Oncology Flashcards
1
Q
4th leading cause of death in children
A
CA
2
Q
MC malignancy in children (nearly 1/3 of all pediatric cancers)
A
Acute Lymphoblastic Leukemia
(ALL)
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)
4
Q
A blood stem cell goes through several steps to become a ? (3)
A
RBC
WBC
platelet
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
6
Q
ALL develops from what type of stem cell?
A
lymphoid blast
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
8
Q
w/u for ALL?
A
-
CBC w/ diff
- 95% have dec in at least one cell type - neutropenia, thrombocytopenia or anemia
- ANC < 1k, even if WBC normal - peripheral smear
- BMP, Uric Acid, LDH, PT/PTT
- DX: BM aspirate
- LP
- CXR
9
Q
- Lymphoblasts on peripheral smear
- Pancytopenia (all 3 cell lines are decreased)
likely dx?
A
ALL
10
Q
ALL - Abnormal white cells reveal hyperdiploidy and translocation of chromosomes ___ and ____
A
12 and 21
11
Q
how to perform BM aspirate and bx?
A
- numbed
- insert Jamshidi needle (a long, hollow needle) hip bone
- Samples of blood, bone, and bone marrow are removed for examination under a microscope
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%
13
Q
goal remission % during phase 1 for ALL?
A
95% remission on bone marrow aspirates
14
Q
RF for ALL
A
- XR
- Downs syndrome
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
16
Q
s/s of AML
A
- Early flu-like sx
- Fatigue
- Bleeding
- Infection
- Adenopathy
- Skin nodules
- HSM
17
Q
w/u for AML
A
- CBC w/ diff
- BM aspirate
- LP, CXR
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
19
Q
AML comes from what stem cell?
A
myeloblast - either myeloid stem cell or myeloid blast
20
Q
general mgmt approach of AML
A
- Less responsive, more intense
- Chemo - systemic and intrathecal - aggressive induction therapy (80-85% remission)
- Radiation - sometimes
- BM transplant
- Cord blood transplant
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
22
Q
RF for AML
A
- Ionizing radiation therapy
- Previous chemo
- Syndromes - Neurofibromatosis, Downs
23
Q
disposition and prognosis of AML
A
- Can reoccur
- Remission when WBC in normal range
- 75-85% remission
- 35-50% long-term survival
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
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%
