WILMS TUMOR(nephroblastoma)

• Wilms tumor is an adenosarcoma in the

kidney region.
• The tumor arises from bits of embryonic tissue
that remain after birth.
• This tissue can spark rapid cancerous growth
in the area of the kidney.
 Epidemiology

• The most common childhood abdominal

malignancy, representing 5-6% of children
<5yrs with average age of incidence is 3
years
• Annual incidence of 7.8 m children <15yrs
Clinical staging

• Stage I: tumor is restricted to the kidney

• Stage II: tumor extends beyond kidney but within perinephric
tissue & it is resectable
• Stage III: tumor spreads beyond perinephric tissue & is not
completely resectable
• Stage IV: with hematogenous spread
• Stage V: bilateral tumor

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Presentation

• Abdominal mass. an abdominal mass occurs in 80% of children

at presentation. large, firm, unilateral smooth, typically doesn’t
cross the midline.
• Abdominal pain. Abdominal pain or hematuria occurs in 25%.
• Tumor hemorrhage. A few patients with hemorrhage into their
tumor may present with hypotension, anemia, and fever.
• hypertension (60%) due to excessive renin production.
• microscopic hematuria
• pyrexia
• nausea/vomiting in 50%
Wt loss, bone pain are rare events
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 DIAGNOSIS
• P /E: hematuria may suggest a renal tumor
• Laboratory studies. Complete blood count, RFTs and LFTs,
urinalysis, coagulation studies, and cytogenic studies are made
to determine Wilms tumor.
• Ultra sound may indicate that mass is intrarenal- at this point the
main DDx is Neuroblastoma
• Abdominal CT scanning. helps in determining the origin of the
tumor, involvement of the lymph nodes, bilateral kidney
involvement, invasion into major vessels (eg, inferior vena cava),
and liver metastases.
• Abdominal MRI scanning.determination of caval patency and
may be important in determining whether the inferior vena cava
is directly invaded by the tumor.
• Arteriography
 Treatment
• Treatment consists of surgical removal as
soon as possible, after the growth is
discovered, combined with radiation
and chemotherapy
•
 Rx…
• Large, necrotic, hrgic tumors may require pre-
op angiographic embolization to facilitate
their extirpation
• For very large tumors, irradiation plus chemo.
Is applied in order to shrink tumor size pre-op;
a significant reduction in size is usually evident
in 7-10 days, allowing immediate
nephrectomy
 Rx….
• Chemo is optimally began 5 days post –op while
irradiation is began 10 days post-op
• Antineoplastics. These chemotherapeutic agents
used to treat patients with Wilms tumor depend on
the stage and histology of disease; commonly used
agents include dactinomycin, vincristine,
doxorubicin, cyclophosphamide, etoposide, and
carboplatin; the dosage depends on the particular
stage of the disease and on the child.
 Rx…
• Vincristin 1.5mg/m2 and Dactinomycvin 0.04mg/kg to
all Pts
• Doxorubicin 60mg/m2 added to regimen for Pts with
stages III and IV, and in all those with unfavourable
histology
• Stage II Pts do not benefit from flank irradiation
• Stage III Pts have similar outcome with 10Gy or 20 Gy
irradiation(Gray is the SI unit of absorbed dose for
ionizing radiation)
 Nursing Mx.

• Prevent transfer of microorganism. Perform hand

washing prior giving care, utilize mask and gown when
needed, provide a private room, monitor for any signs
and symptoms of infection.
• Prevent oral trauma. Instruct the use of a soft-sponge
toothbrush or sponge toothette or gauze when rinsing
the mouth; instruct to avoid foods which are hot,
spicy, or high in ascorbic acid (vitamin C); provide oral
hygiene 30 minutes prior or after meals; instruct to
refrain from eating or drinking for 30 minutes after
completion of oral hygiene; and offer moist, soft, bland
foods.
• Prevent anxiety. Have the parents to stay with the child or
encourage open visitation, provide a telephone number to call for
information; explain all procedures and care in simple, direct,
honest terms and repeat as often as necessary; reinforce physician
information if needed and provide specific information as needed;
and provide consistent nurse assignment with the same personnel;
encourage parents to participate in care.
• Prevent injury. Avoid any palpation of abdominal mass; post sign
on bed stating not to palpate preoperatively; assess incision site for
redness, swelling, drainage, intactness, and healing and change
dressing when soiled or wet; assess oral and perineal area; and
encourage parents to appropriately dress child based on weather
conditions and to refrain from participating on rough activities
or sports.
 Complications and side effects
• Resistance
• Cardiotoxicity
• Nausea
• Alopecia
• Cystitis
• Stomatitis
• Bone marrow surpression
 NEUROBLASTOMA

• A cancer arising in the adrenal gland or less

often from the extra-adrenal sympathetic
chain, including the retroperitoneum, chest,
and neck .
 Etiology
• a germinal mutation-in general, cancer begins
with a genetic mutation that allows normal,
healthy cells to continue growing without
responding to the signals to stop, which
normal cells do. Cancer cells grow and
multiply out of control. The accumulating
abnormal cells form a mass (tumor
• Hirschsprung disease
 Epidemiology

• Age: 40% of patients are younger than 1 year

when diagnosed, 35% are aged 1-2 years, and
25% are older than 2 years when diagnosed
• Sex: Males have a slightly higher incidence of
neuroblastoma than females, with a male-to-
female ratio of 1.2:1
 Staging for neuroblastoma (Evans
Stage Characteristics
I completely resected without local invasion
II Incompletely resected; macro/micro residues
III Bilateral involvement
IV Regional/systemic spread a) Bone negative; marrow
negative
b) Local bone positive; marrow
negative
c) Generalized bone positive
and/or marrow positive
V Involvement of spleen, liver, skin, bone marrow

Infants have good prognosis patient otherwise would be in stage I or II

•
 Pathophysiology:

• Anatomic Origin and migration pattern of

neuroblasts during fetal development explains
the multiple anatomic sites where these tumors
occur; location of tumors appears to vary with
age. Tumors can occur in the abdominal cavity
(40% adrenal, 25% paraspinal ganglia) or involve
other sites (15% thoracic, 5% pelvic, 3% cervical
tumors, 12% miscellaneous). Infants more
frequently present with thoracic and cervical
tumors, whereas older children more frequently
have abdominal tumors.
 Clinical features
• abdominal pain, emesis, weight loss, anorexia,
fatigue, bone pain, and chronic diarrhea.
• bone pain and a limp
• with unexplained fever, weight loss,
irritability, and periorbital ecchymosis
secondary to metastatic disease to the orbits
Diagnosis

• CT, MRI, ultrasound of primary

• Bilateral bone marrow aspirates/biopsies
• Bone scan
• CXR and Chest CT
• Urine catecholamines
• Tumor for biology studies

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 Treatment

• Chemotherapy is the predominant modality of

management of neuroblastoma. A number of effective
drugs have been identified. Cyclophosphamide,
ifosfamide, cisplatin, carboplatin, doxorubicin, and the
epipodophyllotoxin etoposide .
• Neuroblastoma is considered a radiosensitive tumor,
and radiation therapy is effective in achieving local
control or palliation. Attempts have been made to
improve on the modest gains of intensive, combined-
modality therapy by increasing the intensity of therapy
with BMT.
• 21
 Burkitt’s Lymphoma
• It is a solid tumor of B Lymphocytes which
form part of the white cell population in the
blood and lymph glands. It is one form of non-
Hodgkin's lymphoma. The type of cell affected
in Burkitt's lymphoma is the B lymphocyte
which is normally involved in fighting infection
by producing antibodies
 Incidence
• . It is the most common childhood tumor in
equatorial Africa but is very rare in children in
Western countries. Recently Burkitt's
lymphoma has been diagnosed in around 2%
of AIDS patients.
• Males are three times more likely to suffer
from this disorder as opposed to females.
• Occurs in children btw ages 2-15yrs.
 - Burkitt’s Lymphoma -
Causes

* a virus known as Epstein-Barr virus (EBV)

* This virus is very common and over 90% of adults
throughout the world have evidence of past infection.
Other factors are necessary for the disease to develop.
• Chronic malaria
• infection with the AIDS virus (human immunodeficiency
virus - HIV)
 - Burkitt’s Lymphoma –
Signs & Symptoms

* Effects depend on the

site of the tumor in
the body:
* In African Burkitt's
lymphoma the jaw is the
commonest site where it
causes visible swelling of the
cheek and loosening of the
teeth. • This picture shows the tumor cells
of a Burkitt’s Lymphoma patient. Like
all malignant tumors, Burkitt's
lymphoma is very rapidly growing and
often arises in, or spreads to, multiple
sites.
•http://155.37.5.42/eAtlas/HEM/1887.htm
 -Burkitt’s Lymphoma –
Diagnosis

* Burkitt's lymphoma is diagnosed from a biopsy sample

of the tumor. It can be differentiated from other
tumors by the distinctive pattern of tumor cells which
is known as a starry-sky pattern. It is possible to use
specialized laboratory techniques to determine the
presence of EBV in the tumor cells.
 -Burkitt’s Lymphoma –
Treatment
* In African children the drug cyclophosphamide is
the treatment of choice.
* In AIDS patients treatment is less successful because
of the underlying HIV infection. In addition to drugs,
these patients are usually undergo radiation to
shrink the tumor.
Treatment Results

Complete
Remission
 Retinoblastoma
• Childhood cancer
• Caused by immature retinal cells in one or both eyes
• It is most common eye cancer in children
 Cause
• The causes of retinoblastoma are mostly
genetic with the exception of a rare case of a
new case mutation. In fact, the children of a
person with the illness has a 50% chance of
inheriting the disorder. It is caused by a
mutation that occurs because of a cell in the
retina that is duplicating numerously and
therefore turning cancerous
 Clinical features
• Poor vision – When retinoblastoma is present, the vision
clearness decreases because of the tumor clouding the eye.
• A white “glow” in the pupil of the eye in dim lighting-
leukocoria
• Strabismus – also known as a wandering eye or cross eyes
where the eyes appear to be looking around in different
directions.
• Red eye – due to irritation of the eyes
• White spots – There will be white spots that will be floating
around the pupil.
 Diagnosis
• Fundoscopic examination
• Computed tomography scan
• MRI
• Blood tests
• Genetic and/or DNA testing
 Treatment
• Individualized for each patient – patients survival is
primary goal
• Enucleation-This is removal of the eyeball and the tumor
is extracted when no other option is possible due to the
size of the tumor.
• External Beam Radiation
• Radioactive Plaques
• Laser Therapy-The laser beam focuses on the cancerous
tumor, cuts off blood supply to the tumor and shrinks it.
• Cryotherapy-The tumor is frozen and thawed several
times by a cold gas and it deflates the tumor with no signs
of a tumor at all.
• Chemotherapy-shrinks the tumor.