Pediatrics > Oncology > Flashcards

Oncology Flashcards

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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
what are the 3 different forms of Hodgkin's?
* **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**
26
what decreases the risk of childhood form Hodgkin's?
Early exposure to common infections in childhood
27
* **Painless cervical LAD** (70-80%) or supraclavicular (25%) * **Mediastinal mass** (50-75%) and asx * Fatigue, **wt loss**, anorexia, **night sweats** and cough, **fever** dx?
HL *B sx: F, wt loss, night sweats*
28
w/u for HL?
1. CBC 2. ESR - elevated 3. **EBV titers** 4. **LN bx** 5. **BM aspirate;** LP 5. CXR, CT; bone scan; PET
29
Reed-sternberg cell is associated with what dx?
HL
30
suspected HL, Early bx of enlarged LN if:
1. Lack of infectious cause 1. Lymph node > 2 cm 1. Supraclavicular node 1. Abnormal CXR 1. Increasing node size >2 wks of abx, or failing to dec in size in 4-6 weeks
31
tx and prognosis of HL
* Chemotherapy * Disease free survival at 90-95% = Cure * Prognosis correlates with “B sx”
32
* Immature lymphocytes grow out of control and fail to mature and accumulate in lymph tissue (nodes, spleen, thymus) * Arise from **lymphoid cells**
Non-Hodgkin’s Lymphoma (NHL)
33
3rd MC cancer in children?
Non-Hodgkin’s Lymphoma (NHL)
34
s/s of NHL
Cough, dyspnea, orthopnea, facial edema, LAD, **mediastinal mass**, pleural effusion, **abdominal pain or distention**
35
MC site of tumor for NHL
1. **abdomen** 1. chest
36
* 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
Burkitt Lymphoma (Small noncleaved cell lymphoma)
37
* 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?
* Lymphoblastic Lymphoma * 15-18 month therapy like ALL
38
how to differentiate lymphoblastic lymphoma vs ALL?
If >25% blasts in bone marrow - ALL not NHL
39
* **B & T-cell** or Histiocytes * **< 15%** of cases * **MC Abdominal tumor** which type of NHL
Large Cell Lymphoma
40
w/u for NHL
* 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
RF for NHL
1. organ transplants 1. EBV 1. HIV 1. radiation exposure
42
mgmt and prognosis for NHL
* Chemo +/- Radiation * 5 year survival - after 5 years after dx of NHL-free = cured (90%)
43
classic triad of a brain/spinal tumor?
**morning HA, vomiting and papilledema** (30%)
44
s/s of brain/spinal tumor seen in older vs younger child?
* Older children - personality changes, school issues (failure) * Younger children - Irritability, FTT, Dev Delay
45
* 25-30% of all childhood cancers * MC solid tumors of childhood
Brain & Spinal Tumors
46
what tumors are seen with gial cells?
1. Astrocytomas 1. Ependymomas
47
what tumors arise from nonglial cells?
Medulloblastomas and other primitive tumors
48
w/u for brain/spinal tumors?
MRI w/ & w/o contrast
49
what % of tumors occur in the tentorium and posterior fossa?
50/50
50
* MC brain tumor of childhood (about 50%) * In the cerebellum
Astrocytoma
51
Grading for Astrocytoma?
* 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
w/u and mgmt for Gliomas (Glial Cell Tumors)
* **MRI** and confirmed via bx, CSF cytology * steroids + anticonvulsants before surgery; high dose systemic chemo after * 5-8 year survival 60-90%
53
* Middle of brainstem * Challenging to treat - majority no removal * Exclusively in school age children which type of glioma?
brain stem
54
* Lining of ventricles or spinal cord near cerebellum * **Blocks CSF flow** * Slow growing * 5 year old peak age which type of glioma?
Ependymoma
55
Optic Nerve Gliomas Common in what condition?
Neurofibromatosis
56
* **MC malignant brain tumor** * 15% of children * Cerebellum * 5-10 y; Boys > Girls * **Metastasis to spinal cord**
Medulloblastomas (Non-Glial Cell)
57
Tumor of **nerve** tissue **Extremely malignant neoplasm** MC extracranial solid cancer in childhood and **_MC cancer in infancy_** (< 5y/o (90%))
Neuroblastoma
58
Neuroblastoma is primarily found where?
adrenal gland
59
T/F: Neuroblastoma can be inherited
T
60
s/s of neuroblastoma
* **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
w/u for neuroblastoma and findings?
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
mgmt and prognosis for neuroblastoma
* 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
Aka “Wilms Tumor” – one or both kidneys
Nephroblastoma
64
**asx abdominal mass** or swelling (>80%) - **_does not cross midline_** but may be mobile or displaced dx? w/u?
* Nephroblastoma * U/S, CT or MRI
65
2nd MC abdominal tumor?
Nephroblastoma
66
Nephroblastoma affects what pt demographic MC?
* African Americans * MC sporadic, genetic or syndromic link possible * 2-5 y/o
67
how to stage for nephroblastoma?
Nephrectomy, Chemo or Radiation, Clinical Trials
68
MC of met for nephroblastoma?
lungs
69
difference between Osteosarcoma vs Ewing’s sarcoma?
* Osteosarcoma - metaphysis * Ewing’s sarcoma - diaphysis
70
Most common bone cancer in children and adolescents (60% of cases)
Osteosarcoma
71
MC pt demographics of Osteosarcoma
* Adolescents and young adults - 10 -19 years old MC * Males > Females
72
Osteosarcoma affects what cells and genes?
* osteoblasts * 2 suppressor genes (p53 and Rb mutations)
73
1. Adolescent growth spurt, rapid bone growth 1. Taller than peers - greater increase in length and size of bones 1. **Metaphysis** of bones 1. **MC Long, tubular bones** (**distal femur** - 40%, tibia, humerus) 2. lump, limp and wakes at night with pain dx? w/u?
* Osteosarcoma * XR, CT/MRI, tissue sample, chest CT, bone scan * CBC, CMP with Alk phos and LDH for prognostic purposes
74
MC met site for osteoscaroma?
lung
75
mgmt and prognosis of osteosarcoma?
* 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
RF for osteosarcoma?
* age, male, tall stature, possibly radiation exposure * Strong **genetic link** with **retinoblastoma**
77
* 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”
Ewing’s Sarcoma
78
s/s of Ewing's sarcoma? w/u?
* 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
mgmt for Ewing's sarcoma
* 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
Tumor of the retina - necrotic in center with areas of hemorrhage
Retinoblastoma
81
pathophys of Retinoblastoma
Arises from embryonic retinal cells - mutation in RB1 gene in long arm of chromosome 13
82
what makes retinoblastoma more likely to be hereditary?
bilaterally
83
s/s of retinoblastoma
* **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
w/u and mgmt for retinoblastoma?
* 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
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