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Pediatrics > Oncology > Flashcards

Oncology Flashcards

1
Q

4th leading cause of death in children

A

CA

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2
Q

MC malignancy in children (nearly 1/3 of all pediatric cancers)

A

Acute Lymphoblastic Leukemia
(ALL)

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3
Q
  • Leukemic blasts replace bone marrow
  • Uncontrolled proliferation of immature lymphocytes
  • >25% blasts in bone marrow aspirate
  • Unknown cause - but may have a genetic link
  • Peak age: 4 years old (usually 2 - 10 yo)

dx?

A

Acute Lymphoblastic Leukemia
(ALL)

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4
Q

A blood stem cell goes through several steps to become a ? (3)

A

RBC
WBC
platelet

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5
Q

Where does CA start in regard to stem cell development?

A
  • after differentiating from a common lymphoid progenitor
  • before becoming a NK cell and smal lymphocyte
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6
Q

ALL develops from what type of stem cell?

A

lymphoid blast

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7
Q

s/s of ALL

A
  • dec bone marrow production of RBCs, WBCs or platelets
  • fevers, bone pain, pallor, petechiae (and easy bruising), purpura, progressive weakness, dyspnea, infection, fatigue
  • Enlarged liver and spleen, Anemia
  • LAD
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8
Q

w/u for ALL?

A
  1. CBC w/ diff
    - 95% have dec in at least one cell type - neutropenia, thrombocytopenia or anemia
    - ANC < 1k, even if WBC normal
  2. peripheral smear
  3. BMP, Uric Acid, LDH, PT/PTT
  4. DX: BM aspirate
  5. LP
  6. CXR
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9
Q
  • Lymphoblasts on peripheral smear
  • Pancytopenia (all 3 cell lines are decreased)

likely dx?

A

ALL

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10
Q

ALL - Abnormal white cells reveal hyperdiploidy and translocation of chromosomes ___ and ____

A

12 and 21

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11
Q

how to perform BM aspirate and bx?

A
  1. numbed
  2. insert Jamshidi needle (a long, hollow needle) hip bone
  3. Samples of blood, bone, and bone marrow are removed for examination under a microscope
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12
Q

mgmt for ALL

A
  • phase 1: Remission Induction - chemo + dex/pred +/- intrathecal MTX
  • Phase 2: Intensification Consolidation Phase - Intrathecal chemo +/- radiation
  • Phase 3: Continuation Therapy / Maintenance - Daily, oral chemo, wkly MTX, pulses of IV chemo and oral steroids

Treatment for 15 - 18 months, overall cure rate 90%

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13
Q

goal remission % during phase 1 for ALL?

A

95% remission on bone marrow aspirates

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14
Q

RF for ALL

A
  1. XR
  2. Downs syndrome
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15
Q
  • 2nd MC leukemia in children
  • Untreated can result in death within weeks
  • Primarily a cancer of bone marrow and lymph nodes
A

Acute Myeloid Leukemia
(AML)

Also called Acute Myelogenous Leukemia

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16
Q

s/s of AML

A
  • Early flu-like sx
  • Fatigue
  • Bleeding
  • Infection
  • Adenopathy
  • Skin nodules
  • HSM
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17
Q

w/u for AML

A
  1. CBC w/ diff
  2. BM aspirate
  3. LP, CXR
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18
Q
  • CBC reveals neutropenia, anemia, and thrombcytopenia
  • Bone marrow aspirate shows 30% blasts with histochemical staining and morphology of leukemic cells
  • Peripehral smear shows auer rods

dx?

A

AML

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19
Q

AML comes from what stem cell?

A

myeloblast - either myeloid stem cell or myeloid blast

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20
Q

general mgmt approach of AML

A
  1. Less responsive, more intense
  2. Chemo - systemic and intrathecal - aggressive induction therapy (80-85% remission)
  3. Radiation - sometimes
  4. BM transplant
  5. Cord blood transplant
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21
Q

tx phases of AML

A
  • Phase 1: Remission Induction Chemo - 1 month; Kill as many leukemic cells ASAP = cancer goes into remission
  • Phase 2: Consolidation Preventative - Stop spread of cancer to brain and spinal cord; Intrathecal chemo +/- radiation
  • Phase 3: Intensification - high dose of chemo; 1-2 tx lasting 2 mo x 9 mo
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22
Q

RF for AML

A
  • Ionizing radiation therapy
  • Previous chemo
  • Syndromes - Neurofibromatosis, Downs
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23
Q

disposition and prognosis of AML

A
  • Can reoccur
  • Remission when WBC in normal range
  • 75-85% remission
  • 35-50% long-term survival
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24
Q
  • Account for 50% of lymphomas in children
  • peak before adolescence and again >50 y/o
  • Germinal center B cells undergo malignant transformation for unknown reasons
A

Hodgkin’s Lymphoma

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25
Q

what are the 3 different forms of Hodgkin’s?

A
  • Childhood form - < 14 y, inc risk with large family size, dec socioeconomic status
  • Young Adult form - 15 - 34 y, Higher socioeconomic status and earlier birth order (oldest child)
  • Older Adult form
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26
Q

what decreases the risk of childhood form Hodgkin’s?

A

Early exposure to common infections in childhood

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27
Q
  • Painless cervical LAD (70-80%) or supraclavicular (25%)
  • Mediastinal mass (50-75%) and asx
  • Fatigue, wt loss, anorexia, night sweats and cough, fever

dx?

A

HL

B sx: F, wt loss, night sweats

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28
Q

w/u for HL?

A
  1. CBC
  2. ESR - elevated
  3. EBV titers
  4. LN bx
  5. BM aspirate; LP
  6. CXR, CT; bone scan; PET
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29
Q

Reed-sternberg cell is associated with what dx?

A

HL

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30
Q

suspected HL, Early bx of enlarged LN if:

A
  1. Lack of infectious cause
  2. Lymph node > 2 cm
  3. Supraclavicular node
  4. Abnormal CXR
  5. Increasing node size >2 wks of abx, or failing to dec in size in 4-6 weeks
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31
Q

tx and prognosis of HL

A
  • Chemotherapy
  • Disease free survival at 90-95% = Cure
  • Prognosis correlates with “B sx”
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32
Q
  • Immature lymphocytes grow out of control and fail to mature and accumulate in lymph tissue (nodes, spleen, thymus)
  • Arise from lymphoid cells
A

Non-Hodgkin’s Lymphoma (NHL)

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33
Q

3rd MC cancer in children?

A

Non-Hodgkin’s Lymphoma (NHL)

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34
Q

s/s of NHL

A

Cough, dyspnea, orthopnea, facial edema, LAD, mediastinal mass, pleural effusion, abdominal pain or distention

35
Q

MC site of tumor for NHL

A
  1. abdomen
  2. chest
36
Q
  • Boys 5 - 10 y/o
  • Originates from a B-cell phenotype
  • MC cases of NHL
  • Intra-abdominal tumor (MC), Jaw, or bone marrow
  • Chromosome 8
  • abdominal pain, distention, RLQ mass
  • Fastest growing tumor - can block bowels

which type of NHL

A

Burkitt Lymphoma (Small noncleaved cell lymphoma)

37
Q
  • Teen boys
  • Originate in immature T-cells (T-cell surface markers)
  • 25-30% of cases of NHL
  • Mediastinal mass 50-70%, LAD elevating diaphragm (MC)
  • MC airway compression sx: cough, dyspnea and orthopnea
  • Indistinguishable from ALL
  • Translocation of Chromosome 14q11 and 7 with deletion of chromosome 1

which type of NHL? mgmt?

A
  • Lymphoblastic Lymphoma
  • 15-18 month therapy like ALL
38
Q

how to differentiate lymphoblastic lymphoma vs ALL?

A

If >25% blasts in bone marrow - ALL not NHL

39
Q
  • B & T-cell or Histiocytes
  • < 15% of cases
  • MC Abdominal tumor

which type of NHL

A

Large Cell Lymphoma

40
Q

w/u for NHL

A
  • Bx + Histology with tissue removal
  • LDH - reflects tumor burden, marker for disease activity
  • CXR, CT chest, U/S, Bone scan, ECHO, EKG and PFTs
  • BM aspirate and bx
41
Q

RF for NHL

A
  1. organ transplants
  2. EBV
  3. HIV
  4. radiation exposure
42
Q

mgmt and prognosis for NHL

A
  • Chemo +/- Radiation
  • 5 year survival - after 5 years after dx of NHL-free = cured (90%)
43
Q

classic triad of a brain/spinal tumor?

A

morning HA, vomiting and papilledema (30%)

44
Q

s/s of brain/spinal tumor seen in older vs younger child?

A
  • Older children - personality changes, school issues (failure)
  • Younger children - Irritability, FTT, Dev Delay
45
Q
  • 25-30% of all childhood cancers
  • MC solid tumors of childhood
A

Brain & Spinal Tumors

46
Q

what tumors are seen with gial cells?

A
  1. Astrocytomas
  2. Ependymomas
47
Q

what tumors arise from nonglial cells?

A

Medulloblastomas and other primitive tumors

48
Q

w/u for brain/spinal tumors?

A

MRI w/ & w/o contrast

49
Q

what % of tumors occur in the tentorium and posterior fossa?

A

50/50

50
Q
  • MC brain tumor of childhood (about 50%)
  • In the cerebellum
A

Astrocytoma

51
Q

Grading for Astrocytoma?

A
  • WHO Grade I (Juvenile pilocytic) MC - slow growing and cystic; surgical removal only
  • WHO Grades I-II - slow growing, least serious; 80% of astrocytomas
  • WHO Grades III-IV - fast growing and malignant
52
Q

w/u and mgmt for Gliomas (Glial Cell Tumors)

A
  • MRI and confirmed via bx, CSF cytology
  • steroids + anticonvulsants before surgery; high dose systemic chemo after
  • 5-8 year survival 60-90%
53
Q
  • Middle of brainstem
  • Challenging to treat - majority no removal
  • Exclusively in school age children

which type of glioma?

A

brain stem

54
Q
  • Lining of ventricles or spinal cord near cerebellum
  • Blocks CSF flow
  • Slow growing
  • 5 year old peak age

which type of glioma?

A

Ependymoma

55
Q

Optic Nerve Gliomas Common in what condition?

A

Neurofibromatosis

56
Q
  • MC malignant brain tumor
  • 15% of children
  • Cerebellum
  • 5-10 y; Boys > Girls
  • Metastasis to spinal cord
A

Medulloblastomas (Non-Glial Cell)

57
Q

Tumor of nerve tissue
Extremely malignant neoplasm
MC extracranial solid cancer in childhood and MC cancer in infancy (< 5y/o (90%))

A

Neuroblastoma

58
Q

Neuroblastoma is primarily found where?

A

adrenal gland

59
Q

T/F: Neuroblastoma can be inherited

A

T

60
Q

s/s of neuroblastoma

A
  • constitutional sx - bone pain, abd pain, anorexia, wt loss, fatigue and F
  • Abdominal mass - firm, fixed, immobile, and irregularly shaped extending beyond midline (65%), adenopathy and HSM
    < 2 years old MC
61
Q

w/u for neuroblastoma and findings?

A
  1. CBC - anemic, thrombocytopenia
  2. Urine VMA/HVA test - Urinary Catecholamines
  3. Imaging: XR, CT, skeletal survey, bone scan
  4. Tissue bx for staging and BM aspirates and bx
62
Q

mgmt and prognosis for neuroblastoma

A
  • Surgical removal, chemo and radiation
  • < 1 y/o + small tumor - improved prognosis and possibly only surgical removal
  • High rate of metastasis and non-responsiveness to tx/high recurrence
63
Q

Aka “Wilms Tumor” – one or both kidneys

A

Nephroblastoma

64
Q

asx abdominal mass or swelling (>80%) - does not cross midline but may be mobile or displaced

dx?
w/u?

A
  • Nephroblastoma
  • U/S, CT or MRI
65
Q

2nd MC abdominal tumor?

A

Nephroblastoma

66
Q

Nephroblastoma affects what pt demographic MC?

A
  • African Americans
  • MC sporadic, genetic or syndromic link possible
  • 2-5 y/o
67
Q

how to stage for nephroblastoma?

A

Nephrectomy, Chemo or Radiation, Clinical Trials

68
Q

MC of met for nephroblastoma?

A

lungs

69
Q

difference between Osteosarcoma vs Ewing’s sarcoma?

A
  • Osteosarcoma - metaphysis
  • Ewing’s sarcoma - diaphysis
70
Q

Most common bone cancer in children and adolescents (60% of cases)

A

Osteosarcoma

71
Q

MC pt demographics of Osteosarcoma

A
  • Adolescents and young adults - 10 -19 years old MC
  • Males > Females
72
Q

Osteosarcoma affects what cells and genes?

A
  • osteoblasts
  • 2 suppressor genes (p53 and Rb mutations)
73
Q
  1. Adolescent growth spurt, rapid bone growth
  2. Taller than peers - greater increase in length and size of bones
  3. Metaphysis of bones
  4. MC Long, tubular bones (distal femur - 40%, tibia, humerus)
  5. lump, limp and wakes at night with pain

dx?
w/u?

A
  • Osteosarcoma
  • XR, CT/MRI, tissue sample, chest CT, bone scan
  • CBC, CMP with Alk phos and LDH for prognostic purposes
74
Q

MC met site for osteoscaroma?

A

lung

75
Q

mgmt and prognosis of osteosarcoma?

A
  • Chemo (PO + IV), Limb salvage (80% of pts)
  • Prognosis: bad; may have mets at time of dx, >70% receiving surgery alone develop pulmonary mets < 6 mo after
  • 55-80% survival at 5-year f/u (75% local lesion, 30% mets)
76
Q

RF for osteosarcoma?

A
  • age, male, tall stature, possibly radiation exposure
  • Strong genetic link with retinoblastoma
77
Q
  • Genetic change in a primitive cell after birth
  • inc risk for other cancers in future
  • 2nd MC bone cancer in children and adolescents
  • Diaphysis of long bones (middle - long part)
  • Misdiagnosed as “growing pains”
A

Ewing’s Sarcoma

78
Q

s/s of Ewing’s sarcoma?
w/u?

A
  • soreness at tumor site and swelling with warmth to touch
  • Worse with exercise or at night, tender lump or limp
  • XR, CT, MRI, bone scan and tumor biopsy; may have elevated LDH level
79
Q

mgmt for Ewing’s sarcoma

A
  • Chemo (IV) before surgery x 6-9 mo, then removal of tumor
  • PT and Rehab - 6-9 months
  • 50-70% long term disease free survival if no metastasis
80
Q

Tumor of the retina - necrotic in center with areas of hemorrhage

A

Retinoblastoma

81
Q

pathophys of Retinoblastoma

A

Arises from embryonic retinal cells - mutation in RB1 gene in long arm of chromosome 13

82
Q

what makes retinoblastoma more likely to be hereditary?

A

bilaterally

83
Q

s/s of retinoblastoma

A
  • Leukocoria - “white pupillary reflex” is a common sign (cat’s eye appearance) - 60%
  • Strabismus, painful, red eye w/ glaucoma , proptosis - less common
  • Asymmetry of eyes in photos
84
Q

w/u and mgmt for retinoblastoma?

A
  • CT scan, then MRI, bone scan, bone marrow and LP; Exam eye under anesthesia with dilated pupils
  • External beam radiation; Removal of eye
  • 5 year survival rate is 90%

Pediatrics (23 decks)

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