care of children with Hepatoblastoma and bone tumor

1. HEPATOBLASTOMA
AND BONE TUMOR

2. #
HEPATOBLASTOMA

3. #
DEFINITIONS
• Hepatoblastoma: A type of liver cancer
that usually does not spread outside the
liver. This type usually affects children
younger than 3 years old.
• Hepatocellular carcinoma: A type of
liver cancer that often spreads to other
places in the body. This type usually
affects children older than 14 years old.

4. #
Two less common types of
childhood liver cancer are:
• Undifferentiated
embryonal sarcoma of the liver
(UESL): The third most common liver
cancer in children and adolescents. It
usually occurs in children between the
ages of 5 and 10 years.

5. #
• Infantile choriocarcinoma of the liver:
Choriocarcinoma of the liver is a very
rare tumor that appears to originate in the
placenta and presents with a liver mass
in the first few months of life. Infants are
often unstable due to hemorrhage of the
tumor. Clinical diagnosis may be made
without biopsy based on tumor imaging of
the liver associated with extremely high
serum beta-hCG levels and normal AFP
levels for age.

6. #
ETIOLOGY
• Although the exact cause of liver cancer
is unknown, there are a number of
genetic conditions that are associated
with an increased risk for developing
hepatoblastoma. They include:
• Beckwith-Wiedemann syndrome: This
syndrome is characterized by a
combination of Wilms' tumor, kidney
failure, genitourinary malformations and
gonad (ovaries or testes) abnormalities.

7. #
INCIDENCE
• primarily affects children from infancy to
about 5 years of age.
• Most cases appear during the first 18 months
of life.
• Hepatoblastoma affects white children more
frequently than black children, and is more
common in boys than girls up to about age 5,
when the gender difference disappears.
• It occurs more frequently in prematurely born
with very low birth weights.

8. #
• Familial adenomatous polyposis: This
is a group of rare inherited diseases of the
gastrointestinal tract.
• Hemihypertrophy: This condition is the
faster growth of one limb on one side of
the body in comparison with the other
side.
• Children who are exposed to hepatitis B
infection at an early age, or those who
have biliary atresia, are also at increased
risk for developing liver cancer.

9. #
RISK FACTORS
• Being male.
• Having the hepatitis B virus that was
passed from mother to child at birth.
• Certain genetic changes linked with
childhood hepatocellular carcinoma.
• Having one of the following conditions:
– Biliary cirrhosis,Alagille syndrome, Glycogen
storage disease, Progressive familial
intrahepatic disease, Tyrosinemia.

10. #
CLINICAL FEATURES
• The signs and symptoms of pediatric
hepatoblastoma often depend on the
size of the tumor and whether it has
spread to other parts of the body.

11. #
Symptoms may include:
• A large mass in the
abdomen
• Swollen abdomen
• Weight loss
• Decreased appetite
• Vomiting
• Jaundice (yellowing
of eyes and skin)
• Itchy skin
• Anemia

12. #
Other Symptoms
• Fever
• Back pain
• Decreased urination

13. #
DIAGNOSTIC
MEASURES
• Physical exam and history : An exam
of the body to check general signs of
health, including checking for signs of
disease, such as lumps or anything else
that seems unusual. A history of the
patient's health habits and past
illnesses and treatments will also be
taken.

14. #
• Alpha-fetoprotein (AFP) test. AFP
levels in the blood can be used both to
diagnose hepatoblastoma and to
monitor its response to treatment.
• Computerized tomography scan (CT
or CAT scan). It can show a mass in
the liver and whether the cancer has
spread to other organs such as the
lungs.

15. #
• Magnetic resonance imaging
(MRI). An MRI scan uses radio waves
and strong magnets with computer
technology. MRI shows more detailed
images than CT and ultrasound and
can help doctors see if the cancer has
invaded one of the major blood vessels
located near the liver.

16. #
• Complete blood count (CBC):
The number of red blood cells, white
blood cells, and platelets.
The amount of hemoglobin (the protein
that carries oxygen) in the red blood
cells.
The portion of the blood sample made
up of red blood cells

17. #
• Liver function tests : A procedure in
which a blood sample is checked to
measure the amounts of certain
substances released into the blood by
the liver. A higher than normal amount
of a substance can be a sign of liver
damage or cancer.

18. #
• Biopsy. A biopsy (a sample of tissue
that is removed and examined under a
microscope) will help confirm the
diagnosis and help plan treatment.
• Bone scan. This imaging test uses a
special radioactive material that is
injected into a vein. The substance
collects in areas of diseased bone and is
sensed by special cameras that pick up
radioactivity.

19. #
• Abdominal x-ray : An x-ray of the
organs in the abdomen. An x-ray is a
type of energy beam that can go
through the body onto film, making a
picture of areas inside the body

20. #
• Ultrasound exam: A procedure in
which high-energy sound waves
(ultrasound) are bounced off internal
tissues or organs and make echoes.
The echoes form a picture of body
tissues called a sonogram. The picture
can be printed to be looked at later. In
childhood liver cancer, an ultrasound
exam of the abdomen is usually done.

21. #
• Hepatitis assay : A procedure in which
a blood sample is checked for pieces of
the hepatitis virus.
• Epstein-Barr virus (EBV) test: A blood
test to check for antibodies to the EBV
and DNA markers of the EBV. These
are found in the blood of patients who
have been infected with EBV.

22. #
STAGING
• “Staging” is a process of testing and
analysis to determine how far the cancer
has spread. Once doctors know the stage
of the disease, they can choose the most
effective way to treat it. Additional tests
such as a CT scan, an MRI, an
ultrasound, or even surgery may be
required.There are two staging systems
for childhood liver cancer:
presurgical and postsurgical.

23. #
• Presurgical (before surgery) staging: The
stage is based on where the tumor has
spread within the four parts (sections) of
the liver, as shown by imaging procedures
such as MRI or CT. This staging system is
called PRETEXT.
• Stage I: Cancer is found in one section of
the liver. Three sections of the liver that
are next to each other do not have cancer
in them.
• Stage II: Cancer in one or two sections of
the liver. Two sections are next to each
other do not have cancer in them.

24. #
• Stage III: One of the following is true:
– Cancer is found in three sections of the
liver and one section does not have
cancer.
– Cancer is found in two sections of the liver
and two sections that are not next to each
other do not have cancer in them.
• Stage IV: Cancer is found in all four
sections of the liver.

25. #
• Postsurgical (after surgery) staging:
The stage is based on the amount of
tumor that remains after the patient has
had surgery.
• Stage I: The tumor was in the liver only
and all of the cancer was removed by
surgery.

26. #
• Stage II The tumor was in the liver only
and all of the cancer that can be seen
without a microscope was removed by
surgery. A small amount of cancer
remains in the liver, but it can be seen
only with a microscope, or the tumor
cells may have spilled into the abdomen
before surgery or during surgery.

27. #
• Stage III: One of the following is true:
– The tumor cannot be removed by surgery.
– Cancer that can be seen without a
microscope remains after surgery.
– The cancer has spread to nearby lymph
nodes.
• Stage IV: The cancer has spread to other
parts of the body.
• Recurrent childhood liver cancer is
cancer that was once treated but has now
come back, either in the liver or in other
parts of the body.

28. #
TREATMENT
• Chemotherapy
• Surgery
• Radiation therapy
• Percutaneous ethanol injection

29. #
Chemotherapy
• It is a key part of treatment for nearly all
infants and children with
hepatoblastoma. Chemotherapy is used
to both shrink the primary liver tumor
and to treat any hepatoblastoma that
has escaped outside the liver. This is
important for patients with all stages of
the disease, because cancer cells might
have traveled to other parts of the body

30. #
Surgery
• Doctors will operate to remove the cancer
whenever possible. Chemotherapy may be
given before surgery to shrink the tumor, or
after surgery to destroy any remaining cancer
cells.
• The different types of surgery performed are:
• Cryosurgery: Also known as cryotherapy,
this treatment uses an instrument, possibly
guided by ultrasound, to freeze and destroy
abnormal tissue, such as carcinoma in situ.

31. #
• Partial hepatectomy: The cancerous
part of the liver is removed in this
surgery. The part removed may be a
wedge of tissue, an entire lobe, or a
larger part of the liver, along with a
small amount of normal tissue around it.

32. #
• Total hepatectomy and liver
transplant: The entire liver is removed
and replaced with a healthy liver from a
donor in this surgery. A liver transplant
may be possible when cancer has not
spread beyond the liver and a donated
liver can be found. If the patient has to
wait for a donated liver, other treatment
is given as needed.

33. #
• Resection of metastases: This is
surgery to remove cancer that has
spread outside of the liver, such as to
nearby tissues, the lungs, or the brain.

34. #
Radiation therapy
• This treatment uses high-energy x-rays
or other types of radiation to kill or stop
cancer. The radiation may come from a
machine, or it may come from a
substance sealed in needles, seeds,
wires, or catheters placed directly into
the cancer.

35. #
Percutaneous
ethanol injection
• A small needle is used to inject ethanol
(alcohol) directly into a tumor to kill
cancer cells for this treatment.

36. #
PROGNOSIS
• There has been dramatic improvement in
outcomes over the last 20 to 25 years, with
overall survival rates reaching 70 percent.
• Survival is dependent upon staging at
diagnosis.
• Patients with completely resected tumors have
a greater than 85 percent chance of survival.
• For those with unresectable disease that
responds to chemotherapy, survival rates are
about 60 percent.

37. #
• The prognosis for an individual depends
on the stage of the disease:
• Stage I and II: When tumors
are located only in the liver and can be
surgically removed at diagnosis, the
published cure rate is greater than 85
percent when surgery and
chemotherapy are used together.

38. #
• Stage III: When tumors are located only
in the liver but cannot be surgically
removed at diagnosis, the published cure
rate is not as high, approximating 60
percent to 70 percent. The cure rate is
higher if complete surgical removal of all
tumors becomes possible. Anecdotally,
the cure rate appears to be increasing for
children with Stage III disease, at least in
part due to increasing availability of liver
transplantation as a surgical option.

39. #
• Stage IV: When tumors are located in
several places in the body, the
published cure rate is 20 percent to 30
percent using surgery and
chemotherapy. There is preliminary
evidence to suggest that the cure rate
may be increasing for children with
Stage IV disease, possibly due to more
aggressive chemotherapy options and
the increasing availability of liver
transplantation.

40. #
NURSING
MANAGEMENT
• Risk for Infection related to
immunosuppression, chemotherapy,
and presence of invasive lines
• Pain related to the abnormal growth of
tissues and compression of the
adjacent structures by the tumor

41. #
• Imbalanced Nutrition: Less Than Body
Requirements related to inability to ingest
or digest adequate quantities of food or
absorb adequate nutrients
• Ineffective Management of Therapeutic
Regimen related to complex therapy.
• Impaired Growth and Development
related to serious illness.
• Fatigue related to disease state
• Impaired Family Processes related to
situational crisis

42. #
BONE TUMOR

43. #
• Osteosarcoma and Ewing's sarcoma
are the most common malignancies of
bone tissues in children.
Osteosarcoma, the more common of
the two types, usually presents in bones
around the knee. Ewing's sarcoma may
affect bones of the pelvis, thigh, upper
arm, or ribs.

44. #
OSTEOSARCOMA

45. #
Definition
• Osteosarcoma is a type of cancer that
produces immature bone. It is the most
common type of cancer that arises in
bones, and it is usually found at the end
of long bones, often around the knee.

46. #
INCIDENCE
• Most people diagnosed with osteosarcoma
are under the age of 25, and it is thought
to occur more often in males than females.
• In children and young adults,
osteosarcoma usually develops in areas
where the bone is growing quickly, such as
near the ends of the long bones

47. #
• Most tumors develop in the bones around
the knee, either in the distal femur or the
proximal tibia
• The proximal humerus is the next most
common site.
• However, osteosarcoma can develop in
any bone, including the bones of the
pelvis (hips), shoulder, and jaw. This is
especially true in older adults.

48. #
ETIOLOGYAND RISK
FACTORS
• The exact cause of osteosarcoma is
unknown. However, a number of risk
factors are apparent, as follows…
• Rapid bone growth: Rapid bone growth
appears to predispose persons to
osteosarcoma, as suggested by the
increased incidence during the adolescent
growth spurt , and osteosarcoma's typical
location in the metaphyseal area adjacent
to the growth plate (physis) of long bones.

49. #
• Environmental factors: The only
known environmental risk factor is
exposure to radiation. Radiation-
induced osteosarcoma is a form of
secondary osteosarcoma
• Genetic predisposition : Bone
dysplasias, including Paget disease,
fibrous dysplasia, enchondromatosis,
and hereditary multiple exostoses and
retinoblastoma (germ-line form) are risk
factors.

50. #
• Osteosarcoma tends to occur in the
bones of the:
• Shin (near the knee)
• Thigh (near the knee)
• Upper arm (near the shoulder)
• Osteosarcoma occurs most commonly in
large bones in the area of bone with the
fastest growth rate. However, it can occur
in any bone.

51. #
TYPES OF
OSTEOSARCOMA
• Several subtypes of osteosarcoma can be
identified by how they look on x-rays and
under the microscope. Some of these
subtypes have a better prognosis (outlook)
than others.
• Based on how they look under the
microscope, osteosarcomas can be
classified as high grade, intermediate grade,
or low grade.

52. #
• High-grade osteosarcomas: These are
the fastest growing types of
osteosarcoma. When seen under a
microscope, they do not look like normal
bone and have many cells in the process
of dividing into new cells. Most
osteosarcomas that occur in children and
teens are high grade.

53. #
There are many types of high-grade
osteosarcomas :
• Osteoblastic
• Chondroblastic
• Fibroblastic
• Mixed
• Small cell
• Telangiectatic
• High-grade surface (juxtacortical high
grade)

54. #
• Other high-grade osteosarcomas
include:
• Pagetoid: a tumor that develops in
someone with Paget disease of the
bone
• Extra-skeletal: a tumor that starts in a
part of the body other than a bone
• Post-radiation: a tumor that starts in a
bone that had once received radiation
therapy

55. #
• Intermediate-grade
osteosarcomas: These uncommon
tumors fall in between high-grade and low-
grade osteosarcomas.
• Periosteal (juxtacortical intermediate
grade)

56. #
• Low-grade osteosarcomas: These
are the slowest growing
osteosarcomas. The tumors look more
like normal bone and have few dividing
cells when seen under a microscope.
• Parosteal (juxtacortical low grade)
• Intramedullary or intraosseous well
differentiated (low-grade central)

57. #
SIGNS AND
SYMPTOMS
• Bone fracture (may occur after a routine
movement)
• Bone pain
• Limitation of motion
• Limping (if the tumor is in the leg)
• Pain when lifting (if the tumor is in the
arm)
• Tenderness, swelling, or redness at the
site of the tumor

58. #
STAGING
Musculoskeletal Tumor Society (MSTS) Staging
System
• One system commonly used to stage
osteosarcoma is the MSTS system, also
known as the ENNEKING system. It is based
on 3 key pieces of information:
• The grade of the tumor (G)
• The extent of the main (primary) tumor (T)
• If the tumor has metastasized (spread) to
nearby lymph nodes (bean-sized collections of
immune system cells) or other organs (M)

59. #
Stage Grade Tumor Metastasis
IA G1 T1 M0
IB G1 T2 M0
IIA G2 T1 M0
IIB G2 T2 M0
III G1 / G2 T1 / T2 M1

60. #
• In summary:
• Low-grade, localized tumors are stage
I.
• High-grade, localized tumors are stage
II.
• Metastatic tumors (regardless of grade)
are stage III.

61. #
DIAGNOSTIC MEASURES
• Physical exam and history
• X-ray
• CT scan (CAT scan):
• MRI (magnetic resonance imaging)
• Biopsy: Core biopsy , Incisional
biopsy
• Light and electron microscopy

62. #
TREATMENT
• Treatment of osteosarcoma in children
includes chemotherapy (the use of
medical drugs to kill cancer cells and
shrink the cancer) followed by surgery (to
remove cancerous cells or tumors) and
then more chemo (to kill any remaining
cancer cells and minimize chances of the
cancer coming back).
• Surgery often can effectively remove
bone cancer, while chemotherapy can
help eliminate remaining cancer cells in
the body.

63. #
Surgical Treatment
• Surgical treatments for osteosarcoma
consist of either amputation or limb-
salvage surgery.
• Limb-salvage surgery- the affected
bone and muscle are removed, leaving
a gap in the bone that is filled by either
a bone graft or more often a special
metal prosthesis.

64. #
• The risk of infection and fracture is
higher with bank bone replacement and
therefore metal prostheses are more
commonly used for reconstruction of
the bone after removal of the tumor.

65. #
Amputation
• If the cancer has spread to the nerves
and blood vessels surrounding the
original tumor on the bone, amputation
is often the only choice.
• When osteosarcoma has spread to the
lungs or elsewhere, surgery might be
done to remove tumors in these distant
locations.

66. #
Chemotherapy
• Chemotherapy is usually given both before
and after surgery. It eliminates small
pockets of cancer cells in the body, even
those too small to appear on medical
scans.
• A child or teen with osteosarcoma is given
the chemotherapy drugs intravenously or
orally.

67. #
EWING'S SARCOMA

68. #
• Ewing’s sarcoma (ES) was first
described by James Ewing in 1921 as a
"diffuse endothelioma of bone" (Ewing
1921).
• Ewing sarcoma is a malignant
(cancerous) bone tumor that affects
children.

69. #
INCIDENCE
• Ewing sarcoma is the second most
common type of bone cancer in
children, but it is very rare. About 200
children and young adults are found to
have Ewing sarcoma each year in the
United States.
• About half of all Ewing sarcoma tumors
occur in children and young adults
between ages 10 and 20.

70. #
• This type of cancer is rarely found in
African-Americans and Asian-
Americans.
• It affects slightly more boys than girls.
• It does not appear to be inherited
(passed down in families).
• Although not often seen, Ewing
sarcoma can occur as a second cancer,
especially in patients treated with
radiation therapy.

71. #
CAUSES
• The exact causes of primary bone
cancer are unknown.
• The development of Ewing’s sarcoma
may be related in some way to times of
rapid bone growth
• Genetic exchange -- Most cases of
Ewing's sarcoma (85%) are the result of
a translocation between chromosomes
11 and 22.

72. #
CLINICAL
FEATURES
• Swelling and soreness around the
tumor area
• A low fever that at first may seem to be
caused by an infection
• Bone pain, especially pain that worsens
during exercise or at night
• Limping, which is caused by a tumor on
a leg bone

73. #
DIAGNOSTIC MEASURES
• A variety of tests and investigations are
needed to diagnose Ewing’s sarcoma,
including:
A physical exam and medical history
X-rays to locate and identify a potential
bone tumor, and chest x ray
Magnetic resonance imaging (MRI) scan
Computed tomography (CT or CAT) scan

74. #
• Bone scan, which uses an injected
radioactive material and a special
camera to identify potential spots in the
skeleton where the cancer may have
spread
• Positron emission tomography (PET)
scan, which also uses a radioactive
material and special camera to identify
other spots in the body that may have a
tumor
• Biopsy of the tumor

75. #
TREATMENT MEASURES
• A combination of various treatments is
used to treat Ewing's sarcoma.
Treatment will depend on a number of
factors, including the size and position
of the tumour. These include
chemotherapy, surgery and
radiotherapy.

76. #
Chemotherapy
• They are often given before surgery
and continued afterwards in order to
destroy any remaining cancer cells and
prevent the sarcoma from spreading.
Chemotherapy given in this way is
called adjuvant chemotherapy.

77. #
Surgery
• Amputation
• Limb-sparing surgery
• replacing the bone with a prosthesis (a
specially designed artificial part)
• replacing the bone with bone taken
from another part of the body (a bone
graft).

78. #
Radiotherapy
• External radiation uses machines
outside the body to deliver the X-ray
dose.
• Internal radiation uses needles,
seeds, wires or catheters (tubes) to
deliver the radiation directly into or
close to the cancer.

79. #
SIDE EFFECTS OF
TREATMENT
• Side effects can include:
• feeling sick (nausea) and being
sick vomiting
• hair loss
• increased risk of infection
• bruising and bleeding
• tiredness
• diarrhea

80. #
NURSING MANAGEMENT
• Nursing
Assessment
• Nursing Diagnoses
• Nursing
Interventions
• Evaluation

81. #

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