Ped Oncology - Exam 3 Flashcards

1
Q

Cancer is the ____ cause of death in children

A

4th

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2
Q

_____ is the MC malignancy in children. What is the underlying cause? What percent?

A

ALL

Leukemic blasts replace bone marrow leading to uncontrolled proliferation of immature lymphocytes

More than ____ blasts in the bone marrow aspirate

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3
Q

What is the MC pt for ALL? What age range? What is the peak age?

A

male white

2-10 years old

peak age is 4

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4
Q

What parent cell does ALL affect? Draw the tree

A

lymphoid stem cell -> lymphoid blast that then differentiates into B and T lymphocytes

ALL affects the lymphoid blast cell

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5
Q

Intermittent fevers, bone pain, pallor, petechiae, easy bruising, purpura, progressive weakness, dyspnea, infection, fatigue, bone pain

What am I?
What are the s/s related to?
What are 3 common PE findings?
What is a common lab finding?

A

ALL

Related to decreased bone marrow production of RBCs, WBCs or platelets

enlarged liver and spleen, lymphadenopathy

may have anemia

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6
Q

What lab is the most useful in diagnosis ALL? **What will you see on peripheral smear?

A

CBC with diff - most useful

lymphoblasts on peripheral smear

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7
Q

What will the CBC show in a pt with ALL? What will the ANC level be? What will the CBC show?

A

95% have a decrease in at least one cell type

ANC usually <1000, even if WBC count is normal

pancytopenia: all 3 lines are decreased

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8
Q

What am I? What dx?

A

Lymphoblasts

ALL

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9
Q

_____ is needed for confirmation in ALL. What will it show?

A

Bone marrow aspirate and biopsy

> 25% blasts in aspirate

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10
Q

Why should you do a lumbar puncture in ALL?

A

Lumbar puncture to check for CNS involvement and to administer intrathecal chemo

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11
Q

What is the genetic underlying cause of ALL?

A

translocation of chromosomes 12 and 21

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12
Q

What are the 3 phases of tx for ALL?

A

phase 1: Remission Induction Phase

phase 2: Intensification Consolidation Phase

phase 3: Continuation Therapy / Maintenance

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13
Q

What is the tx for ALL? What % does this usually cure?

A

Chemo (ex. vincristine, asparaginase, etc.) with dexamethasone/prednisone and/or intrathecal methotrexate (MTX)

95% remission on bone marrow aspirates

then move on to phase 2: Intrathecal chemo & possibly radiation to kill lymphoblasts in meninges

then phase 3:
Daily, oral chemo, weekly methotrexate, pulses of IV chemo and oral steroids

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14
Q

What are risk factors for ALL? What is the cure rate?

A

Prenatal and postnatal exposure to x-rays

Down’s Syndrome

overall cure rate is over 90%

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15
Q

______ is the 2nd MC leukemia in children. What does it primarily affect?

A

AML

Primarily a cancer of bone marrow and lymph nodes

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16
Q

How does Acute Myeloid Leukemia
(AML) compare to ALL?

A

AML is more aggressive than ALL and can result in death within weeks if not treated

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17
Q

Early flu-like symptoms
Fatigue
Bleeding
Infection
Adenopathy
Skin nodules
Hepatosplenomegaly

What am I?
How do you dx?

A

AML

CBC with diff and bone marrow aspirate with bx

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18
Q

What will the CBC with diff show in AML? What will the bone marrow aspirate show?

A

neutropenia (69%)
anemia (44%)
thrombocytopenia (33%)

Again , pancytopenia

30% or more BLASTS with histochemical staining and morphology of leukemic cells

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19
Q

Draw the diagram that shows where the cancer is located for AML on the stem cell tree

A
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20
Q

What am I? What dx?

A

Auer rods

AML

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21
Q

What is the tx for AML?

A

Chemo - systemic and intrathecal - aggressive induction therapy (80-85% remission)

bone marrow transplant

cord blood transplant

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22
Q

What is the expanded tx for AML?

A

remission induction chemo for 1 month to try and kill as many leukemic cells as possible!!

then phase 2: Stop spread of cancer to brain and spinal cord even if none detected
Intrathecal chemo and sometimes radiation

then phase 3: 1-2 treatments lasting 2 months at a time for approximately 9 months

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23
Q

How long does phase 3: intensification therapy last in AML?

A

Once in remission - high dose of chemo to kill any remaining cells

1-2 treatments lasting 2 months at a time for approximately 9 months

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24
Q

What are the risk factors for AML? What % of patients will go into remission? What is the long term prognosis?

A

Neurofibromatosis
Downs
Ionizing radiation therapy
Previous chemo

75-85% remission (compared with 98% ALL)

Still only about 35-50% long-term survival

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25
_______ account for 50% of lymphomas in children. What sex? What age range?
Hodgkin’s Lymphoma males before adolescence (10-19) and then again over 50
26
________ cell in Hodgkin's Lymphoma undergo malignant transformation for unknown reasons
Germinal center B cells
27
What are the 3 different forms of Hodgkin's lymphoma?
childhood form young adult form older adult form
28
When is childhood form of Hodgkin's lymphoma seen? What increases the risk? What decreases risk?
childhood form: HL younger than 14 increased risk: large family size decreased socioeconomic status decreased risk: Early exposure to common infections in childhood decreases risk (think kids who go to daycare)
29
What age range do you see young adult form of HL? What are the 2 risk factors?
15 - 34 years old Higher socioeconomic status earlier birth order (oldest child)
30
Painless cervical lymphadenopathy Mediastinal mass Fatigue, weight loss, anorexia, night sweats and cough What am I? What are the "B" symptoms?
Hogdkin's Lymphoma Fever (>38 degrees Celsius) Weight Loss (10% or more) Drenching night sweats
31
What will the CBC show in a pt with HL? ESR? _____ approximately 20% of these tumors are associated with it
CBC often normal Elevated ESR elevated EBV titers
32
______ is needed for dx of HL. **What will it show? How would you want to confirm?
bx of node with FNA **Reed-Sternberg cell on biopsy confirm with bone marrow aspirate and bx and LP to see if the cancer has reached the bone and spinal fluid
33
What am I? What dx? What is an important thing to note?
Reed-Sternberg cell Hodgkin's Lymphoma abnormal lymphocytes that may contain more than 1 nucleus
34
What are the suspicious lymph node bx criteria?
Lack of infectious cause Lymph node > 2 cm Supraclavicular node Abnormal CXR Increasing node size after 2 weeks of antibiotic treatment, OR failing to decrease in size in 4-6 weeks
35
What is the tx for HL? What is disease free survival percentage? What does the prognosis correlate with?
chemotherapy!! Disease free survival at 90-95% = Cure Prognosis correlates with “B symptoms”
36
What is the underlying cause of non-Hodgkin's lymphoma? It arises from ______
Immature lymphocytes grow out of control and fail to mature and accumulate in lymph tissue (nodes, spleen, thymus) arises from lymphoid cells
37
_____ is the 3rd MC cancer in kiddos. What sex? What race?
Non-Hodgkin's lymphoma male white> black
38
What are the 3 organ systems involved in NHL?
spleen thymus lymph
39
Cough, dyspnea, orthopnea, facial edema, lymphadenopathy, mediastinal mass, pleural effusion, abdominal pain or distention What am I? Where are the 2 MC sites for these tumors to spread?
NHL abdomen #1 chest #2
40
What are the 3 types of NHL?
Burkitt Lymphoma (Small noncleaved cell lymphoma Lymphoblastic Lymphoma Large Cell Lymphoma
41
What is the MC pt in Burkitt Lymphoma?
boys 5-10 years old
42
Burkitt's lymphoma originates from _____ phenotype. How common is it when compared to other types of NHL?
Originates from a B-cell phenotype 40-50% of cases of NHL
43
Burkitt Lymphoma tend to present with ______ tumor. What is the genetic cause?
Intra-abdominal tumor that grows very fast and may block bowels Chromosome 8
44
What is the MC pt with lymphoblastic NHL? How common is it? What cell does it originate from?
teen boys 25-30% of cases of NHL Originate in immature T-cells -> T-cell surface markers
45
What is the genetic cause of lymphoblastic NHL? How long is therapy for?
Translocation of Chromosome 14q11 and 7 with deletion of chromosome 1 15-18 month therapy like ALL
46
How can you tell lymphoblastic lymphoma NHL apart from ALL?
Indistinguishable from ALL If >25% blasts in bone marrow - ALL not NHL
47
cough, dyspnea and orthopnea Mediastinal mass 50-70%, Lymphadenopathy elevating diaphragm (50-80%) What am I? How common is it?
Lymphoblastic Lymphoma NHL 25-30% of cases of NHL
48
What cell lines are affected in large cell lymphoma? What tumor? How common is it?
Abdominal tumor most common B and T-cell or Histiocytes < 15% of cases
49
How do you dx NHL? What lab reflects tumor burden and serves as a marker for disease activity?
Bone marrow aspirate and biopsy LDH
50
What are the risk factors for NHL? How would you describe the tumor?
organ transplants EBV HIV radiation exposure tumor is typically aggressive but responds well to treatment
51
What is the prognosis in NHL?
5 year survival: those who live at least 5 years after diagnosis and free of disease are likely cured (90%)
52
What are the triad of brain/spinal tumors? How common are they?
morning headache, vomiting and papilledema (30%) 25-30% of all childhood cancers
53
What is the MC solid tumor of childhood? ____ and _____ are glial cell tumors
brain/spinal tumors Glial Cell - Astrocytomas and Ependymomas
54
Non-glial cell tumors include ______ and _____. How do you dx?
Medulloblastomas and other primitive tumors MRI w/ and w/o contrast
55
What is the brain location breakdown of brain tumors?
56
Where are astrocytomas commonly found?
in the cerrebellum closer to the front
57
_______ the MC brain tumor of childhood and are found in the _______. How do you dx?
astrocytomas cerebellum MRI mainstay of diagnosis and confirmed via biopsy, CSF cytology
58
What are the different grades of astrocytomas? What are the major differences?
WHO grade I- IV WHO grade I-II: slow growing, least serious which make up 80% of astrocytomas WHO grades III-IV: fast growing and malignant
59
What is the MC types of astrocytoma? What is the tx?
WHO Grade I (Juvenile pilocytic) most common - slow growing and cystic requiring surgical removal only
60
What is the tx for an astrocytoma? What is the 5-8 year survival rate?
Treatment - steroids and anticonvulsants prior to surgery followed by high dose systemic chemo 5-8 year survival: 60-90%
61
Where are brain stem gliomas found? What is the tx? What age group?
Middle of brainstem Challenging to treat - majority no removal- NO SX!!! usually just chemo/radiation Exclusively in school age children
62
_______ are ______ that are found in the lining of the ventricles or spinal cord near cerebellum. Why are they bad?
Ependymoma gliomas blocks CSF flow
63
Are ependymoma fast or slow growing? What age?
Slow growing 5 year old peak age
64
What type of gliomas are common to see in pts that also have neurofibromatosis?
Optic Nerve Gliomas
65
______ is the MC malignant brain tumor. What age range? What sex? Where do they like to metastasize?
Medulloblastomas (Non-Glial Cell) in the cerebellum 5-10 years old Boys > Girls spinal cord
66
What is a neuroblastoma? What age? Where are they primarily found?
Tumor of nerve tissue that is extremely malignant usually under age of 5 (90%) Primarily found in the adrenal gland
67
______ are the MC extracranial solid cancer in childhood and most common cancer in infancy. Where does it arise from? What sex?
Neuroblastoma Arises from neural crest tissue of synthetic ganglia or adrenal medulla Boys > Girls
68
Where are the 2 MC locations to find a neuroblastoma? Will it cross over the midline?
#1 adrenal gland #2 spinal nerve roots neuroblastomas WILL cross over midline!!
69
Abdominal mass - firm, fixed, immobile, and irregularly shaped extending beyond midline (65%), adenopathy and HSM Bone pain, abdominal pain, anorexia, weight loss, fatigue and fever - constitutional symptoms What am I? What age? **What is the highlighted finding?
Neuroblastoma < 2 years old at diagnosis most common Abdominal mass - firm, fixed, immobile, and irregularly shaped *extending beyond midline*
70
What lab tests should you order in neuroblastoma?
CBC for anemia (60%) **Urinary Catecholamines (Urine VMA/HVA test) - tumor secrets this and it will be increased in 90% of patients at diagnosis, thrombocytopenia
71
What is the tx for neuroblastoma? What is an improved prognosis? How likely it is to metastasize?
Surgical removal, chemo and radiation <1 year old and small tumor - improved prognosis and possibly only surgical removal High rate of metastasis and non-responsiveness to treatment/ high recurrence
72
What is another name for a nephroblastoma?
"Wilms Tumor" on one or both kidneys
73
How will a nephroblastoma present?
Asymptomatic abdominal mass or swelling (>80%) that does NOT cross midline but may be mobile or displaced
74
_____ is the 2nd MC abdominal tumor. Where does it arise from? What race? How are they usually found?
Nephroblastoma Arises from kidney African Americans Presents as an asymptomatic abdominal mass found by parents or increased size of abdomen
75
What age range is associated with a nephroblastoma? What imaging should you order?
Uncommon over age of 5 (usually 2-5 y/o) U/S and then CT abdomen
76
Abdominal pain, fever, N/V, hematuria, elevated BP - but these constitutional symptoms not common, usually asymptomatic Abnormally large abdomen What am I? Where is the MC site of metastasis? What is the 5 year survival?
Nephroblastoma lungs 5 year survival around 90%
77
Where in the bone are osteosarcomas more likely to be found? Ewing's sarcoma?
Osteosarcoma: metaphysis Ewing’s sarcoma: diaphysis
78
______ are the MC bone cancer in children and adolescents. What age range? What sex?
Osteosarcoma 10 -19 years old most common males> females
79
Why does osteosarcoma occur?
Cancer affecting osteoblasts 2 suppressor genes (p53 and Rb mutations)
80
What are some PE findings in osteosarcoma?
often found in adolescents around the time of a major growth spurt due to rapid bone growth kiddos are sometimes taller than peers due to greater increase in length and size of bones
81
What bones are osteosarcomas more likely to involve? Where along the bone?
Long, tubular bones primarily affected (distal femur - 40%, tibia, humerus) metaphysis
82
lump, limp and wakes at night with pain What am I? Where are metastasis more likely to occur? What labs?
Osteosarcoma pulmonary metastasis CBC, CMP with Alk phos and LDH
83
What is the tx for osteosarcoma? Has a strong genetic link with ______
chemo both PO and IV and limb salvage retinoblastoma
84
What is the prognosis for osteosarcoma? What is the 5 year survival rate?
15 - 20% already have mets at time of diagnosis and >70% receiving surgery alone develop pulmonary mets within 6 months after surgery 55-80% survival at 5 year follow-up
85
What are the risk factors for osteosarcoma?
age, male, tall stature, possibly radiation exposure
86
What are some xray findings associated with osteosarcoma?
sun burst appearance Codman's triangle
87
What is the underlying cause of Ewing's sarcoma? What does it due to future risks?
Genetic change in a primitive cell after birth Increased risk for other cancers in future
88
_____ is the 2nd MC bone cancer in children and adolescents. Where does it occur in the bone? What sex?
Ewing’s Sarcoma Diaphysis of long bones ( middle - long part ) males> females
89
What is Ewing's sarcoma commonly misdiagnosed as?
Misdiagnosed as “growing pains”
90
Present with soreness at tumor site and swelling with warmth to touch Worse with exercise or at night, tender lump or limp What am I? What lab? What is the tx?
Ewing's sarcoma may have elevated LDH Chemo (IV) before surgery for 6-9 months, then removal of tumor
91
Ewing's sarcoma has a ___ long term disease free survival if no metastasis
50-70%
92
What is a retinoblastoma? What does it arise from?
Tumor of the retina - necrotic in center with areas of hemorrhage Arises from embryonic retinal cells - mutation in RB1 gene in long arm of chromosome 13
93
How is a retinoblastoma commonly found? What is the underlying cause if bilateral?
Most picked up by the parents or caretakers , Cat’s eye Bilateral eyes - hereditary
94
____ is known as a "white pupillary reflex" and is a common sign for retinoblastoma
Leukocoria
95
How do you dx a retinoblastoma?
Diagnosis by CT scan of orbits, followed by MRI, bone scan, bone marrow and LP Examination of eye under anesthesia with dilated pupils
96
What is the tx for a retinoblastoma?
External beam radiation is mainstay of tx if that does NOT work then removal of eye and 90% need no further treatment
97
What is the 5 year survival rate of a retinoblastoma?
5 year survival rate is 90%
98