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Pediatrics Exam 3 > Pediatric Oncology > Flashcards

Pediatric Oncology Flashcards

1
Q

Acute Leukemias

A
  • Myeloid or Lymphoid
  • Most Pediatric Acute Leukemias have
    NO clear cause
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2
Q

What differentiates the Two main types of Acute Leukemia?

A
  • Epidemiology is different
  • Diagnostic findings are Similar, a few important differences
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3
Q

Pathophysiology of ALL

A

Lymphoid Progenitor Cells
in the bone marrow become
malignant & begin to proliferate
uncontrolled

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4
Q

Pathophysiology of AML

A

Myeloid Progenitor Cells become malignant & proliferate uncontrolled

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5
Q

_____ = MOST COMMON childhood malignancy!

A

ALL

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6
Q

ALL epidemiology

A
  • Incidence of ALL peaks at the age of 5 years (3-7)
    ■ ALL = MOST COMMON childhood malignancy!
  • Incidence ↓ after 5 yo, then ↑ after 50 yo
  • 80% in kids
  • 20% in adults
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7
Q

Epidemiology of AML

A
  • 80% of all new cases of
    ADULT leukemias
  • Adults over 50 years
    of age (mean = 60)
  • It can occur in children & young adults, but less often
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8
Q

Which leukemia is associated with Trisomy 21?

A

AML

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9
Q

Clinical Presentation (AML & ALL)

A
  • Chief complaint may be fever, fatigue,
    weight loss, irritability, arthralgias,
    bleeding
    (often the gums → “pink in the sink”)
  • If CNS involvement, may have headache,
    stiff neck, visual disturbances,
    or vomiting
  • Gingival hypertrophy from leukemic
    infiltration is common
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10
Q

Physical exam of ALL and AML may reveal:

A

○ Pallor
○ Petechiae
○ Ecchymosis
○ Papilledema
○ Retinal hemorrhages
○ Focal neurologic deficits
○ Hepatomegaly (more common in ALL)
○ Splenomegaly (more common in ALL)
○ Lymphadenopathy (more common in ALL)

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11
Q

You are more likely to see hepatomegaly, splenomegaly and lymphadenopathy in ____

A

ALL

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12
Q

Hyperleukocytosis

A

■ High circulating blasts in the blood
leads to clumping blasts in capillaries
● Impaired circulation
● Headache, confusion, dyspnea
● Tx = emergent leukapheresis
& chemotherapy

● MOST EXTREME presentation

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13
Q

MOST EXTREME presentation in AML and ALL?

A

Hyperleukocytosis

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14
Q

Diagnosis of Acute leukemias

A
  • Bone Marrow Biopsy
    ○ Hypercellular
    ■ > 20% Blasts
  • CBC
    o Normocytic anemia
    o Thrombocytopenia
    o Often neutropenia
  • WBC count can be low, normal, or high
  • Chest x-ray
  • ALL
  • Possible Anterior Mediastinal mass
    ○ specifically T-cell ALL
  • Peripheral Blood Smear in
    AML may show Auer Rods
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15
Q

Hallmark of both AML and ALL?

A
  • Pancytopenia and circulating blasts
  • Lymphoid or myeloid
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16
Q

CNS infiltration is common in ____

A

ALL

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17
Q

Management & Referral of Acute leukemias

A
  • Acute Leukemia tx’d by Hematology Oncology
  • Treatment for both ALL & AML includes “Induction Chemotherapy”
  • CNS infiltration is common in ALL
  • Intrathecal chemotherapy
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18
Q

What is “Induction Chemotherapy”?

A
  • aggressive combination chemo
  • goal = “inducing” remission
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19
Q

Childhood ALL – ___ % tx
success within 4 weeks

A

98

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20
Q

Adult ALL & AML outcomes

A
  • Remission ~60-80%
  • Complete cure is less likely in adults
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21
Q

Hodgkin’s Lymphoma epidemiology

A

● Bimodal age distribution
1. ↑ incidence in young-teen to young-adulthood
■ Average around 20 years of age (20’s)
2. 2nd peak occurs later in life at about 50 years of age (50’s)
● ~85 % male

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22
Q

Characterized by the presence of
Reed-Sternberg cells

A

Hodgkin’s Lymphoma

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23
Q

Some studies have suggested a
correlation between EBV infection
& lymphomas with _____

A

Hodgkin’s Lymphoma

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24
Q

Hodgkin’s Lymphoma Pathophysiology

A

○ Starts in a localized region of lymph
nodes
○ Spreads in an orderly, predictable
fashion to adjacent sites of lymph tissue
■ Opposite of Non-Hodgkin’s
○ Often starts in the cervical or
supraclavicular regions

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25
Q

Hodgkin’s Lymphoma Presentation

A

■ Painless lump in neck area
■ Fevers/chills
■ Night sweats
■ Weight loss
■ Anorexia
■ General malaise
■ Excessive fatigue in children
○ May report a febrile pattern called Pel-Ebstein pattern

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26
Q

Interestingly, adults with Hodgkin’s will
experience pain/soreness at lymph
node with _____

A

ETOH ingestion

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27
Q

Hodgkin’s Lymphoma diagnosis

A

Lymph node biopsy
■ Excisional biopsy is preferred over fine-needle aspiration
○ CT chest/abdomen/pelvis required for Ann Arbor Staging

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28
Q

Management & Referral of Hodgkin’s Lymphoma

A

○ General surgeon or ENT surgeon for biopsy
○ If diagnosis is established, contact Oncology right away to set up consultation

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29
Q

Prognosis of Hodgkin’s Lymphoma

A

■ 5 year survival rate for Stage 1 is > 95%
● For Stage 4, about 65%

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30
Q

Non-Hodgkin’s Lymphoma

A

A group of dozens of forms of Lymphoma all classified as
Non-Hodgkin’s Lymphoma (Non-Hodgkin’s Disease)

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31
Q

T/F Non-Hodgkin’s is much more common than Hodgkin’s

A

T

32
Q

Characteristic Signs & Symptoms of Non-Hodgkin’s Lymphoma

A

○ Like Hodgkin’s, NHL is characterized by
painless lymphadenopathy
■ However, NHL lymphadenopathy seems to be more generalized rather than localized to one region
■ 2/3rd have retroperitoneal, mesenteric, or pelvic nodal involvement

33
Q

Constitutional symptoms (e.g. fever, night sweats, weight
loss) can occur, but a much less common in ____

A

NHL

34
Q

Non-Hodgkin’s Lymphoma diagnosis

A

Tissue biopsy is required for
definitive diagnosis
○ Peripheral blood smear is usually normal,

35
Q

Treatment of NHL

A

○ If diagnosis is confirmed, treatment usually involves a chemotherapy & possibly radiation
■ Treatment is based on stage & apparent aggression of the malignancy
■ If indolent, may not treat initially
○ 70-95% of pediatric patients with NHL can be cured with current therapies

36
Q

____ tumors are the most common solid tumors seen in
children

A

Brain & spinal cord

37
Q

Brain & Spinal Cord Tumors clinical presentation

A

○ Clinical presentation
depends upon tumor
location
■ Headaches
■ Bulging fontanelle
■ Irritability
■ Nausea & vomiting
■ Imbalance (cerebellar involvement)
■ Neck or back pain
■ Eye/facial movement problems

38
Q

Diagnostic Techniques for Brain & Spinal Cord Tumors

A

If H&P suggests concern for CNS tumor,
imaging is immediately indicated
○ CT scan possible
○ Prefer MRI with & without contrast
■ Brain or spine, depending on
presentation
■ Biopsy/Excision confirms Dx

39
Q

The most common type of primary
CNS malignancy seen in children!

A

Medulloblastoma

40
Q

Medulloblastoma frequently spreads to
other locations within the CNS by way
of ____

A

the CSF seeding

41
Q

Pilocytic Astrocytoma

A

○ Tumors arising from astrocytes
○ “Low-grade” or “High-grade”
○ Slow growing, well demarcated
considered mostly noncancerous

42
Q

Most common pediatric brain tumor

A

Pilocytic Astrocytoma

43
Q

Management of Brain & Spinal Cord Tumors

A

○ Treatment depends on the type of tumor, location of the tumor, & age of the child
○ Surgery, radiation therapy, & chemotherapy are often utilized
○ In general, 3 out of 4 children will survive at least 5 years after being diagnosed

44
Q

____ is the 2nd most common solid tumor in children

A

Neuroblastoma

45
Q

Neuroblastoma

A

Neuroendocrine cancer arising
from the neural crest element of
the sympathetic nervous system
Most commonly originates in an adrenal gland
● May also develop in nervous tissue of the chest, abdomen, pelvis, or neck
● Incidence peaks before 2 yo

46
Q

Neuroblastoma Most commonly originates in _____

A

an adrenal gland

47
Q

Neuroblastoma clinical presentation

A

○ Abdominal mass or swelling
○ Neck mass
○ Horner’s Syndrome with Chest Mass
○ Back pain
○ Bowel or bladder dysfunction
○ Bulging eye with bruising
○ Bone pain
○ Anemia bleeding

48
Q

Diagnostic Techniques for Neuroblastoma

A

■ CT scan of the body
■ MRI scan is preferred if concern
of spinal column involvement
■ Nuclear medicine scans
■ CMP & CBC
■ Bone marrow studies

49
Q

Management of Neuroblastoma

A

○ Surgery for complete total excision is preferred as long as vital organs have not been invaded
○ Chemotherapy & radiation

50
Q

Retinoblastoma

A

Rare tumor of the immature cells of the retina
* Generally occurs in children less than
5 years old
○ Peak incidence at 18-20 mo

51
Q

T/F Retinoblastoma can run in families

A

T
○ Important that siblings of affected
children are evaluated by an
ophthalmologist as well
○ 1⁄3 of cases are bilateral

52
Q

White light reflex is called

A

Leukocoria

53
Q

Common Clinical Presentation of Retinoblastoma

A

○ Worsening vision over weeks to months
○ Misaligned eyes develop (weeks to
months)
■ A growing retinal tumor starts to
displace the eye
○ Change in the pupil size
Leukocoria = white light reflex

54
Q

Retinoblastoma diagnostic techniques

A

○ MRI of the brain with contrast
○ Eye exam under anesthesia performed by an ophthalmologist, with possible biopsy (unless classic appearance)

55
Q

Management of retinoblastoma

A

○ Chemotherapy is often utilized
○ Radiation is avoided, but is
sometimes necessary
○ If recovering full vision with
chemotherapy is not possible,
removal of the eye is
recommended
○ Untreated retinoblastoma is
deadly, however, with
current treatments, prognosis
is generally favorable if
caught early

56
Q

Osteosarcoma

A

Most common malignant bone tumor in youth

57
Q

Most Common Location of Osteosarcoma = _____

A

Metaphysis of long bones
○ Distal femur, proximal humerus, tibia or fibula,

58
Q

Common Clinical Presentation of Osteosarcoma

A

○ Pain in the affected bone (most common)
○ Visible swelling or malformation
○ Limited range of motion
○ Pathologic fracture may be first sign

59
Q

Diagnostic Techniques for Osteosarcoma

A

○ X-ray is a good initial test
○ MRI or CT for more detail
○ CT of the chest, abdomen, pelvis should be performed to rule out metastases
○ Biopsy is always required
○ Labs: ↑ALP, LDH, ESR

60
Q

Osteosarcoma management

A

○ Most children can be cured
○ Treatment depends on size,
position, & stage of the disease
○ Chemotherapy is often used
to shrink the tumor before surgery
○ Radiation is rarely used;
only if surgery is not an option
○ Tumor removal is necessary; may require amputation

61
Q

Ewings sarcoma

A

Less common bone cancer that osteosarcoma
● May arise anywhere in the body, but more common in the
pelvis, axial skeleton, femur
● Although more often
occurs in bone, it can
develop in the soft
tissues near bone as well
● Most common during
teenage years

62
Q

Common Clinical Presentation of Ewings sarcoma

A

○ Bone pain is the most common symptom
○ Swelling or enlargement of affected bone
○ Pathologic fracture with minor event
○ Fever
○ Fatigue
○ Weight loss
○ Anemia

63
Q

Diagnostic Techniques of Ewings sarcoma

A

○ XR, CT, MRI, Biopsy
○ Same as osteosarcoma

64
Q

Management of Ewings sarcoma

A

○ Usually a combination of chemotherapy,
radiation therapy, & surgery
○ Depends on size & spread of tumor
○ Most patients can be cured

65
Q

Rhabdomyosarcoma

A

Cancer of muscle tissue
● Accounting for approximately
3-4% of childhood cancers
● It can appear in any muscle in the
body, but more often arises in
head/neck region, extremities,
abdominal or chest wall, orbit, or
pelvic cavity (psoas or pelvic floor)

66
Q

Rhabdomyosarcoma is Most commonly diagnosed in patients under the age of ___

A

6

67
Q

Rhabdomyosarcoma clinical presentation

A

○ Symptoms usually arise as the mass
grows to a point where it pushes
on other tissue (mass effect)

68
Q

Diagnostic Techniques for Rhabdomyosarcoma

A

○ CT scan or MRI will both provide very useful images of the mass, with characteristics suggesting a muscular origin
○ Biopsy is necessary for diagnosis

69
Q

Rhabdomyosarcoma management

A

○ Chemotherapy is usually used initially to
shrink the size of the mass
○ Surgery and/or radiation therapy are
then utilized to treat local sites of tumor
visible on imaging
○ >60% of patients will survive
○ If detected early, cure rate = 80%+

70
Q

Nephroblastoma is also known as a

A

“Wilm’s Tumor”

71
Q

Nephroblastoma

A

“Wilm’s Tumor”
● Cancer of the kidneys that occurs in
children under the age of 9 years
○ Peak incidence at ages 2-5
○ About 10% of Wilm’s Tumor
patients will have malignant
tumor in BOTH kidneys

72
Q

Wilm’s can spread to lymph nodes in:

A
  • Abdomen
  • Chest
  • Occasionally to the liver
73
Q

Nephroblastoma clinical presentation

A

○ Abdominal swelling
○ Palpable abdominal mass
○ Abdominal pain
○ Poor appetite
○ Constipation or bowel obstruction
○ Hematuria
○ Hypertension
○ Fatigue & generalized malaise

74
Q

Diagnostic Techniques for a Nephroblastoma

A

○ After a detailed H&P reveals a mass…
■ CT of chest, abdomen, & pelvis
■ Oral & IV contrast
■ Ultrasound may be used to assess
vascular flow around the tumor
■ Urinalysis looking for hematuria
■ CMP & CBC
■ If both kidneys are involved, biopsy
may be done to confirm Dx

75
Q

Management of a Nephroblastoma

A

○ If the tumor only involves one
kidney & is not extending into major blood vessels, surgery will entail removal of the entire involved kidney
○ Children do well with 1 remaining
kidney
○ Chemotherapy is important in tx
○ If both kidneys are involved,
chemotherapy will be used to
shrink the kidneys before
considering resection surgery
○ Overall cure rate = 85%

Pediatrics Exam 3 (7 decks)

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