Ped Oncology Flashcards
1
Q
number one cause of disease related death in children?
A
cancer bro
2
Q
*Most common cancers of childhood?
A
1) leukemia
2) CNS
3) soft tissue
4) non hodgkin and kidney
5) bone
6) hodgkin
3
Q
is incidence of childhood cancer increasing?
A
Yes a little bit for the common tumors but not so much for the less common!
4
Q
5 yr survival for common bone marrow related cancers
A
lowest to highest:
- AML
- NB
- ALL
- Wilms
5
Q
Epidemiology of childhood cancer
A
-1/330 Americans develops cancer before 20
~46 U.S. children & adolescents diagnosed each day
6
Q
Long-term effects of childhood cancer
A
- 1:750 20-year-olds alive U.S. is a survivor of childhood cancer
- 3/5 children diagnosed w/ cancer suffer long-term or late onset side effects
7
Q
2 childhood cancers that may be increasing in incidence
A
- ALL
2. Brain & CNS
8
Q
Most improved outcomes has been for which childhood cancer?
A
ALL
9
Q
Where are children usually treated for cancer?
A
> 90% seen & treated at a COG affiliated institute
-Consistent tx across U.S; Don’t need to travel somewhere else to get the best treatment
10
Q
General points about causes of childhood cancers
A
Largely unknown:
- Specific chromosomal/genetic abnormalities, & ionizing radiation exposures explain only a small % of cases
- Environmental causes of childhood cancer have long been suspected by many scientists but have been difficult to pin down
11
Q
Familial/genetic diseases associated with increased cancer risk:
A
- Neurofibromatosis
- Familial polyposis
- Li-Fraumeni syndrome
12
Q
Major categories of diseases linked with an increased cancer risk include:
A
- Immune deficiencies
- Metabolic disorders
- Disorders of chromosome stability
13
Q
*General points about presentation of childhood cancers
A
- S/S of cancer are relatively non-specific and mimic a variety of more common childhood problems
- Oncologists are highly suspicious for cancer; Primary care physicians are opposite
- You have to think about the possibility of cancer before you can make the diagnosis
14
Q
*Red flags for Leukemia
A
- Unexplained fever(s) >101F >7 days
- Petechiae
- Unexplained anemia / pallor
- Generalized lymphadenopathy
- Hepatosplenomegaly
* *6. Bone/joint pain (30%) not relieved w/ pain medications or that wakes from sleep (Expanding marrow space) - sudden weight loss
- hypertension
15
Q
*Red flags for Brain Tumors
A
Pediatric tumors are often situated that they interfere w/ CSF circulation-> in ↑ intracranial P. ->
-Headaches & vomiting (early am especially) are common presenting signs
16
Q
*Conditions Suggesting Radiographic Evaluation in Headaches
A
- Presence of neurologic abnormality
- Ocular findings, papilledema
- Persistent vomiting/ increasing or Preceded by recurrent HAs
- Changing character of the HA
- Recurrent a.m HAs or HAs that awaken or incapacitate the child
- Short stature/deceleration of linear growth
- Dx of Neurofibromatosis
- Previous leukemia or CNS radiation
17
Q
Lymph nodes:
- Large=?
- Common cause of enlargement?
A
-
Lymph Nodes are considered large if > 10 mm*; exceptions:
1. Epitrochlear nodes > 5 mm
2. Inguinal node > 15 mm - Most enlarged lymph nodes in children are related to infections:*
- Bacterial: Staph & Strep
- Atypical mycobacterium
- Cat scratch disease
- Viral: EBV & other herpes viruses
18
Q
*Lymph node biopsy is Suggested by the following S/S
A
- Enlarging nodes after 2-3 weeks of antibiotics
- Nodes that aren’t enlarging, but haven’t diminished in 6-8 weeks
- Nodes associated w/ any abnormal CXR
- Adenopathy w/ associated weight loss, hepatosplenomegaly, unexplained fevers, and/or drenching night sweats
- Adenopathy in the posterior auricular, epitrochlear or supraclavicular areas
19
Q
Evaluating masses
A
Abdominal, Thoracic & Soft Tissue Masses (without a traumatic explanation) all require evaluation
20
Q
Bone/Joint pain & cancer
A
-Most cancer pain is caused by bone, nerve or visceral involvement or encroachment
-Bone pain is usually not an early symptom of cancer except for bone malignancies
(Ewing’s sarcoma, osteosarcoma)
-Come & go early on disappearing for weeks-months
-Bone or joint pain is a presenting symptom in about 30% of ALL pts with
**-Can be confused w/ rheumatic diseases*
21
Q
*Bone/Joint pain evaluation should be performed when:
A
- Bone/joint pain is persistent
- Associated with swelling/mass
- Limited mobility or joint motion
- Consistently wakes from sleep
- Not relieved by NSAIDs
22
Q
***Most common malignancy in childhood
A
ALL
23
Q
Populations most at risk for ALL -
A
- 2-5yr
- Males
- Caucasians
- -Hispanics
24
Q
**Childhood leukemia S/S
A
Bone marrow infiltration:
- **1. Anemia
- Pallor, lethargy
- Dyspnea, murmur
- **2. ↓ Platelets
- Bleeding, petechiae, purpura
- **3. Neutropenia
- Fevers and infections
- **4. Bone pain
- Limp, ↓walking, irritability
- **5. Extrameduallary Disease
- **6. Fever of Malignancy
25
Symptoms of extramedullary disease
- Lymphadenopathy, Hepatosplenomegaly
- Orthopnea, Cough
- Mediastinal mass, tracheal compression
- Facial nerve palsy
- Testicular enlargement
- Skin lesions, Gingival hypertrophy
26
*CBC findings in ALL
* *-50% will have ↑WBC
* *-80% will have lymphoblasts on the peripheral smear
* *-95% will have >2 Cytopenias
- Only 4% will have 1 cytopenia
- Only 1% will have a normal CBC & differential
27
Common diseases mimicking leukemia S/S
1. Mononucleosis (EBV)
2. Acute anemia
3. Parvovirus B19
4. Idiopathic thrombocytopenia (ITP)
28
Risk factors for ALL
* **1. Down syndrome
2. Prenatal exposure to X-rays
3. Postnatal exposure to high doses of radiation
29
* Precursor B-cell ALL
- Frequency?
- Presentation?
-80-85% of ALL's
-Presentation- ***Primarily BM involvement***
-Extramedullary involvement frequent
(CNS, skin, gonads, lymph nodes, liver, spleen)
30
* Precursor T-cell ALL
- Who is affected?
- Presentation?
~15% childhood ALL
- Adolescents > young children
- Males>Females
1. Mediastinal mass (50%-70%), ↑ WBC
2. Lymph nodes, CNS, skin, liver, spleen, gonads
31
NCI ROME leukemia risk criteria
Standard risk:
- Age: 1 - 10 years
- WBC: 10y
- WBC: >50,000
32
*ALL tx
* Chemotherapy: IV, oral, IT Intrathecally - into CSF), and IM
- Induction, Consolidation, Interim. Main (2), -Delayed intensification & Maintenance
- Average Risk: Girls & Boys-2½ years
- Higher Risk: Boys-3½ years
- Goals of our current clinical trials
- CNS prophylaxis in T cell or those CNS+
- F/U for 5yr after chemo, then annually for 10yr
33
*ALL prognosis -
- >95% remission
| - 75-85% survive w/o recurrence at least 5 years from Dx
34
*Late effects of ALL tx
1. Osteonecrosis, Osteopenia
2. Neuropathies
3. Neuropsychometric concerns
4. Obesity
35
AML Tx, outcomes & late e ffects of Tx:
Cytarabine, Dauno, VP-16 + BMT
Outcome: EFS remains at approximately 50%
Late Effects: Cardiac toxicity, 2nd malignancies, endocrinopathies, etc
36
*Most common solid tumor of childhood
Brain tumors
| =20% of childhood malignancies
37
Most common brain tumors -
Astrocytoma
| -Grade 1 - "JPA" - seen in association with NF, malignant and by location
38
*Symptoms of ↑Intracranial P. (brain tumors)
1. HA, Nausea/vomiting (am)
2. Double vision
3. Head tilt
4. ↓ Alertness, Lethargy/irritability
5. Poor feeding, FTT
6. Endocrine dysfunction
7. Unexplained behavior changes:
affect, motivation, energy level
39
*Signs of ↑Intracranial P. (brain tumors)
1. Papilledema, optic atrophy
2. Loss of vision
3. OFC (head circumference) increased
4. Bulging fontanelles, spreading sutures
5. "Setting sun" sign (Parinaud syndrome)
6. ↑ BP, ↓ pulse-> herniation?
40
Posterior Fossa primary tumor presentation
1. Ataxia & Tremors
2. Dysarthria & Stiff neck
3. Papilledema
41
Brainstem primary tumor presentation
1. Extremity weakness
2. Cranial nerve signs
- Double vision
- Facial weakness
- Swallowing dysfunction
42
Hemispheric Tumors presentation
1. Hemiparesis
2. Hemianopsia
3. Aphasia
4. Seizures
43
*Work up for brain tumors
1. CT: 65-95% sensitivity to detect brain tumors
* **2. MRI: with and without contrast
3. CSF examination
4. Biopsy or preferred excision biopsy/resection
44
*Brain tumor tx
* **1. Primarily Surgery (Gross total resection)
- Exception: Optic glioma
* **2. Radiation: Potential in all tumors
- Exception: choroid plexus tumors
* **3. Chemo: Adjunct in most
- Particularly in GCT (germ cell tumor), medulloblastoma
45
*Risk factors for brain tumors
* **1. Astrocytomas= associated w/ NF-1 in 50-80%
2. Li-Fraumeni syndrome
- Germline mutation p53 tumor suppressor
3. Radiation to head & neck
- Survivors of childhood leukemias!
46
****Leading cause of morbidity & mortality in pediatric cancers
Brain tumors
47
***2nd most common abdominal malignancy in children.
Pathogenesis?
Wilms' Tumor
WT1 mutation: Tumor suppressor gene critical for normal renal development; Mutations predispose to embryonal tumor formation
48
*Presentation of Wilms' tumor
1. *Large palpable abdominal mass**
| 2. HTN, gross hematuria & fever in 5-30%
49
*Evaluation of Wilms' tumor
1. CBC, CMP -
2. CT Abdomen (Assess renal function prior to contrast - CREATININE!)
3. Chest XR & CT
4. Abdominal Ultrasound
50
* Tx of Wilms' tumor
| - Prognosis?
Stage 1=nephrectomy
Stage 2+= Nephrectomy + Chemotherapy + Radiation
80-90% survival w/ multi-modal therapy
51
* Retinoblastoma:
- Epidemiology
- Pathogenesis
- Tx & prognosis
- 80% occur in children <3
- 30% are bilateral
- Loss of both alleles of the RB gene
- 60% spontaneous, 40% hereditary=FAMILY HISTORY SUPER IMPORTANT!
- Tx: radiation, chemotherapy, enucleation
- 85% long term survival
52
* **Neuroblastoma:
- Epidemiology
- Findings
- Tx
***-Most common extra-cranial solid tumor in children
-75% are <4 yr (Average=18Months)***
more aggresive if 18+ months
-Findings: abdominal mass, pallor, weakness, bone pain, fever & weight loss
-FAMILY HISTORY SUPER IMPORTANT!
-TX: chemotherapy, surgery, radiation, stem cell transplant
53
*Poor prognostic indicators for neuroblastoma
1. Age >18 months
2. Myc-N amplification
3. Unfavorable Histology
54
*Neuroblastoma work up
1. Urine Catecholamines: HVA, VMA
2. Imaging Studies: CT, bone scan, MIBG scan
3. Bone Marrow Biopsies
4. Lymph Node evaluation
55
Botryoid (grape-like) vaginal mass
Rhabdomyosarcoma
S/S depend on age & site
56
* Ewing's Sarcoma & Osteosarcoma:
- Epidemiology
- Presentation
- Prognosis
* **-Peak in adolescents ***
* **-More common in males ***
* **-Pain, swelling, often following sports injury ***
- Staging directly related to prognosis (very variable)
-treatment: limb salvage important!
57
Hodgkin's Disease:
- Age
- Clinical features -Age:
```
adolescents >> young child
-Painless lymphadenopathy
-Progresses over weeks- months
-95% Located in:
1. Cervical/supraclavicular ↑ LNS
(Unilateral or bilateral)
2. Mediastinum ± hilum
3. LN below diaphragm and spleen
```
58
*Hodgkins disease symptoms
Systemic "B" symptoms (first 3):
1. Fevers
2. Night sweats
3. Weight loss
4. Pruritus
59
most difficult area to get imaging done on a kid?
posterior fossa
60
astrocytomas are strongly associated with what?
NF-1
61
Wilms tumor more/less likely in which races?
| Ages?
more likely in AA
less likely in Asians
rare in children>5
62
neuroblastoma location?
any neural crest tissue
- adrenal
- paraspinal sympathetic tissue: cervical, thoracic, pelvic
63
signs and symptoms of neuroblastoma:
clincal presentation?
- irritability
- weight loss
- bone pain
- fever
- RACCOON EYES -periorbital ecchymoses
- Proptosis
- bone leisions
- large abdominal mass - often crosses midline
- lower extremity weakness-spinal cord compression
- cervical high thoracic mass--> HORNERS SYNDROME (MIOSIS, PTOSIS,ANHYDROSIS)
64
Racoon eyes associated with what disease?
Neuroblastoma
65
most common extra cranial solid tumor in children?
neuroblastoma
66
myc-N associated neuroblastma.. prognosis?
...eh not good bud
67
neuroblastoma staging:
- 1- localized tumor, complete excision
- 2A- unilateral, incomplete gross resection; negative microscopic nodes
- 2B- unilateral, positive ipsilateral nodes; negative contralateral
- 3- across midline, or contralateral nodes
- 4- dissemination: BM, liver, skin bones
- 4S- <1yrs: local stage 1-2 with mets to BM liver and skin
68
most common soft tissue sarcoma in children:
rhabdomyosarcoma
69
rhabdomyosarcoma
- arrises from?
- origination?
- incidence peaks when?
- survival related to?
- arises from undifferentiated mesenchymal cells that differentiate into muscle
- originate anywhere
- peaks bw age 2-6
- survival directly related to staging higher stage=higher risk of death
