PAEDIATRICS ONCOLOGY CONDITIONS
(CHILDHOOD MALIGNANCIES)
PYUZA,MD
1.Burkitt’s Lymphoma
2.Nephroblastoma
3.Neuroblastoma
Learning Objectives
By the end of the session, students are expected to be
able to:
• Define common childhood malignancies
• List the causes and risk factors for common
childhood malignancies
• Describe the clinical presentation of common
childhood malignancies
• List the treatment modalities for childhood
malignancies
BURKITT’S LYMPHOMA
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Outline
• Definition
• Classification
• Aetiology/risk factors
• Epidemiology
• Clinical features
• Diagnosis
• DDx
• Complications
• Treatment
• Prognosis
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Definition
• Burkitt’s Lymphoma (BL) is a highly
aggressive tumor of B-cell lymphocytes,
the lymphocytes that the immune
system uses to make antibodies.
• It is a non-Hodgkin Lymphoma (NHL)
• BL is extremely rapidly growing tumor
• It was first described in East Africa (Ug)
by Denis Parsons Burkitt in 1958.
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Classification
• Burkitt’s Lymphoma can be classified as:
Endemic
Sporadic
Immunodeficiency-related Burkitt’s
lymphoma
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Endemic BL
• It is common in Africa
• It is associated with the Epsten-Barr
Virus (EBV)
• Most commonly involves the jaw and
facial bone (orbit) in > 50% of cases.
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Sporadic BL (Non-endemic)
• This is rare type seen all over the world
• No association with EBV
• Most often presents as abdominal
tumors with bone marrow involvement
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Immunodeficiency-related Burkitt
lymphoma cases usually as with nodal
involvement with frequent bone marrow
involvement
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Aetiology/Risk Factors
• The exact cause and mechanisms of
Burkitt’s lymphoma are not known
• Implicated factors include:-
– Chromosomal abnormalities:
– Viral infection: Epsten-Barr Virus (EBV)
– Malarial infection
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Epidemiology
– Incidence in these areas of endemic
disease is 100 per million children.
– BL is a very rare tumor in the US, with
about 100 new cases occurring each
year.
– The male-to-female ratio is 2-3:1.
– BL is most common in children
– In Africa, the mean age is 7 years
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Clinical features
• Jaw/facial swelling, rapidly growing (most
common)
– May involve orbit
• Abdominal masses, which can cause abdominal
pain, distention & ascites
– GI symptoms: Nausea, vomiting, loss of appetite,
change in bowel habits, features of acute abdomen
Intestinal perforation
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• CNS involvement
– Meningeal infiltration, with or without
cranial nerve (CN) involvement (CN III and
CN VII); most common mode of
presentation with CNS disease
• Headaches
• visual impairment
• and paraplegia
These may be the initial presenting
features in some cases
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• "B" systemic symptoms
– may be associated with other presenting
symptoms (eg, fever, weight loss, night
sweats, fatigue
• Other
– Bone marrow involvement
– Painless lymphadenopathy (adults >
children)
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Stages of NHL
I Single tumor /node NOT in mediastinum
or abdomen
II 1-2 nodes same side of diaphragm or
resectable GI primary
III 2+ nodes both sides of diaphragm;
intrathoracic or extensive intra-abd
IV Any of above with CNS and/or BM
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Diagnosis
• Biopsy of swelling to confirm the diagnosis.
• Abdominal pelvic US
• FBP
• Renal function test (Serum Creatinine & BUN)
• Serum electrolytes, calcium and phosphate
• Liver function test (Liver enzymes)
• HIV serology
• Peripheral smear
• Reticulocyte count
• LDH, Uric Acid 16
Differential Diagnosis
• Acute Lymphoblastic Leukemia (ALL)
• Other Lymphomas (eg.Diffuse Large Cell Lymphoma, Follicular,
Lymphoblastic, Mantle Cell Lymphoma)
• Neuroblastoma
• Wilms Tumor (Nephroblastoma)
• Ludwig angina
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Complications
• Renal failure - as a result of retroperitoneal
disease and renal involvement.
• Gastrointestinal bleeding
• Acute abdomen
• Intestinal perforation
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Treatment
• Chemotherapy
• Treatment of associated complications
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Prognosis
• For children: early diagnosis and
treatment have good prognosis.
• Patients with bone marrow and CNS
involvement have a poor prognosis
• Adults especially those in the advanced
stage has poor prognosis.
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NEPHROBLASTOMA
(Wilm’s Tumour)
Definition
Nephroblastoma (Wilm’s Tumor)
Is the cancer of the kidney(s) that typically
occurs in children.
– Is highly responsive to treatment, with about 90%
of patients surviving at least five years
Risk factors for Nephroblastoma
• People of African descent have the highest
rates of Wilm’s tumour.
• Females are more likely to develop the
tumour than males
• Most instances occur among children < 5
years
EPIDEMIOLOGY.
• The incidence of Wilms tumor is approximately 8 cases per
million children <15 yr of age.
• It usually occurs in children between 2–5 yr of age, although it
has also been encountered in neonates, adolescents, and
adults.
• It accounts for approximately 6% of pediatric cancers and is
the second most common malignant abdominal tumor in
childhood. It may arise in one or both kidneys; the incidence
of bilateral Wilms tumor is 7%.
• It may be associated with hemihypertrophy, aniridia, and
other congenital anomalies, usually of the genitourinary tract.
It also has been described in association with a variety of
syndromes
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Clinical Presentation
• Common is abdominal swelling
or intraabdominal mass
(discovered by a parent while
bathing the child) that is
painless and lateralized to one
side deeply.
– Most are unilateral, bilateral
occurs in < 5% of cases
• Hematuria
• Hypertension (raised blood pressure)
• There may be
– Fever
– loss of appetite and weight loss
• Metastasis is usually to the lungs (respiratory
features)
• The rupture of the tumour puts the patient at
risk of haemorrhage and dissemination.
– Bleeding of the tumour can cause irritation in the
area of the kidney which may be painful
DIAGNOSIS
• HISTORY TAKING AND PHYSICAL EAXMINATION
• Any abdominal mass in a child must be considered malignant until
diagnostic imaging and laboratory findings define its true nature.
• If there is any doubt, biopsy or excision and histological verification
is the final arbiter. Wilms tumor must be differentiated from a
variety of malignant abdominal and pelvic tumors
• Once an abdominal mass is discovered, a complete physical
examination should be performed, followed by a complete blood
count, liver and kidney function studies, and a search for specific
tumor markers secreted by the suspected tumor.
• Imaging studies include a flat plate of the abdomen,
ultrasonography, and CT and/or MRI.
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DDx
Management
• PRE REFERRAL MANAGEMANT.????????
• Refer the child to hospital without delay
• At the referral hospital
– Further investigation
– Management
• Surgery: Nephrectomy (partial or total)
• Chemotherapy
• Radiation
PROGNOSIS.
•There is remarkable correlation among the
DNA content in the cells of Wilms tumor,
histologic subtype, and treatment outcome.
•The prognosis is worse with a larger tumor
(>500 g), advanced stage (III and IV), and an
unfavorable histologic subtype.
•Wilms tumor is a paradigm of successful
multidisciplinary treatment, and >60% of
patients with all stages generally survive.
Stages I through III have a cure rate of >90%. 30
NEUROBLASTOMA
Definition
• Neuroblastoma is the Cancer of the
embryonal peripheral sympathetic nervous
system
• The most common extracranial solid tumour
in infancy
• Cause is unknown
– no specific environmental exposure or risk factors
have been identified
• EPIDEMILOGY
• Male-to-female ratio is 1.2 : 1
• 40% of patients are < 1 year old when
diagnosed
– incidence decreases every consecutive year up to
the age of 10 years, after which the disease is rare
• Age and stage are important prognostic
factors
RISK FACTORS
• The genetic event that initially triggers formation of NB is
not known.
• The pathogenesis is likely to be related to a succession of
mutational events, prenatally and perinatally, that may be
caused by environmental and genetic factors.
• Increased incidence of NB is associated with some maternal
and paternal occupational chemical exposures, work in
farming, and work related to electronics.
• Familial NB is found in 1–2% of cases. Neuroblastoma and
congenital cardiovascular malformations also may be
associated.
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Clinical Presentation
• Signs and Symptoms (vary with site of
presentation e.g heart, blood vessels, bronchi, and GI tract)
• GI symptoms
– Abdominal pain
– Vomiting
– Weight loss
– Anorexia
– Fatigue
• If catecholamine producing tumor
– Sweating
– Hypertension (raised blood pressure)
– Profound diarrhea
• Redness of the eyes
(Racoon eyes) due to
metastatic disease to the
orbits
– Probably related to
obstruction of the palpebral
vessels {branches of the
ophthalmic and facial
vessels}
• Unexplained fever
• Irritability
• Approximately 2/3 of patients have
asymptomatic abdominal mass (usually
discovered by the parents)
• More than 50% of patients present with
advanced stage disease
– usually to the bone and bone marrow, thus bone
pain and a limp
• Metastatic lesions of the skin are common in
infants < 6 months
• Paraspinal dumbbell tumours can extend and
impinge on the spinal cord
– causing neurologic dysfunction (lower extremity
weakness or paraplegia)
DAGNOISIS
• HISTORY TAKING AND PHYSICAL EXAMINATION
• NB usually is discovered as a mass or multiple masses
on plain radiographs, CT, or MRI).
• Tumor markers, including homovanillic acid (HVA) and
vanillylmandelic acid (VMA) in urine, are elevated in
95% of cases and help to confirm the diagnosis.
• A pathologic diagnosis is established from tumor tissue
obtained by biopsy. NB can be diagnosed in a typical
presentation without a primary tumor biopsy if the
patient has neuroblasts observed in bone marrow and
elevated VMA or HVA in the urine.
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DDx
• Esthesioneuroblastoma
• ganglioneuroblastoma
• pheochromocytoma
• Rhabdomyosarcoma
• Wilms' tumor
Management
• PRE REFELLAR MANAGEMENT??????
• Refer the child to hospital without delay
• At the referral hospital
– Chemotherapy
– Surgery
– Radiation
PREVENTION.
•Currently there is no recognized way to
prevent neuroblastoma. A worldwide attempt
to improve survival by early detection through
infant catecholamine screening has proven to
be ineffective
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END
• THANK U
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