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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
Childhood cancer associated with Philadelphia chromosome?
Chronic myeloid leukemia
26
Myeloproliferative disorder characterized by uncontrolled proliferation of mature and maturing granulocytes
Chronic Myeloid Leukemia (CML)
27
CML is rare, accounting for only ____% of childhood leukemia
5%
28
A history of __________ is something to look for when suspecting CML
Ionizing radiation exposure | Because it can lead to the mutation —> Philadelphia chromosome
29
What IS the Philadelphia chromosome?
Reciprocal translocation between chromosomes 9 and 22 Associated with chronic myeloid leukemia (CML) - ask about exposure to ionizing radiation in patient history
30
CML presents in what three phases?
Chronic phase —> accelerated phase —> blast phase
31
Clinical presentation of CML
20-50% asymptomatic Bone pain B symptoms (fever, night sweats, fatigue) Pallor, ecchymosis Massive splenomegaly, variable hepatomegaly
32
Lab findings for CML patients
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)
33
Treatment for CML
Tyrosine kinase inhibitor allows for dysregulated cellular proliferation But Hematopoietic Stem Cell Transplant is the only consistently curative intervention
34
50% of childhood lymphomas are ___________
Hodgkin Lymphoma
35
Gender predominance in HL
4:1 male predominance in the first decade
36
Epstein-Barr virus is associated with what childhood cancer?
Hodgkin Lymphoma
37
Diagnostic peripheral smear finding for HL
Reed-Sternberg cells Terminal-center B cells that have undergone malignant transformation
38
Prognosis for HL in children vs adults
Children with Hodgkin lymphoma have a better response to treatment than adults 90% 5-10 year survival rate
39
Clinical presentation for HL
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
40
What is superior vena cava syndrome?
Dyspnea, cough, orthopnea, facial/upper extremity edema due to mediastinal mass Associated with Hodgkin Lymphoma
41
Dx of HL
Tissue biopsy showing presence of Reed-Sternberg cells
42
How do we stage HL patients?
CXR, CT scan (chest, abdomen, and pelvis) and bone marrow biopsy
43
Treatment for HL
Chemo Radiation if indicated Autologous HSCT may improve survival rates
44
5th most common pediatric cancer for patients <15 years of age
Non-Hodgkin Lymphoma (NHL)
45
Gender predominance for Non-Hodgkin Lymphoma
3:1 male predominance
46
Non-Hodgkin Lymphoma can arise ...
In any site of lymphoid tissue Can spread anywhere but usually spreads to distant nodes
47
Conditions associated with Non-Hodgkin Lymphoma
Congenital and acquired immunodeficiency syndrome EBV
48
Unlike adult NHL, virtually all childhood Non-Hodgkin Lymphomas are...
Rapidly proliferating, high-grade, diffuse malignancies
49
Clinical findings in Non-Hodgkin Lymphoma
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
Dx of NHL is via...
Tissue biopsy
51
Treatment for Non-Hodgkin Lymphoma
Chemotherapy - WATCH FOR TUMOR LYSIS SYNDROME Autologous or allogenic HSCT is an option for those who relapse
52
Prognosis for Non-Hodgkin Lymphoma
90% have long term survival
53
The most common solid tumor of childhood
Brain tumor Children have a better prognosis than adults though, so there’s that
54
Modalities for evaluating for brain tumors
Performed even in neonatal/infant exams Measure head circumference Observe gait
55
30% of pediatric brain tumors present with this triad...
AM headache Vomiting Papilledema
56
SSx of brain tumors more common in younger children
Vomiting, unsteadiness, lethargy, irritability, macrocephaly | Failure to thrive, delayed development
57
SSx of brain tumors more common in older children
Headache, visual symptoms, seizure, focal neurological deficits School failure and personality changes
58
Preferred diagnostic study for pediatric brain tumors
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
Two categories of brain tumors accounting to the cell of origin
Glial tumors • Most common = astrocytomas • Ependymomas Nonglial tumors • Medulloblastoma
60
Initial approach for the treatment of pediatric brain tumors
Surgical removal of as much of the tumor as possible Add radiation and chemo when indicated
61
Prognosis for pediatric brain tumors
60-90% 5-10 year survival with low grade astrocytoma
62
Most common abdominal tumor in children
Neuroblastoma BUT, it makes up only 7-10% of pediatric malignancies
63
Most common solid neoplasm outside of the CNS
Neuroblastoma
64
Most common primary site for Neuroblastomas
Adrenal gland (40% of them start here)
65
How soon do neuroblastomas usually get diagnosed?
90% are diagnosed < 5 years of age 50% are diagnosed < 2 years of age
66
Clinical manifestations of neuroblastomas
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
Laboratory findings for neuroblastomas
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
Treatment for neuroblastoma
The mainstay of therapy is surgical resection coupled with chemo Surgery alone if low grade Radiation sometimes necessary as well
69
Prognosis for neuroblastoma
Long term survival <40% in children with high-risk disease
70
Second most common abdominal tumor in children
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
Clinical findings in nephroblastoma
Asymptomatic abdominal mass/swelling (83%) • Smooth, firm, well demarcated • Rarely crosses midline • can extend inferiorly into the pelvis Fever, hematuria, HTN
72
Diagnosing nephroblastoma
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
Treatment for nephroblastoma
Surgical exploration, chemo, and radiation 90% overall 5 year survival
74
Most common primary bone malignancy in pediatric patients
Osteosarcoma
75
Most common demographics for osteosarcoma patients
Male predominance with peak incidence between 13-16 years (or 10-19) - the time of rapid bone growth
76
Most common location for osteosarcoma to develop
In long bones (metaphysic) 40% in distal femur
77
Cardinal signs of bone tumors
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
Primary site for metastasis of osteosarcoma
Lungs
79
Imaging modalities for osteosarcoma
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
Treatment and prognosis for osteosarcoma
Tx with surgery and chemo Prognosis is 70-75% long term survival with a localized tumor
81
Second most common primary bone tumor in pediatrics
Ewing Sarcoma
82
Who gets Ewing sarcoma?
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
Clinical findings for Ewing Sarcoma
Worsening localized pain +/- swelling Bone pain worse at night Fatigue, fever, +/- weight loss
84
Dx of Ewing Sarcoma
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
Treatment and prognosis for Ewing sarcoma
Tx with chemo, surgery, radiation, or a combo if needed Prognosis is 70-75% long term survival if small localized tumor
86
10-15% of all cancers diagnosed in the first year of life
Retinoblastoma 90% of tumors diagnosed before age 5
87
Most common intraocular tumor in pediatrics
Retinoblastoma BL in 20-30% of cases Causes 5% of cases of childhood blindness
88
DDx to consider if BL white pupillary reflex
Retinopathy of prematurity (more likely to be bilateral than retinoblastoma)
89
Genetic factors related to retinoblastoma
Can be inherited - parent may be a silent carrier
90
Metastasis is rare with retinoblastoma, but if they occur...
Death within a year is typical
91
Clinical presentation of retinoblastoma
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
Diagnosing retinoblastoma
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
Treatment of retinoblastoma
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
Most common soft tissue sarcoma in childhood
Rhabdomyosarcoma Rare: 7% of all childhood cancers 70% of children are diagnosed before age 10 years Slight male predominance
95
Any body part can be affected in rhabdomyosarcoma but _____________ most common
Head and neck Can resemble fat, fibrous tissue and muscle
96
Clinical presentation of rhabdomyosarcoma
Painless, progressively enlarging mass Orbital rhabdomyosarcoma: • Proptosis/exophthalmos Bladder Rhabdomyosarcoma: • Hematuria • Urinary obstruction (palpate distended bladder) • Pelvic mass
97
Diagnosis of rhabdomyosarcoma
Imaging: xray and CT or MRI of mass Chest CT to rule out pulmonary mets Skeletal survey to rule out bone mets
98
Treatment of rhabdomyosarcoma
Any combo of surgery, chemo, radiation Prognosis: 70-75% 3 year survival if localized
99
________ of liver masses found in childhood are malignant
2/3rds 90% of hepatic malignancies are either hepatoblastoma or hepatocellular carcinoma
100
___________ is often elevated and is an excellent marker for response to treatment of hepatic tumors
Serum alpha-fetoprotein (AFP)
101
Clinical presentation of hepatic tumors
Usually present because of an enlarging abdomen - 10% found on routine exam
102
Dx and Tx of Hepatic tumors
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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