Oncology Flashcards
(101 cards)
1
Q
What is B cell ALL standard risk?
A
Age 1-10 and WCC <50 at presentation
All others are high risk
2
Q
Is hyperdiploid (more chromosome in malignant cells) good or bad?
A
It is good - having more than 50 chromosomes is protective
However T21 is bad!
3
Q
What translocations in ALL are bad?
A
t9: 22 (robertsonian)
4: 11 translocation KMT2a (MLL)
4
Q
What translocation in ALL are good?
A
t(12:21) ; TEL/AML1 or ETV6 -RUNX1
5
Q
What is the cure rate for all leukaemia?
A
90%
Standard risk - 92%
6
Q
Fusion of BCR (from chromosome 22) and ABL1 (from chromosome 9) creates abnormality in which protein?
A
Tyrosine Kinase
7
Q
What tyrosine kinase inhibitors are used alongside?
A
Imatanib and Dasatinib
8
Q
What does Auer rod mean?
A
AML !
9
Q
What does a Reed Sternberg cell mean and describe it
A
Owl eye appearance - big cell with two part and two distinct nuclei
Hodgkins lymphoma
10
Q
How does Blinatumomab work?
A
Cd3 and Cd19 combo - CD 19 binds to B cell and atracts T cell to kill the bad B cell
11
Q
What is CAR-T cell therapy?
A
These are engineered or autologous chimeric antigen receptor T-cells to target specific cells
12
Q
What is Brentuximab and what is it used to treat?
A
CD30 antibody -> leads to cell apoptosis
Used to treat Hodgkin and ALCL
13
Q
What is Gemtuzumab?
A
It targets CD33 and used against AML
14
Q
Haematopoetic stem cell is characterised by CD __
A
CD34 expression
15
Q
What is the ideal source for HSCT?
A
1) Matched
2) Marrow
3) Sibling
If mismatched - preference for mismatched family donor -marrow or
Mismatched cord blood
be mindful that in large kids (>50kg) may not get enough from cord
16
Q
_______ donor is better for metabolic disorders
A
Unrelated cord blood
17
Q
Which chemo agents can predispose for VOD?
A
Busulphan
Gemtuzumab
18
Q
Von Hippel–Lindau (autosomal dominant) is associated with which sort of brain tumours?
A
Haemangioblastoma
19
Q
What are Rosenthal fibers and what are they associated with?
A
They are condensed masses of glial filaments occurring in compact areas with low mitotic potentials helps establish the diagnosis of pilocytic astrocytomas
20
Q
Chemotherapy agents given singly or in combination for _____ include carboplatin, vincristine, lomustine, procarbazine, temozolomide, and vinblastine
A
Low grade astrocytomas
21
Q
Astrocytomas associated with tuberous sclerosis have responded to _________
A
Everolimus - mammalian target of rapamycin inhibitor
22
Q
Oligodendrogliomas consist of __________ (histology). Observation of a __________ (radiology) on CT in a patient presenting with a seizure is suggestive of oligodendroglioma
A
Histology - rounded cells with little cytoplasm and microcalcifications
Radiological findings - calcified cortical mass
23
Q
What is the most common brain tumour in children <1yr
A
Choriod plexus tumours
24
Q
Choriod plexus tumours are commonly associated with ______
A
Li Fraumeni syndrome
25
Neuronal differentiation is more common among Medulloblastoma and is characterized histologically by the presence of _________________
Homer Wright rosettes and immunopositivity for synaptophysin.
26
The most common abnormality in medulloblastoma involves _____________, which occur in 30–40% of all cases
Chromosome 17p deletions
27
________ are collections of myeloid leukemia cells and can occur throughout the neuraxis
Chloromas
28
Most common brain tumour to metastasize?
Medulloblastoma
29
bHCG is 100% positive in
Choriocarcinomas
30
What is the mechanism of action of Allopurinol and what complications can occur?
Allopurinol is a Xanthine Oxidase inhibitor - it inadvertently alkalises the urine and can precipitate Calcium phosphate stones
31
How does Rasburicase work?
It is a urate oxidaser and converts uric acid into allatoin which is more water soluble
32
What are the three cardinal electrolyte abnormalities with TLS?
HypoCa, HyperK+, HyperPO4
33
Name 8 common live vaccines?
```
BCG (bacille Calmette–Guérin) vaccine
Japanese encephalitis vaccine
MMR (measles-mumps-rubella) vaccine
rotavirus vaccine
oral typhoid vaccine
varicella vaccine
yellow fever vaccine
zoster vaccine
```
34
Diagnostic criteria for MDS include cytopoenia in 1 or more lineage (most commonly erythrocytes), dysplastic cells on film and ______ blasts in blood and bone marrow.
<20% blasts -> if more, it is AML
35
Osteosarcoma occurs in teenage boys but is associated with a ________ on x-ray.
Sunburst lesion
36
Onion peel or moth eaten appearance is associated with ________
Ewings Sarcoma
37
Name three Ribonucloetide reductase inhibitors and their mechanism of action?
Hydroxyurea - blocks RRI by binding to iron molecule
| Fludarabine/Gemcitabine - blocks RRI and DNA polymerase
38
Name two topoisomerase inhibitors and their mechanism of action?
Irinotecan/topotecan - inhibit topoisomerase I
| Etoposide/Teniposide - inhibit topoisomerase II
39
What are three antitumour antiobiotics and how do they work?
Bleomycin - binds to DNA, produces free O2 and oxidase DNA bases
Dactinomycin - intercalates DNA bases, prevents replication
Anthracyclines - intercalates DNA, inhibit Topoisemerase II and produce free O2 radicals
40
_________ can prevent cardiotoxicity from Anthracyclines
Dexrazoxane
41
Antimetabolites mimic nucleobases or folic acid to prevent DNA syntheses
- ________ are mimiced by Azathioprine and Cladribine
- _________ are mimiced by Cytarabine and 5-Fluorouracil
- Purines are mimiced by Azathioprine and Cladribine (A and G)
- Pyrimidines are mimiced by Cytarabine and 5-Fluorouracil (C and T)
42
What is the mechanism of action of cytarabine?
Binds to DNA polymerase
43
Methotrexate can cause bone marrow suppression and megaloblastic anaemia (can be reversed by _______)
Can be reversed by Leuovorin
44
Which chemotherapy agent is associated with delayed nausea by RACP?
Cisplatin
45
XLP type I is associated with the absence of _______ can lead to an ___________
absence of SH2D1A can lead to an uncontrolled cytotoxic T-cell immune response to EBV
46
Three common manifestations of XLP?
1) Fulminant mononucleosis (50%)
2) Lymphoma (25%)
3) Hypogammaglobulinaemia (25%)
47
Methotrexate competitively inhibits _____________, an enzyme that participates in the tetrahydrofolate synthesis
Dihydrofolate reductase (DHFR)
48
Folic acid is needed for the de novo synthesis of the nucleoside ____________, required for DNA synthesis. Also, folate is needed for purine base synthesis, so all purine synthesis will be inhibited.
Thymidine
49
What lab anomaly are seen with rhabdomyosarcoma?
Hypercalcemia; blood cytopenia caused by bone marrow involvement
50
What is WAGR and what is the associated mutation?
Wilms, Aniridia, GU anomalies and mental retardation
Assoc with Del of 11p13 and WT1 and PAX
51
What are the clinical manifestations of Denys-Drash and what is the associated genetics?
Renal failure + Mesangial sclerosis + male pseudohermphrodism
genetics - WT1 missense mutation
52
What is the genetics for Beckwith Weideman syndrome?
11p15.5 duplication or uniparental diosomy
The responsible gene is located on chromosome 11, and may be over-expressed due to a methylation abnormality, paternal uniparental disomy, or gene mutation.
53
What are the clinical manifestations of Frasier syndrome?
Undermasculinisation of genitalia, FSGS and gonadoblastoma (also Wilms)
WT1 intron 9 variant
54
Chromosome 13q deletion syndrome is associated with which oncological manifestation?
RB1 gene deletion - retinoblastoma and sarcoma
Also associated with intellectual disability, characteristic craniofacial abnormalities
55
Trisomy 21 is associated with increased risk of which cancer?
ALL, AML, AMKL, TMD
Risk of ALL is increased 20-fold, risk of AMKL is increased 500-fold; high cure rates; more prone to chemotherapy toxicity; AMKL associated with GATA1 mutations
56
Klinefelter syndrome (47, XXY) is associated with which oncological anomaly?
Breast cancer, extragonadal germ cell tumors
57
Xeroderma pigmentosum is associated with which oncological anomaly?
And mode of inheritance?
SCC, BCC and melanoma
Autosomal recessive; failure to repair UV-damaged DNA; XP gene mutations
58
Fanconi anemia is associated with which cancer?
AML, MDS, ALL
Autosomal recessive, mutations in FANCX gene family
59
Which cancers is Bloom syndrome associated with?
AML, MDS, ALL, Lymphoma
Associated with growth deficiency, malar rash; autosomal recessive; increase sister chromatid exchange (SCE); mutations in BLM gene; member of the RecQ helicase gene
60
Ataxia-telangiectasia is associated with which oncological anomaly?
Lymphoma, leukemia, less often central nervous system and other solid tumors
61
What is the mode of inheritance of ataxia-telengectasia and other clinical findings?
Autosomal recessive; biallelic mutation in ATM tumor-suppressor gene
Associated with progressive ataxia, oculocutaneous telangiectasias; sensitive to radiation-induced DNA damage; increased risk of treatment-related morbidity;
62
This syndrome is associated with increased risk of leukaemia and lymphoma - it is associated with microcephaly, characteristic facies, immunodeficiency; biallelic mutations in NBN gene
Nijmegen breakage syndrome
63
This syndrome is associated with soft tissue carcinoma and melanoma - you also see with accelerated aging; autosomal recessive and mutations in WRN gene
Werner syndrome (progeria)
64
X-linked recessive; WASP gene mutations and associated increased risk of leukaemia and lymphoma....
which syndrome?
Wiskott Aldrich syndrome
65
X-linked agammaglobulinemia (XLA) is associated with increased risk of which cancer?
Lymphoproliferative disorders, colorectal cancer
66
X-linked or autosomal recessive; mutations in IL2RG and ADA genes and increased risk of leukaemia/lymphoma - which syndrome?
SCID
67
NF2 is associated with increased risk of which syndrome?
Bilateral acoustic neuromas, meningiomas
68
Noonan syndrome:
Genetics involved?
Increased risk of which malignancy?
Malignancy - JMML, ALL, neuroblastoma, brain tumors
Genetics - Autosomal dominant; caused by RAS/MAPK pathway mutations (most frequently PTPN11)
69
Which syndrome?
Autosomal dominant; mutations in PTCH1 or SUFU gene and increased risk of BCC and meduloblastoma
Gorlin-Goltz syndrome
70
Apart from Wilms which other cancers is BWS associated with?
Wilms tumor, hepatoblastoma, neuroblastoma, rhabdomyosarcoma
71
Von Hippel Landau syndrome:
Genetics?
Clinical features ? (malignancy)
Genetics - Autosomal dominant; mutations of tumor-suppressor VHL gene
Clinical features - Hemangioblastomas of the brain and retina, pheochromocytoma, renal cell carcinoma
72
MEN1 is associated with hyperparathyroidism, ZES;
Caused by autosomal dominant; mutations in MEN1 tumor suppressor gene
What are the associated malignancies?
Parathyroid, pancreatic islet cell and pituitary tumors
73
What are the associated malignancy with MEN2?
Medullary thyroid carcinoma, parathyroid tumors, pheochromocytoma
74
Apart from colon cancer what are the two other malignancies associated with FAP?
Hepatoblastoma and thyroid cancer
75
Autosomal dominant; mutations in BMPR1A and SMAD4 gene ... which syndrome?
Juvenile polyposis
76
Which syndrome:
Autosomal dominant; mutations in DNA mismatch repair genes MSH2, MLH1, PMS1, PMS2, and MSH6
Increased risk of colorectal cancer, endometrial and stomach cancer, many other cancers
Hereditary nonpolyposis colorectal cancer (HNPCC, Lynch syndrome)
77
Which syndrome:
Autosomal dominant; mutations in APC or MLH1 gene
Increased risk of colorectal cancer, brain tumors (glioblastoma, medulloblastoma)
Turcot syndrome
78
This syndrome is associated with hamartomatous polyps of GI tract; freckling of mouth, lips, fingers, and toes; autosomal dominant; mutations in STK11 gene
What is the syndrome and what are the two associated malignancies?
Peutz-Jeghers syndrome
Associated with breast cancer, colorectal cancer
79
Hereditary haemachromatosis is associated with which malignancy?
HCC
80
Glycogen storage disease type 1 (von Gierke disease) is associated with which malignancy?
Hepatocellular carcinoma, liver adenomas
81
What is the genetics and what are the common malignancies associated with Diamond Blackfan anaemia
Autosomal dominant; mutations in the small or large subunit-associated ribosomal protein genes (most often RPS19)
Colorectal and other GI cancers, AML, MDS, osteogenic sarcoma
82
Which syndrome?
```
Increased risk of:
Pleuropulmonary blastoma (PPB), cystic nephromas, ovarian-Sertoli-Leydig tumors, multinodular goiter
```
Autosomal dominant; associated with mutations in DICER1 gene
DICER1 syndrome
83
Autosomal dominant; mutations in ALK or PHOX2B gene is associated with _______
Neuroblastoma
84
Which syndrome?
Autosomal dominant; mutations in the mitochondrial enzyme succinate dehydrogenase protein family (SDHA, B, C, or D)
Associated with Paraganglioma, pheochromocytomas
Hereditary paraganglioma-pheochromocytoma syndrome
85
Hearing loss with _______ (chemo drug) is usually unilateral or bilateral and occurs in the 4,000 to 8,000 Hz range (high tone)
Cisplatin
86
Which oncological issue has highest risk of secondary malignancy?
Retinoblastoma,
then Hodgkin,
then Soft tissue sarcoma
87
Which childhood leukaemia is associated with DIC at presentation?
APML - Acute promyelocytic leukaemia
88
Risk factor for subsequent papillary thyroid cancer?
Ionising radiation!
89
Which congenital tumour is often present at birth affecting the kidneys?
Mesoblastic nephroma
Wilm's in preschool to school age children
90
Four ways to differentiate Wilms tumour and neuroblastoma?
Wilms: Happy ; Neuroblastoma: sick and sad
Wilm's: Unilateral flank mass ; Neuroblastoma: Crosses midline
Wilm's tumour: hypertension common; Neuroblastoma: uncommon
Wilm's tumour: haematuria ; Neuroblastoma: Urine catelcholamines
Wilm's tumour: metastasise to lungs; Neuroblastoma: almost never got o lungs
91
Is Wilm's or Neuroblastoma most likely to metastasise to the lungs?
Wilms
92
Which type of lymphoma commonly associated with intusucception?
Burkitts Lymphoma
| Mature B cells
93
Differentiating medulloblastoma and pilocytic astrocytoma?
Medulloblastoma is more solid; astrocytma is more cystic in appearance
94
Osteosarcoma typically occurs at which location?
Ewings occur at which location?
Osteosarcoma - long bones, near the joint i.e. knee
Ewings - long bones and mid shaft
95
15:17 translocation associated with ..
APML
good prognosis if you survive DIC
96
Etoposide can have a common classic side effect ...
Therapy related AML
97
Rituximab targets CD_
CD 20
98
Which biologic do you use for Burkitts Lymphoma?
Rituximab - CD 20
99
Which CNS tumour is associated with multiple cranial nerve palsies?
Diffuse intrinsic pontine glioma
100
Which cancer is associated with anti-NMDA receptor encephalitis?
Ovarian teratoma
101
Where is the primary site of disease in a Non-Hodgkin lymphoma of B-Cell lineage?
Intestine
