Cancer in children

Hematology-Oncology
Division
Child health Departement
School of Medicine USU
1.Retinoblastoma (RB)
Is a malignant tumor of the
embrionic neural retina.
Unilateral or Bilateral
Hereditery and nonhereditary
Disease can be sporadic or familial
Incidence
Incidence : 1/20,000 live birth in the United State.
Approximately 40% of cases are hereditery.......
Bilateral,
60% of cases are nonhereditary ..... unilateral
The most common : < 2 years.

Clinical manifeatation :
A white pupilay reflex : leukocoria/ Cats eyes reflex.
Strabismus
Decreased visual acuity
Orbital inflammation, hyphema
Vitreous hemorrhage, resulting in black pupil.
Proptosis
Pain
LEUKOCORIA
 Patterns of Spread
The most common routes of metastatic spread are
intraocular and extraocular
Intraocular spread with direct infiltration via the optic
nerve to central nervous system, or spread via the
choroid to the orbit.
Extraoculer spread occurs via a variety of ways
:dispersion of the tumor cells through the
subarachnoid space to the contralateral optic nerve or
cerebrospinal fluid to central system, hematogenous
dissemination to the lung,brain, and lymphatic
dissemination...spreads anteriorly into conjungtivae
and eyelid or extends into extraocular tissue
Screening
All children should have screening
performed as part of well-child
check-ups, primarily by eliciting red
reflexes in the eye.
Staging
Intraocular Retinoblastoma:
Reese-Ellsworth
International Classification System
Treatment
Systemic chemotherapy
External beam radiotherapy
Local opthalmic therapy
Intra-arterial chemotherapy
Enucleation
RETINOBLASTOMA STD. IV
2. NEUROBLASTOMA

The most common solid tumor in childhood outside

the CNS, 7% of all malignancies.
The peak incidence :2 years of age.

Clinical manifestation :
It present as :
- A tumor mass along symphathetic ganglia.
- An abdominal mass (70%).
Tumor can also be found : neck, thorax & pelvis.
The signs & symptoms depend on the site of the
tumor, size & degree of spread.
Hepatomegaly, anemia, a coagulopathy, bone
pain.
 Horners syndrome : contracted pupil,
ptosis, enopthalmus, anhidrosis).
Racoon eyes with periorbtal hemorrhage.

Diagnostic evaluation :
History
Physical examination
Laboratorium studies
Diagnostic imaging : CT scan
Biopsy
RACOON EYES
3. NEFROBLASTOMA (WILMS TUMOR)
Its a tumor of the developing kidney.
The second retroperitoneal tumor in children.
Approximately : 6% of childhood malignancies.
Occurs in young children : 1 & 5 years.

Cinical manifestation :
Abdominal mass
Abdominal pain, malise, hypertension,
microscopic hematuria.
Bleeding : Anemia, pallor, fatgue
Thrombus, polycythemia.
4.Soft- Tissue Sarcomas
(STS)
A heterogeneous group of malignant
tumors that are derived from primitive
mesenchymal cells
Can be from muscle, connective tissue,
supportive tissue and vascular tissue
Locally highly invassive, have a high
propensity for local recurrence
Can be divided : Rhabdomyosarcoma
(RMS) and non- rhabdomyosarcoma soft
tissue sarcomas (NRTST)
 RHABDOMYOSARCOMA
A tumor of striated muscle, most common pediatric
STS
Incidence : 5-8% of childhood cancers.
Rhabdomyosarcoma is the third most common solid
tumor following neuroblastoma and wilms tumor
There are two age peaks: 2-6 years and 15-19 years.
May occur in any anatomic location of the body
where there is skletal muscle.
Most often found : head & neck (40%), genitourinary
tract (29%), extremities (14%), trunk (12%).
Clinical manifestation :
A mass that may or may not be painful.
Symptoms due to displacement or obstruction of
normal structure.
Nasal congestion, epistaxis, mouth breathing,
cranial nerve paralysis, blindess, headache,
vomiting, trismus, etc.
 Diagnostic Evaluation
Complete history
Physical examinations
Complete blood count
Urinalysis
Serum electrolytes, blood urea nitrogen (BUN)
,creatinine, ALT, AST,LDH and Alkaline phospathase.
MRI and CT of primary lesion
Chest CT Scan
Bone scan
Bilateral bone maroww aspiration and biopsy
Biopsy of suspicious lymph nodes
 Treatment
Local control : Surgery and / or
radiotherapy
Systemic control : Chemotherapy,

Prognosis
Can be curable in the of children receiving
optimal therapy ( > 70% susvival 5 years
after diagnosis.)
RHABDOMYOSARCOMA
5. TERATOMA

Germ cell neoplasma >> in the children.

Incidence : 4.2 cases permillion population per
year.
In males : most common in white.
Male : female = 1:4

Clinical manifestation :
Present as masses, depends on location.
AFP is elevated.
SACROCOCCYGEAL TERATOMA
6. OSTEOSARKOMA

The high risk period : the adolescent growth

spurt.
Patient : taller than their peers of similar age.
Etiology : unknown.

Clinical manifestation :
Pain & swelling : >>>
Initial complaints : sport injury & sprain.
Limitation of motion, joint effusion,
tenderness, warmth.
OSTEOSARKOMA
OSTEOSARCOMA
 Case 2

OSTEOSARKOMA
With lung metastasis
Swelling
7.Lymphomas of childhood

The Malignant lymphomas:

1. Hodgkins disease
2. Non-Hodgkins lymphoma
(NHL)
 .. The malignant lymphoma

Approximately 10% of all cancer in

children < 15 yr., the third most
frequently after leukemia and brain
tumors.
Male : female = 3 : 1
NHL : HD = 1.5 : 1
HodgkinS Disease

Insiden 0 15 tahun
Male predominance
Children have a better response to
treatment
Survival rate > 20 years for
75%
 Symptoms and signs

Painless cervical adenopathy (70-80%)

Supraclavicular adenopathy (25%)
Mediastinal mass
Node > 2 cm,Ruberry texture, discrete
or matted together , are not fixed to
surrounding tissue
Lymphadenopathy increasing in size
after 2weeks or failing to resolve
within 4-8 weeks.
 symptoms and signs

Fatigue , anorexia, weight loss , fever

, night sweats , pruritus , cough
Half of patients : asymptomatic
mediastinal disease (adenopathy or
anterior mediastinal mass)
Splenomegaly or hepatomegaly :
associated advanced disease
 Diagnostic investigations and
staging
1. Biopsy
2. Imaging
3. Laparotomy
4. Bone marrow aspirates and
trephines
5. Blood tests:CBC,ESR,viral titers for
EBV,CMV,measles,chicken
pox,rubella,renal and liver function
6. Ann Arbor Staging
 Ann Arbor staging system for
Hodgkins disease
Stage I : involvement of 1 lymph node region(1) or a single
extralymphatic organ or site (IE)
Stage II :involvement of 2 or more lymph node regions on the
same side of the diaphragma (II) or solitary involvement
of an extralymphatic organ or site and of 1 or more lymph
node regions on the same site of diaphragma (IIE)
Stage III : involvement of lymph node regions on both sides
of the diaphragm (III) which may be accompanied by
localized involvement of extra-lymphatic organ or site
(IIIE) or by involvement of the spleen (IIIS) or both of the
these (IIISE)
Stage IV: Diffuse or disseminated involvement of 1 or more
extralymphatic organs or tissues with or without
associated lymph node enlargement
Ann Arbor Staging
 Treatment

1. Radiotherapy
2. Chemotherapy
3. Combined modality
 HODGKINS LYMPHOMAS
Enlargement of the lymph nodes
NON HODGKINS LYMPHOMA

Divers group of cancers , 5 15% of

malignancy in children < 15 yr.
Childhood Lymphoma are rapidly
proliferating, high grade , diffus
malignancy
Hystologically
classified:lymphoblastic lymphoma
(LL) ,small non-cleaved cell
lymphoma (Burkit lymphoma (BFM)
 Burkitt-like lymphoma (BLL) , Large
B-cell lymphoma (LBCL) , Anaplastic
large cell lymphoma ( ALCL)
 Symptoms and signs

Childhood NHL: any site of lymphoid

tissue, lymph nodes, thymus, liver ,
spleen.

Extralymphatic sites : bone, bone

marrow, CNS, skin, testes.
 Location of tumor

Intra abdominal primary tumors : 31%

Mediastinal : 27%
Head and neck primary sites : 29%
Tissue of Waldeyers ring
Cervical lymph nodes
Peripheral lymph nodes,skin,
bone,epidural space
 Initial symptoms

Cough Constitutional
Sore throat symptoms :
Abdominal pain fever,night
Vomiting sweats ,weight
Adenopathy loss uncommon
Painless,rapidly in NHL , more
enlarging suggestive of
lymphadenopathy Hodgkins
Disease
 Most rapidly growing human tumors
Airways compression
(cough,dyspnea,orthopnea)
Superior v.cava obstruction ( facial edema,
facial plethora,venous engorgement)
result of mediastinal NHL .These
symptoms are a true
emergencyrapid diagnosis and
treatment
 Pleural or pericardial effusions
CNS and bone marrow involvement

Bone marrow contains more than

25% lymphoblast- patients are
diagnosed with ALL
 Diagnostic

Physical examination
CBC,peripheral blood smear,renal
and liver fuction,electrolyte
Biopsi: histopathologic ,
,immunophenotyping, cytogenetics
Bone marrow
St.Jude Children s Research Hospital Staging System for
Non-Hodgkins Lymphoma

Stage 1: NHL is limited to one lymph node group (e.g., neck,

underarm, groin, etc.) or tumor outside of the abdomen or
mediastinum (middle chest)

Stage 2: NHL is limited to one tumor with local lymph node

involvement, or NHL is limited to two or more tumors or lymph node
groups on the same side of the diaphragm, or NHL is limited to a
primary tumor of the gastrointestinal tract with/without involvement
of local lymph nodes.

Stage 3: NHL includes tumors or lymph node groups on both sides of

the diaphragm, or any primary NHL tumor within the thorax (trunk) or
extensive NHL within the abdomen, or any NHL around the spine or
the outermost membrane of the brain and spinal cord (dura mater).

Stage 4: NHL is in the bone marrow or central nervous system (CNS),

with/without other sites of involvement. Bone marrow NHL is defined
as 5% malignant cells in an otherwise normal bone marrow with
normal blood counts and smears. By contrast, lymphoblastic
lymphoma that produces more than 25% malignant cells in the bone
marrow is defined as leukemia.
 LIMFOMA NON HODGKIN
ENLARGEMENT OF LYMPH NODES