
• Complete Blood Count
• Serum Electrolytes, Calcium , Phosphorus , Uric acid
• LFT’s & RFT’s
• Bone Marrow Aspiration & Biopsy
• CSF Examination
• Chest X Ray
• CT Scan
– Head & Neck
– Chest
– Abdomen & Pelvis
• PET Scan & Bone Scan
Staging system for childhood Non- Hodgkin
lymphoma
STAGE I:
• A single tumor with the exclusion of the mediastinum and
abdomen
STAGE II
• A single extranodal tumor with regional node involvement
• Two or more nodal areas on the same side of the diaphragm
• A primary gastrointestinal tract tumor (usually in the
ileocecal area), with or without involvement of associated
mesenteric nodes, that is completely resect able
• STAGE III:
– Two or more extranodal tumor(s) (including bone or skin) above
and/or below the diaphragm.
– Two or more nodal areas above and below the diaphragm.
– Any intrathoracic tumor (mediastinal, hilar, pulmonary, pleural, or
thymic).
– Intraabdominal and retroperitoneal disease, including liver, spleen,
kidney, and/or ovary localizations, regardless of degree of resection
– Any paraspinal or epidural tumor, whether or not other sites are
involved.
– Single bone lesion with concomitant involvement of extranodal
and/or nonregional nodal sites
• STAGE IV:
– Any of the above findings with initial involvement
of the central nervous system (stage IV CNS), bone
marrow
– (stage IV BM), or both (stage IV combined)
DIFFERENTIAL DIAGNOSIS
• Hodgkin Disease
• Leukemia
• Germ Cell Tumor
• Wilms Tumor
• Neuroblastoma
• Rhabdomyosarcoma
• Reactive lymphadenitis
 TREATMENT
• Systemic Chemotherapy
• Intra-thecal chemotherapy
• Radiotherapy indicated in
– CNS Disease
– SVC Syndrome
– Paraplegia
• Chemotherapy Regimens:
– COPAD (Cyclophosphamide , Vincristinr ,
Prednisolone , Doxorubicin)
– COMP (Cyclophosphamide , Vincristine ,
Methotrexate , 6 Mercaptopurine , Prednisolone)
• Duration of Treatment Burkitt Lymphoma &
Diffuse Large B Cell Lymphoma (6 weeks to 6
months)
• Lymphoblastic Lymphoma (24 months)
 Supportive Treatment

• Antibiotic prophylaxis
• Blood & platelet transfusions
• Allopurinol
• Parenteral nutrition
 COMPLICATIONS
• Infections
• Mucositis
• Pancytopenia
• Electrolyte imbalance
• Poor nutrition
• Growth retardation
• Cardiac Toxicity
• Gonadal Toxicity with Infertility
• Secondary malignancies
Thanks