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Peds II Unit I > Peds Oncololgy > Flashcards

Peds Oncololgy Flashcards

1
Q

Leading cause of death in pediatric patients aged 1-19

A

Cancer

Still, it’s pretty rare

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2
Q

Why is pediatric cancer difficult to diagnose in early stages?

A

Non specific associate Sx

Mimic other more common concerns

Lack of clinician experience

Clinicians reluctant to consider it as a Dx

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3
Q

Optimal treatment for childhood cancer requires…

A

High level of suspicion

Early referral to PEDS onco

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4
Q

Warning signs for childhood cancer (using the “CHILD CANCER” acronym)

A

Continued unexplained weight loss
Headaches (often w vomiting or early night/early morning)
Increased swelling or pain in bones, joints, back, legs
Lump/mass in abdomen, neck, chest, pelvis (LAD that DOESNT go away)
Development of excessive bruising, bleeding, rash

Constant infections
A whitish color behind the pupil
Nausea which persists or vomiting without nausea
Constant tiredness or noticeable paleness
Eye or vision changes that occur suddenly and persist
Recurrent fevers of unknown origin

Seriously, this is the worst acronym ever

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5
Q

The most common malignancy of childhood

A

Acute Lymphoblastic Leukemia (ALL)

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6
Q

ALL results from uncontrolled proliferation of …

A

Immature lymphocytes

85% B-precursor lineage

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7
Q

Peak incidence for ALL

A

2-5 years of age (85% Dx 2-10 years)

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8
Q

Genetic factor contributing to ALL

A

Down syndrome (14%)

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9
Q

Associations with ALL

A

Viral infections

Exposure to radiation

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10
Q

Clinical presentation of ALL

A 
Intermittent fever, fatigue, pallor
Bleeding (50%) - petechiae, purpura
BONE PAIN (25%) - esp pelvis, vertebral bodies, and legs (consider in a little kid limping for unknown reason or refusing to walk)
Hepatosplenomegaly (64/61%)
Lymphadenopathy (50%)
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11
Q

Laboratory findings in ALL

A

Anemia and/or thrombocytopenia with normal or depressed WBD (but WBC can be >50K in 20% of patients - more likely to have increased WBC if acute)

Differential shows neutrocytopenia (ANC<1000)

Peripheral smear shows LYMPHOBLASTS

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12
Q

Dx standard for ALL

A

Bone marrow biopsy: leukemic blasts (lymphoblasts) replacing normal marrow

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13
Q

Treatment modalities for ALL

A

Chemotherapy

Hematopoietic stem cell transplant (HSCT) - best if from a matched sibling

Tx may take 2-3 years to complete

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14
Q

Prognosis for ALL

A

> 85% overall survival rate

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15
Q

Oncologic emergency that must be anticipated when initiating chemotherapy (esp if starting aggressive chemo regime)

A

Tumor Lysis Syndrome

Massive tumor cell lysis:
• Hyperkalemia
• Hyperuricemia
• Hyperphosphatemia
• Acute renal failure
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16
Q

Acute Myeloid Leukemia (AML) arises from…

A

Precursor cells (Myeloblasts) committed to the myeloid line of cellular development —> reduced capacity to differentiate into more mature cells

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17
Q

AML accounts for only ______% of leukemia in children

A

15-25%

BUT it’s responsible for 1/3 of deaths from leukemia in children/teenagers

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18
Q

Predisposing factors for AML

A

Often no identifiable risk factors

Congenital: Down Syndrome, Neurofibromatosis

Environmental exposures: radiation, benzene (smoking), and previous chemotherapy

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19
Q

Clinical findings in AML

A

Fatigue, pallor, bleeding, or infection (fever)

CNS involvement present in 5-15% at diagnosis (HA, lethargy, mental status changes, cranial nerve palsies)

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20
Q

Laboratory findings in AML

A

Anemia (44%)

Thrombocytopenia (33%)

Neutropenia (ANC<1000 in 69%)

WBC >100,000 in 20% of patients at diagnosis (HYPERLEUKOCYTOSIS)

Auer rods on peripheral smear

Circulating myeloblasts ≥20%

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21
Q

Auer Rods on a peripheral smear is pathognomonic for…

A

Acute Myeloid Leukemia (AML)

Auer rods = granular structures in the cytoplasm

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22
Q

A WBC > 75,000 is a medical emergency called ___________, associated with life-threatening complications from _________.

A

Hyperleukocytosis —> AML

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23
Q

Dx of Acute Myeloid Leukemia (AML)

A

Requires BOTH:

BM biopsy: 20% or more blasts
The leukemic cells must be of myeloid origin

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24
Q

Tx for AML

A

Chemo

HSCT

Prognosis: 65-75% overall survival rate

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25
Q

Childhood cancer associated with Philadelphia chromosome?

A

Chronic myeloid leukemia

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26
Q

Myeloproliferative disorder characterized by uncontrolled proliferation of mature and maturing granulocytes

A

Chronic Myeloid Leukemia (CML)

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27
Q

CML is rare, accounting for only ____% of childhood leukemia

A

5%

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28
Q

A history of __________ is something to look for when suspecting CML

A

Ionizing radiation exposure

Because it can lead to the mutation —> Philadelphia chromosome

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29
Q

What IS the Philadelphia chromosome?

A

Reciprocal translocation between chromosomes 9 and 22

Associated with chronic myeloid leukemia (CML) - ask about exposure to ionizing radiation in patient history

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30
Q

CML presents in what three phases?

A

Chronic phase —> accelerated phase —> blast phase

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31
Q

Clinical presentation of CML

A

20-50% asymptomatic

Bone pain

B symptoms (fever, night sweats, fatigue)

Pallor, ecchymosis

Massive splenomegaly, variable hepatomegaly

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32
Q

Lab findings for CML patients

A

Anemia, thrombocytosis, and marked leukocytosis (>100,000/µl)

Peripheral smear: myeloid cells in all stages of maturation, increased basophils and blast cells

Blast cells >20% in a blast crisis

CML confirmed if Philadelphia chromosome present (not always there but diagnostic if it is)

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33
Q

Treatment for CML

A

Tyrosine kinase inhibitor allows for dysregulated cellular proliferation

But Hematopoietic Stem Cell Transplant is the only consistently curative intervention

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34
Q

50% of childhood lymphomas are ___________

A

Hodgkin Lymphoma

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35
Q

Gender predominance in HL

A

4:1 male predominance in the first decade

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36
Q

Epstein-Barr virus is associated with what childhood cancer?

A

Hodgkin Lymphoma

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37
Q

Diagnostic peripheral smear finding for HL

A

Reed-Sternberg cells

Terminal-center B cells that have undergone malignant transformation

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38
Q

Prognosis for HL in children vs adults

A

Children with Hodgkin lymphoma have a better response to treatment than adults

90% 5-10 year survival rate

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39
Q

Clinical presentation for HL

A

PAINLESS cervical (70-80%) or supraclavicular (25%) LAD

MEDIASTINAL MASS (75%) - caution for superior vena cava syndrome

Weight loss, fever, night sweats, fatigue (B SYMPTOMS)

Splenomegaly +/- hepatomegaly

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40
Q

What is superior vena cava syndrome?

A

Dyspnea, cough, orthopnea, facial/upper extremity edema due to mediastinal mass

Associated with Hodgkin Lymphoma

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41
Q

Dx of HL

A

Tissue biopsy showing presence of Reed-Sternberg cells

42
Q

How do we stage HL patients?

A

CXR, CT scan (chest, abdomen, and pelvis) and bone marrow biopsy

43
Q

Treatment for HL

A

Chemo

Radiation if indicated

Autologous HSCT may improve survival rates

44
Q

5th most common pediatric cancer for patients <15 years of age

A

Non-Hodgkin Lymphoma (NHL)

45
Q

Gender predominance for Non-Hodgkin Lymphoma

A

3:1 male predominance

46
Q

Non-Hodgkin Lymphoma can arise …

A

In any site of lymphoid tissue

Can spread anywhere but usually spreads to distant nodes

47
Q

Conditions associated with Non-Hodgkin Lymphoma

A

Congenital and acquired immunodeficiency syndrome

EBV

48
Q

Unlike adult NHL, virtually all childhood Non-Hodgkin Lymphomas are…

A

Rapidly proliferating, high-grade, diffuse malignancies

49
Q

Clinical findings in Non-Hodgkin Lymphoma

A

Fast, usually 1-3 weeks

Common: enlarging, non-tender LAD

May see abdominal pain, fevers, cough, dyspnea, weight loss, night sweats

Hepatomegaly/Splenomegaly in advanced stage

50
Q

Dx of NHL is via…

A

Tissue biopsy

51
Q

Treatment for Non-Hodgkin Lymphoma

A

Chemotherapy - WATCH FOR TUMOR LYSIS SYNDROME

Autologous or allogenic HSCT is an option for those who relapse

52
Q

Prognosis for Non-Hodgkin Lymphoma

A

90% have long term survival

53
Q

The most common solid tumor of childhood

A

Brain tumor

Children have a better prognosis than adults though, so there’s that

54
Q

Modalities for evaluating for brain tumors

A

Performed even in neonatal/infant exams

Measure head circumference

Observe gait

55
Q

30% of pediatric brain tumors present with this triad…

A

AM headache
Vomiting
Papilledema

56
Q

SSx of brain tumors more common in younger children

A

Vomiting, unsteadiness, lethargy, irritability, macrocephaly

Failure to thrive, delayed development

57
Q

SSx of brain tumors more common in older children

A

Headache, visual symptoms, seizure, focal neurological deficits
School failure and personality changes

58
Q

Preferred diagnostic study for pediatric brain tumors

A

MRI is first choice - HOWEVER, it’s hard to get kids to lay still for the required 30 minutes

CT is an alternative (only takes 10 min)

Final diagnosis is by tissue biopsy

59
Q

Two categories of brain tumors accounting to the cell of origin

A

Glial tumors
• Most common = astrocytomas
• Ependymomas

Nonglial tumors
• Medulloblastoma

60
Q

Initial approach for the treatment of pediatric brain tumors

A

Surgical removal of as much of the tumor as possible

Add radiation and chemo when indicated

61
Q

Prognosis for pediatric brain tumors

A

60-90% 5-10 year survival with low grade astrocytoma

62
Q

Most common abdominal tumor in children

A

Neuroblastoma

BUT, it makes up only 7-10% of pediatric malignancies

63
Q

Most common solid neoplasm outside of the CNS

A

Neuroblastoma

64
Q

Most common primary site for Neuroblastomas

A

Adrenal gland (40% of them start here)

65
Q

How soon do neuroblastomas usually get diagnosed?

A

90% are diagnosed < 5 years of age

50% are diagnosed < 2 years of age

66
Q

Clinical manifestations of neuroblastomas

A

Abdominal mass (65%) - firm, fixed, and irregular in shape, often extending beyond midline

Bone pain from metastatic disease (60%)

Fever, weight loss, irritability, abdominal pain, anorexia

67
Q

Laboratory findings for neuroblastomas

A

Anemia is present in 60% of children

Urinary catecholamines elevated in 90% at diagnosis (b/c adrenal gland is common primary site)

Chemicals made by the nervous system (dopamine, NE, epi)

68
Q

Treatment for neuroblastoma

A

The mainstay of therapy is surgical resection coupled with chemo

Surgery alone if low grade

Radiation sometimes necessary as well

69
Q

Prognosis for neuroblastoma

A

Long term survival <40% in children with high-risk disease

70
Q

Second most common abdominal tumor in children

A

Nephroblastoma or Wilms Tumor (2nd after neuroblastoma)

5-6% of cancers in children <15 years

Most common between 2-5 years of age

Most have solitary tumor (5-7% have bilateral kidney involvement)

71
Q

Clinical findings in nephroblastoma

A

Asymptomatic abdominal mass/swelling (83%)
• Smooth, firm, well demarcated
• Rarely crosses midline
• can extend inferiorly into the pelvis

Fever, hematuria, HTN

72
Q

Diagnosing nephroblastoma

A

Imaging - ultrasound or CT of the abdomen
CT of the chest (b/c commonly spreads to lungs)

Dx by histologic confirmation from biopsy or from surgical excision

73
Q

Treatment for nephroblastoma

A

Surgical exploration, chemo, and radiation

90% overall 5 year survival

74
Q

Most common primary bone malignancy in pediatric patients

A

Osteosarcoma

75
Q

Most common demographics for osteosarcoma patients

A

Male predominance with peak incidence between 13-16 years (or 10-19) - the time of rapid bone growth

76
Q

Most common location for osteosarcoma to develop

A

In long bones (metaphysic)

40% in distal femur

77
Q

Cardinal signs of bone tumors

A

Bone pain at the site

Mass formation

Fracture through the area of cortical destruction

Presentation: bone pain over involved area w or w/o associated soft tissue mass/swelling and antalgic gait

78
Q

Primary site for metastasis of osteosarcoma

A

Lungs

79
Q

Imaging modalities for osteosarcoma

A

X-ray: will see destruction of normal trabecular pattern and irregular margins

MRI: to evaluate soft tissue involvement

Bone scan and CT scan of the chest for evaluation of possible metastasis

Dx via imaging, with histological confirmation from tissue

80
Q

Treatment and prognosis for osteosarcoma

A

Tx with surgery and chemo

Prognosis is 70-75% long term survival with a localized tumor

81
Q

Second most common primary bone tumor in pediatrics

A

Ewing Sarcoma

82
Q

Who gets Ewing sarcoma?

A

Primarily affects white males during their second decade of life

Most often arises in the long bones (diaphysis)
• Extremities and pelvis
• Rarely in soft tissue

83
Q

Clinical findings for Ewing Sarcoma

A

Worsening localized pain +/- swelling

Bone pain worse at night

Fatigue, fever, +/- weight loss

84
Q

Dx of Ewing Sarcoma

A

Imaging: X-ray and CT/MRI of primary lesion

Staging: CT scan of chest, bone scan, bone marrow aspirated and biopsy

Dx confirmed by tissue biopsy

85
Q

Treatment and prognosis for Ewing sarcoma

A

Tx with chemo, surgery, radiation, or a combo if needed

Prognosis is 70-75% long term survival if small localized tumor

86
Q

10-15% of all cancers diagnosed in the first year of life

A

Retinoblastoma

90% of tumors diagnosed before age 5

87
Q

Most common intraocular tumor in pediatrics

A

Retinoblastoma

BL in 20-30% of cases

Causes 5% of cases of childhood blindness

88
Q

DDx to consider if BL white pupillary reflex

A

Retinopathy of prematurity (more likely to be bilateral than retinoblastoma)

89
Q

Genetic factors related to retinoblastoma

A

Can be inherited - parent may be a silent carrier

90
Q

Metastasis is rare with retinoblastoma, but if they occur…

A

Death within a year is typical

91
Q

Clinical presentation of retinoblastoma

A

Leukocoria (white pupillary reflex) in patients under 2
• most common sign (60% of patients) but not definitive

Other SSx: strabismus, nystagmus, and red inflamed eye

92
Q

Diagnosing retinoblastoma

A

Detailed ophthalmologist exam under anesthesia

Chalky off white retinal mass with soft, friable consistency is diagnostic

Ocular ultrasound

MRI of the brain and orbits

93
Q

Treatment of retinoblastoma

A

Variety of options available for children, including several “vision-sparing” therapies

External beam irradiation

Chemo if confined to the globe

Prognosis: 90% 5 year survival if confined to the retina

94
Q

Most common soft tissue sarcoma in childhood

A

Rhabdomyosarcoma

Rare: 7% of all childhood cancers

70% of children are diagnosed before age 10 years

Slight male predominance

95
Q

Any body part can be affected in rhabdomyosarcoma but _____________ most common

A

Head and neck

Can resemble fat, fibrous tissue and muscle

96
Q

Clinical presentation of rhabdomyosarcoma

A

Painless, progressively enlarging mass

Orbital rhabdomyosarcoma:
• Proptosis/exophthalmos

Bladder Rhabdomyosarcoma:
• Hematuria
• Urinary obstruction (palpate distended bladder)
• Pelvic mass

97
Q

Diagnosis of rhabdomyosarcoma

A

Imaging: xray and CT or MRI of mass

Chest CT to rule out pulmonary mets

Skeletal survey to rule out bone mets

98
Q

Treatment of rhabdomyosarcoma

A

Any combo of surgery, chemo, radiation

Prognosis: 70-75% 3 year survival if localized

99
Q

________ of liver masses found in childhood are malignant

A

2/3rds

90% of hepatic malignancies are either hepatoblastoma or hepatocellular carcinoma

100
Q

___________ is often elevated and is an excellent marker for response to treatment of hepatic tumors

A

Serum alpha-fetoprotein (AFP)

101
Q

Clinical presentation of hepatic tumors

A

Usually present because of an enlarging abdomen - 10% found on routine exam

102
Q

Dx and Tx of Hepatic tumors

A

Imaging: abdominal ultrasound, CT scan, or MRI

Tx: Surgery or chemo, but complete resection is essential for survival

Liver transplantation when tumors are unresectable

Prognosis: up to 1/3 with complete resection are long term survivors

Peds II Unit I (4 decks)

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