Oncology Flashcards
1
Q
What genetic conditions have a predisposition for leukemia?
A
Trisomy 21 NF 1 Shwachman-Diamond syndrome Li-Fraumeni syndrome Chromosomal fragility – Bloom’s, Fanconi’s anemia, Ataxia Telangiectasia Paroxysmal Nocturnal Hemoglobinuria Diamond-Blackfan Anemia Kostmann syndrome SCID (Severe combined immune deficiency)
2
Q
What are typical findings on CBC in Leukemia?
A
WBC may be low, normal or high.
Hemoglobin and platelets may be normal or low.
3
Q
What % of patients with leukemia will not have circulating blasts?
A
20%
4
Q
How do patients with leukemia typically present?
A
Low Hemoglobin
Pallor, fatigue, irritability
Low White cells
Fever, sepsis
Low Platelets
Bruising/bleeding, petechiae
Leukemic Blasts
Bone pain, limp
5
Q
How do you distinguish between leukemia and sJIA?
A
Wbc low (<4),
Plt low normal (150-250),
Night time pain
6
Q
What x-ray findings do you see in leukemia?
A
Transverse radiolucent metaphyseal growth arrest lines
Periosteal elevation with reactive Subperiosteal cortical thickening
Osteolytic lesions
Diffuse osteopenia/ osteoporosis
7
Q
What are some clinical features of leukemia that indicate extramedullary disease?
A
Lymphadenopathy HSM Gingival hyperplasia Leukemia Cutis Chloroma (subcutaneous collection of AML cells) Lymphomatous mass CNS disease* Testicular disease*
8
Q
What are indications for BMA?
A
Unexplained and significant depression ≥ 1 peripheral blood cell elements
Blasts on peripheral smear
Leukoerythroblastic changes on peripheral smear
Association with unexplained lymphadenopathy or hepatosplenomegaly
Association with an anterior mediastinal mass
9
Q
What is the most common type of ALL?
A
80-85% are precursor B, remainder precursor T
10
Q
What is the prognosis of ALL?
A
90% cure
11
Q
How long is treatment for ALL?
A
2.5-3.5 years
12
Q
How long is treatment for AML?
A
6 months, intensive
13
Q
What is the prognosis of AML?
A
60% cure
14
Q
What are poor prognostic signs in ALL?
A
AGE: < 1 and > 10 yrs*
WHITE CELL COUNT: > 50 *
CNS and/or TESTICULAR DISEASE
CYTOGENETICS
Good:t (12;21) = TEL-AML; hyperdiploidy
Poor: MLL gene rearrangement (11q23)
Very Poor: t (9;22) = Ph chromosome, and hypodiploidy
DISEASE RESPONSE
As assessed by minimal residual disease (MRD) testing at the end of induction chemotherapy
15
Q
What is a differential diagnosis for cervical adenopathy?
A
Viral URI Bacterial adenitis EBV Cat scratch Atypical mycobacterium Malignancy Kawasaki TB Rare infections-toxo, tularemia, brucellosis, leptospirosis Sarcoidosis
16
Q
Differential diagnosis of generalized adenopathy?
A
Syphilis
HIV
CMV
Toxo
Rare: leukemia, TB, serum sickness, SLE, JRA, sarcoidosis, fungal, histoplasmosis, LCH
17
Q
What are the predictive factors for malignancy with lymphadenopathy?
A
Age >10
Node >2.5 cm
Supraclavicular (TB in ddx)
18
Q
What are the indications for excisional biopsy with lymphadenopathy?
A
>2 cm Increase over 2 weeks No decrease in size after 4 weeks Supraclavicular Hard, matted, rubbery consistency Abnormal CXR Fever Weight loss HSM
19
Q
What are the types of lymphoma in children?
A
Hodgkins
- Classical (chemo + rads)
- Lymphocyte predominant (only surgery if localized)
Non-hodgkins
- Mature B cell: burkitt’s and diffuse B cell
- Precursor B/T cell: Lymphoblastic lymphoma: like leukemia, except <25% BM involvement
- Mature T cell: Anaplastic
Rough ddx:
<5 years:
Lymphoblastic lymphoma
> 5 years:
Hodgkin lymphoma
20
Q
How does Hodgkin’s lymphoma typically present?
A
Painless cervical lymphadenopathy
Slow growing
1/3 present with B symptoms
21
Q
What tests do you need to do pre-chemo for Hodgkins?
A
PFTs (Bleomycin)
Echo (Anthracyclines)
22
Q
What is the prognosis of Hodgkin’s lymphoma?
A
> 90% cure
23
Q
What chemotherapy agents are used in Hodgkin’s treatment and what are their late toxicities?
A
Akylator/Etoposide and RT- any
BUT risk of breast cancer 12-20% by 30y!
Anthracycline and RT- CV ds, heart failure
Bleomycin and RT- pulmonary fibrosis
24
Q
What is more aggressive, NHL or HL?
A
NHL
Diffuse, aggressive, high grade
25
What is the average at diagnosis for NHL?
8-10yo
26
What are conditions that predispose to NHL?
Think immunodeficiency, especially in younger child with NHL:
```
Ataxia telangiectasia
Wiskott-Aldrich syndrome
Congenital hypogammaglobulinemia
Post-solid organ transplant
HIV
```
27
How does burkitt's lymphoma typically present?
- 80% have abdominal mass (mimics appendicitis, intussuseption)
- Can be endemic (EBV driven)
- High risk of TLS
28
Differential diagnosis for splenomegaly?
1) HEMATOLOGIC
HEMOLYTIC:
- Thalassemia
- Sickle cell anemia
- Hereditary spherocytosis
- Autoimmune hemolysis
LYMPHOPROLIFERATIVE:
- Leukemia
- Lymphoma
- ALPS
MYELOPROLIFERATIVE:
- CML
- Polycythemia vera
- Essential thrombocytosis, Myelofibrosis
HISTIOCYTOSES:
- HLH
- LCH
ONCOLOGIC
- Neuroblastoma
- Metastases
- Hemangioma
INFECTIOUS
EBV, CMV, Toxoplasmosis, Viral hepatitis, Typhoid, Tuberculosis, Syphilis, HIV
Malaria, Leishmaniasis, Schistosomiasis
RHEUMATOLOGIC / INFLAMMATORY
Systemic lupus erythematosus
Rheumatoid arthritis
Sarcoidosis
INFILTRATIVE
Gaucher’s disease, Amyloidosis
```
CONGESTIVE
Sequestration
Hypersplenism
Cirrhosis / Liver failure
Hepatic vein thrombosis
Cardiac failure
```
29
Differential diagnosis of anterior mediastinal mass?
Lymphoma
- Hodgkins
- NHL
Thymus
Teratoma
Thyroid
30
Name 4 complications of a mediastinal mass
Tracheal/Bronchial compression
SVC compression/obstruction (SVC Syndrome)
RVOT Obstruction
Pericardial effusion/tamponade
31
What are the two most common tumour to cause diplopia, headache and ataxia?
Posterior fossa-cerebellar astrocytoma, medulloblastoma
Brainstem gliomas are unlikely to cause raised ICP
32
Name 5 types of pediatric brain tumours
```
Low Grade/Pilocytic Astrocytoma
Medulloblastoma (MOST COMMON PEDIATRIC MALIGNANT BRAIN TUMOUR)
Ependymoma
Glioblastoma Multiforme
Brain Stem Glioma
```
33
How do supratentorial tumours typically present?
Localizing signs
SEIZURES
Visual changes
Hemiparesis , hemisensory loss, hyperreflexia
Subtle localizing signs/symptoms:
Behavioral problems: frontal lobe
Neuroendocrine deficits: hypothalamus/pituitary
DI, precocious/delayed puberty, hypothyroidism
Diencephalic syndrome in <3yo: FTT, hunger, euphoria
34
In what age group are supratentorial tumours more common?
Infants
| Teens/adults
35
What is the differential for supratentorial tumours?
1) Glioma (low or high grade)
- Optic pathway/hypothalamic (eg NF1)
- Cerebral
2) Craniopharyngioma
3) Ependymoma
36
How do infratentorial tumours typically present?
Raised ICP (cerebellar>brainstem)
Ataxia(clumsineness, nystagmus)
Long tract signs (worsening handwriting, appendicular dysmetria)
Cranial Neuropathy (diplopia, difficulty swallowing)
37
In what age group are infratentorial tumours more common?
Children
38
What is the differential for infratentorial tumours?
1) Glioma (low or high grade)
- Cerebellar
- Brainstem
2) Medulloblastoma
3) Ependymoma
39
How do you distinguish between a brainstem and cerebellar tumour?
CN neuropathy, long tract signs, ataxia=Brainstem tumor
↑ ICP, ataxia in absence of other signs= Cerebellar tumor
40
What are headache red flags?
Change in type, increasing severity/frequency
Sudden/severe onset
Associated with trauma
Early AM, awakens from sleep, assoc w/ straining
Abnormal neurologic signs/symptoms
Seizures or loss of consciousness
Linear growth regression
School or behavior change
41
What type of brain tumour does CT head often miss?
Posterior fossa tumours
42
What are the clinical features of osteoid osteoma?
Dull pain worse at night
| Better with NSAIDs
43
What are x-ray findings in osteoid osteoma?
Well-defined lucency surrounded by sclerotic bone
Proximal femur and tibia are most common sites
44
What are clinical features of osteochondroma?
Asymptomatic or non-painful bony mass
Lesion enlarges until reach skeletal maturity
45
What are the x-ray findings in osteochondroma?
Usually at metaphysis of long bones
Stalk/broad-based projection
46
What is an enchondroma?
Benign lesion of hyaline cartilage centrally in bone, often in hands
Asymptomatic usually
47
What is an aneurysmal bone cyst?
Reactive lesion of bone seen in persons <20 yr of age
Cavernous spaces filled with blood and solid aggregates of tissue
48
Where are aneurysmal bone cysts usually found?
Metaphysis of any bone
49
How do malignant bone tumours present?
Pain
Limp
Swelling
Nighttime awakening
50
At what age does osteosarcome typically present?
Around puberty (rapid bone growth)
51
What part of bones is osteosarcoma usually located?
Metaphysis of long bones (distal femur, proximal tibia/fibula)
52
What is the typical x-ray appearance of osteosarcoma?
```
Sunburst appearance***
Hair on end
Osteoblastic activity
Severe periosteal reaction with new bone formation
Periosteal elevation
Mottling***
```
53
Where does osteosarcoma metastasize?
Lungs
| Bone
54
In what bones is Ewing's sarcoma typically found?
Diaphysis of long bones or flat bones
Axial skeleton more common
55
What is the appearance of Ewing's sarcoma on XR?
Onion-skinning on XR
Primary lytic lesion (moth-eaten radiolucency) with multilaminar periosteal reaction
Cortical destruction
Soft tissue mass
56
Where does Ewing's metastasize?
Lungs
Bone
Bone marrow
57
How can you tell if leg pains are growing pains?
```
Children 4-12y
Poorly localized, often lower limbs
Occur at night but never persists until morning
No disability
Normal physical exam
Normal x-rays
```
58
What is the malignant differential for abdominal mass?
In younger kids (<5yo), think of embryonal tumors (“blastoma”)
Wilms (nephroblastoma)
Neuroblastoma
Hepatoblastoma (< 3yo)
In older, think of:
Lymphoma - Burkitt’s! (older than 3yo)
Germ Cell Tumour (bimodal)
Sarcoma (EWS, Rhabdo)
59
What investigations do you order for abdominal mass?
Ultrasound
CT
```
CBC and Diff, lytes, urate, LDH
Liver enzymes/function
AFP, BHCG (GCTs)
Urinanalysis
Urine HVA/VMA (NB)
Consider BMA
```
60
What is the clinical presentation of neuroblastoma?
Adrenal: Abdo mass, hypertension
Thoracic: Dyspnea or Horner syndrome
Paraspinal: spinal cord compression
Bone and Bone Marrow involvement:
Anemia, bruising, periorbital ecchymosis
Paraneoplastic syndrome:
Opsoclonus-Myoclonus-Ataxia
VIP-induced diarrhea
61
What are good prognostic factors in neuroblastoma?
Age, age, age, age - young age is better
Less than 12 – 18 months
Stage
CT/MRI, MIBG, bone scan, BM, Urine HVA/VMA
```
Biology
Histology
N-Myc amplification
Ploidy
Others: 1p deletion, 17q gain
```
62
What is Stage 4S neuroblastoma?
Skin involvement ONLY
Nontender, bluish subcutaneous nodules
Massive hepatomegaly
BM involvement <10%
63
What does the presence of opscolonus myoclonus imply for prognosis in neuroblastoma?
Good tumor, bad cognition
64
How do you treat opsoclonus myoclonus in neuroblastoma?
Steroids, IVIG
65
What age does hepatoblastoma typically present?
<3 years
66
What are risk factors for hepatoblastoma?
BWS
Hemihypertrophy
Family Hx of FAP
Prematurity
67
What is the biochemical marker in hepatoblastoma?
90% have ↑↑↑ alphafetoprotein (AFP)
68
What is the prognosis of hepatoblastoma?
Good prognosis for localized
| Unresectable may require liver transplant
69
How does Wilm's tumour present?
Painless abdo mass
Hematuria
Abdo pain with rupture
Also detected with screening at-risk patients
70
What is the prognosis of Wilm's tumour?
Excellent prognosis for localized and regional spread
71
What conditions increase your risk of TLS?
Leukemia with high WBC count (>50)
Lymphoma (esp lymphoblastic lymphoma, Burkitt)
Advanced solid tumours
Pre-existing renal issues
72
What are the electrolyte abnormalities associated with TLS?
High K, PO4, urate
| Low Ca
73
How do you treat TLS?
HYDRATION, diuresis
Anti-urate therapy (allopurinol, rasburicase)
Monitoring and management of electrolyte abnormalities
74
What is the WBC cut off for hyperleukocytosis?
WBC >100,000/mm3
75
What is the clinical presentation of hyperleukocytosis?
Pulmonary: exertional dyspnea, severe respiratory distress, hypoxemia
Neurological: confusion, somnolence, stupor, coma, headache, dizziness, gait instability, blurred vision, papilloedema, cranial nerve defects
Renal: decreased urine output, elevated creatinine
76
What is the most common cause of death in hyperleukocytosis?
Most early deaths are due to respiratory failure and intracranial hemorrhage
77
How do you treat hyperleukocytosis?
Hydration
Rasburicase
Platelet transfusion-to prevent ICH
Antibiotics
Avoid prBC tx (if need to small amounts 2-5 ml'/kg) because increases viscosity!
78
What types of cancers can cause spinal cord compression?
Sarcomas
Neuroblastoma
Lymphoma
CNS tumor mets
79
What are the signs of spinal cord compression?
```
Back pain
Local tenderness
Neurologic sx
Urinary retention
Constipation
Hyper-reflexia
Babinski
Parasthesias
```
80
What treatment should you add for prolonged febrile neutropenia?
Anti-fungal
81
What are factors that can influence fertility in cancer patients?
Type and cumulative doses of chemotherapy
| Radiation fields
82
What options do oncology patients have to deal with infertility?
Sperm banking
| Counseling about donor sperm, adoption…
83
What side effects are common to all chemotherapy?
```
Fatigue
Nausea/vomiting
Bone marrow suppression
Hair loss
Mucositis
Skin rash
```
84
Acute toxicity associated with vincristine
SIADH
Peripheral Neuropathy
-constipation, hyporeflexia, CNs
85
Late toxicity associated with vincristine
None
86
Acute toxicity associated with bleomycin
Pulmonary
Hypersensitivity
Rash
87
Late toxicity associated with bleomycin
Pulmonary fibrosis
88
Acute toxicity associated with cyclophosphamide/ifosfamide
Hemorrhagic cystitis
| Encephalopathy (ifosfamide)
89
Late toxicity associated with cyclophosphamide/ifosfamide
```
Infertility
Cardiac dysfunction
SMN
Pulmonary fibrosis
POF
```
90
Acute toxicity associated with L-asparginase
Allergic reaction
Hepatopathy
Coagulopathy/thrombosis
Pancreatitis
91
Late toxicity associated with L-asparginase
None
92
Acute toxicity associated with doxorubicin
Dilated cardiomyopathy
| Mucositis
93
Acute toxicity associated with anthracyclines (doxorubicin)
Dilated cardiomyopathy
| Mucositis
94
Late toxicity associated with anthracyclines (doxorubicin)
Cardiomyopathy
| Secondary leukemia
95
Late toxicity associated with etoposide
Secondary leukemia
96
Acute toxicity associated with cisplatin
Emesis
97
Late toxicity associated with cisplatin
Ototoxicity
| Nephrotoxicity
98
Acute toxicity associated with methotrexate
Renal failure
| Mucositis
99
Late toxicity associated with methotrexate
Leukoencephalopathy
100
Late toxicity associated with steroids
AVN, cataracts
101
List 6 long-term side effects of Hodgkin's Lymphoma treatment - radiation to neck and chest, chemo including doxorubicin, cyclophosphamide, bleomycin, etoposide, and steroids
```
Hypothyroidism (RT)
Infertility (Cyclopphosphamide, RT)
Cardiomyopathy, CAD (doxorubicin
Pulmonary Fibrosis (Bleomycin, RT)
Second CA – breast, thyroid, skin, …(RT)
MDS/Leukemia (Cyclophosphamide)
Avascular Necrosis (Steroid)
```
102
What is the pathology in LCH?
Clonal proliferation of epidermal dendritic cells
103
Clinical presentation of LCH
Exam presentation: Exopthalmos, refractory diaper rash, HSM, bony lucencies of skull of XR
```
Single bone lesion
Multiple bone lesions
Multi-organ involvement:
Bone (skull, limb, single or multiple, BM)- 80%
Skin/scalp (scaly, waxy rash) – 50%
CNS, Pituitary (DI)
Lungs
GI, liver, spleen
Eyes, ears, mouth
```
104
What are poor progonistic signs in LCH?
```
Young age (<18 months) at diagnosis
Hepatomegaly
Anemia and/or thrombocytopenia
Bone marrow involvement
Hemorrhagic skin lesions
```
105
How do you treat LCH?
Observe if localized
| Chemotherapy if systemic
106
What are the diagnostic criteria for HLH?
Need 5 out of 8:
```
fever
bicytopenia
splenomegaly
hemophagocytosis
hyperferritinemia
increased sIL2R
hypertriglyceridemia
hypofibrinogemia
low/absent NK cell activity
```
107
How do you treat HLH?
IVIG
| Chemo : Steroids, Etoposide (VP-16)
108
What are indications for allogeneic BMT?
```
Hematologic malignancy
Bone marrow failure
Select immunodeficiencies
Regfactory/relapsed lymphoma
Metabolic disorders
Hemoglobinopathies
Osteopetrosis
```
109
What are indications for autologous BMT?
Autologous transplant is basically a ‘rescue’ from ultra high dose chemotherapy
(For example, after chemotherapy for NB, you do autologous BMT to reconstitute the BM)
110
What is GVHD?
Only in allogeneic transplants
GVHD occurs when immune cells transplanted from a non-identical donor (the graft) recognize the transplant recipient (the host) as foreign, thereby initiating an immune reaction that causes disease in the transplant recipient
111
What are risk factors for GVHD?
Mismatched and unrelated donor
Bone marrow more than cord
Increasing age
112
What systems are involved in acute GVHD?
Skin
Liver
Gut
113
What systems are involved in chronic GVHD?
Any system can be involved
114
After how many days would you consider GVHD chronic?
>100 days
115
What is osteopetrosis?
Inherited bone disease involving dense, sclerotic bones prone to fracture
116
What are clinical features of osteopetrosis?
```
Growth impairment
Fractures
Dental abnormalities
Hepatosplenomegaly
Epilepsy
Intellectual disability
Bone marrow can be affected due to bone density causing cytopenias
```
117
What is Kasabach Meritt syndrome?
Vascular tumor (hemangiomas) leads to sequestration/destruction of platlets, thrombocytopenia and consumptive coagulopathy (DIC)
118
What is the most common type of tumour associated with Kasaback Meritt syndrome?
Kaposiform hemangioendotheliomas
119
How do you treat Kasabach Meritt?
Steroids
| Chemotherapy
120
What is the impact of a cancer diagnosis on siblings?
Siblings are at risk both during illness and after death
Parents have extraordinary demands placed on them to meet needs of ill child – healthy children may feel own needs aren’t being met or acknowledged and this may lead to concerns about own health and resentment
Normal response/encouragee to maintain normal routines
Siblings tend to adjust better at the time of and after death
Acknowledge and validate the sibling’s feelings
121
Name 4 principles of treatment with opoids for palliative oncology patients
Characterize the pain: neuropathic or mixed?
Decide on route of administration: can she swallow?
Dose with long acting opiods with short acting breakthrough
Ensure timely reassessment of symptoms
Decide if adjuvant medications are required: gabapentin for neuropathic pain
122
Associated malignancies with Down's Syndrome
TMD
ALL
AMKL
123
Associated malignancies with Beckwith-Wiedemann
Wilm's tumour
| Hepatoblastoma
124
Associated malignancies with WAGR (Wilms, Aniridia, GU abnormalities, Retardation)
Wilm's tumour
125
Associated malignancies with NF-1
Optic pathway glioma
Astrocytoma, brainstem gliomas
Soft tissue sarcoma
Leukemia
126
Associated malignancies with Retinoblastoma (RB1)
Retinoblastoma
Osteosarcoma
Melanoma
127
Associated malignancies with Li Fraumeni (p53)
Bone, soft tissue sarcomas
Brain tumours
Breast
Others
128
Associated malignancies with Multiple Endocrine Neoplasia
MEN1=Parathyroid glands, anterior pituitary, and entero-pancreatic endocrine cells
MEN2=Medullary thyroid CA, pheochrompcytoma
129
Associated malignancies with Familial Adenomatous Polyposis
Colorectal carcinoma
Hepatoblastoma
Thyroid cancer
130
What percentage of patients with Down Syndrome have TMP?
10%
131
What are the clinical and laboratory features of TMP?
↑ WBC
Peripheral blasts
Pancytopenia
Hepatosplenomegaly
132
How do you treat TMP?
Most = no treatment, resolves over days-weeks
133
What cancer does TMP put you at risk for?
AML
134
What are the clinical features of Beckwith Wiedemann?
```
Hemihypertrophy
Macrosomia
Macroglossia
Visceromegaly
Omphalocele
Umbilical hernia
Neonatal hypoglycemia
```
135
What is the genetic mutation in Beckwith Wiedemann?
11p15 imprinting/uniparental disomy
136
How often and with what do you screen patients with Beckwith Wiedemann for malignancy?
Screen every 3 months with:
AFP until age 4
Abdo U/S until age 8
137
What conditions are associated with Wilm's tumour?
Beckwith-Wiedemann
WAGR (Wilms, Aniridia, GU abnorm, Retardation)
Denys Drash (renal dz, pseudohermaphroditism, Wilms)
11p deletions
Hemihypertrophy
138
When do you screen for subependymal astrocytomas in TS?
1-3 years of age
139
What are the important emergency department management steps for all children with suspected leukemia?
```
TLS bloodwork
CXR
Hyperhydration
Allopurinol (or raburicase)
Antibiotics if febrile regardless of WBC count
```
140
What are benign causes of abdominal mass?
Hydronephrosis
Liver/spleen
Fecal mass
Renal vein thrombosis
141
What is the most common risk factor for renal vein thrombosis in neonates?
Mother with GDM
142
What is the most common cause of abdominal mass in a neonate?
MCDK
143
What are 5 physical exam findings associated with neuroblastoma
```
Hypertension (catecholamine release)
Abdominal mass
Hyperreflexia (SCC compression)
Opsoclonus-myoclonus (paraneoplastic)
Bruising/petechiae (BM involvement)
Periorbital ecchymosis (mets)
```
144
Until how many months after chemo do you give VZIg if an oncology patient is exposed to chicken pox?
3-6 months
145
What are two factors associated with cancer treatment than can effect fertility?
Type and cumulative dose of chemotherapy
| Radiation fields
146
What are 2 options for dealing with infertility to offer cancer patients?
Sperm banking
| Counseling about donor sperm and adoption
147
What chemotherapy would cause sinus vinus thrombosis (oncology patient with headache)?
L-asparginase
148
How do you diagnose LCH?
Skin biopsy
149
Describe xray findings in aneurysmal bone cyst
Eccentric lytic destruction and expansion of the metaphysis surrounded by a thin sclerotic rim of bone
