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BPT exams > Oncology > Flashcards

Oncology Flashcards

1
Q

What is B cell ALL standard risk?

A

Age 1-10 and WCC <50 at presentation

All others are high risk

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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!

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3
Q

What translocations in ALL are bad?

A

t9: 22 (robertsonian)
4: 11 translocation KMT2a (MLL)

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4
Q

What translocation in ALL are good?

A

t(12:21) ; TEL/AML1 or ETV6 -RUNX1

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5
Q

What is the cure rate for all leukaemia?

A

90%

Standard risk - 92%

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6
Q

Fusion of BCR (from chromosome 22) and ABL1 (from chromosome 9) creates abnormality in which protein?

A

Tyrosine Kinase

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7
Q

What tyrosine kinase inhibitors are used alongside?

A

Imatanib and Dasatinib

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8
Q

What does Auer rod mean?

A

AML !

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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

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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

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11
Q

What is CAR-T cell therapy?

A

These are engineered or autologous chimeric antigen receptor T-cells to target specific cells

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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

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13
Q

What is Gemtuzumab?

A

It targets CD33 and used against AML

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14
Q

Haematopoetic stem cell is characterised by CD __

A

CD34 expression

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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

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16
Q

_______ donor is better for metabolic disorders

A

Unrelated cord blood

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17
Q

Which chemo agents can predispose for VOD?

A

Busulphan

Gemtuzumab

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18
Q

Von Hippel–Lindau (autosomal dominant) is associated with which sort of brain tumours?

A

Haemangioblastoma

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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

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20
Q

Chemotherapy agents given singly or in combination for _____ include carboplatin, vincristine, lomustine, procarbazine, temozolomide, and vinblastine

A

Low grade astrocytomas

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21
Q

Astrocytomas associated with tuberous sclerosis have responded to _________

A

Everolimus - mammalian target of rapamycin inhibitor

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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

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23
Q

What is the most common brain tumour in children <1yr

A

Choriod plexus tumours

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24
Q

Choriod plexus tumours are commonly associated with ______

A

Li Fraumeni syndrome

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25
Q

Neuronal differentiation is more common among Medulloblastoma and is characterized histologically by the presence of _________________

A

Homer Wright rosettes and immunopositivity for synaptophysin.

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26
Q

The most common abnormality in medulloblastoma involves _____________, which occur in 30–40% of all cases

A

Chromosome 17p deletions

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27
Q

________ are collections of myeloid leukemia cells and can occur throughout the neuraxis

A

Chloromas

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28
Q

Most common brain tumour to metastasize?

A

Medulloblastoma

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29
Q

bHCG is 100% positive in

A

Choriocarcinomas

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30
Q

What is the mechanism of action of Allopurinol and what complications can occur?

A

Allopurinol is a Xanthine Oxidase inhibitor - it inadvertently alkalises the urine and can precipitate Calcium phosphate stones

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31
Q

How does Rasburicase work?

A

It is a urate oxidaser and converts uric acid into allatoin which is more water soluble

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32
Q

What are the three cardinal electrolyte abnormalities with TLS?

A

HypoCa, HyperK+, HyperPO4

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33
Q

Name 8 common live vaccines?

A 
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
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34
Q

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.

A

<20% blasts -> if more, it is AML

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35
Q

Osteosarcoma occurs in teenage boys but is associated with a ________ on x-ray.

A

Sunburst lesion

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36
Q

Onion peel or moth eaten appearance is associated with ________

A

Ewings Sarcoma

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37
Q

Name three Ribonucloetide reductase inhibitors and their mechanism of action?

A

Hydroxyurea - blocks RRI by binding to iron molecule

Fludarabine/Gemcitabine - blocks RRI and DNA polymerase

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38
Q

Name two topoisomerase inhibitors and their mechanism of action?

A

Irinotecan/topotecan - inhibit topoisomerase I

Etoposide/Teniposide - inhibit topoisomerase II

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39
Q

What are three antitumour antiobiotics and how do they work?

A

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

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40
Q

_________ can prevent cardiotoxicity from Anthracyclines

A

Dexrazoxane

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41
Q

Antimetabolites mimic nucleobases or folic acid to prevent DNA syntheses

  • ________ are mimiced by Azathioprine and Cladribine
  • _________ are mimiced by Cytarabine and 5-Fluorouracil
A
  • Purines are mimiced by Azathioprine and Cladribine (A and G)
  • Pyrimidines are mimiced by Cytarabine and 5-Fluorouracil (C and T)
42
Q

What is the mechanism of action of cytarabine?

A

Binds to DNA polymerase

43
Q

Methotrexate can cause bone marrow suppression and megaloblastic anaemia (can be reversed by _______)

A

Can be reversed by Leuovorin

44
Q

Which chemotherapy agent is associated with delayed nausea by RACP?

A

Cisplatin

45
Q

XLP type I is associated with the absence of _______ can lead to an ___________

A

absence of SH2D1A can lead to an uncontrolled cytotoxic T-cell immune response to EBV

46
Q

Three common manifestations of XLP?

A

1) Fulminant mononucleosis (50%)
2) Lymphoma (25%)
3) Hypogammaglobulinaemia (25%)

47
Q

Methotrexate competitively inhibits _____________, an enzyme that participates in the tetrahydrofolate synthesis

A

Dihydrofolate reductase (DHFR)

48
Q

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.

A

Thymidine

49
Q

What lab anomaly are seen with rhabdomyosarcoma?

A

Hypercalcemia; blood cytopenia caused by bone marrow involvement

50
Q

What is WAGR and what is the associated mutation?

A

Wilms, Aniridia, GU anomalies and mental retardation

Assoc with Del of 11p13 and WT1 and PAX

51
Q

What are the clinical manifestations of Denys-Drash and what is the associated genetics?

A

Renal failure + Mesangial sclerosis + male pseudohermphrodism

genetics - WT1 missense mutation

52
Q

What is the genetics for Beckwith Weideman syndrome?

A

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
Q

What are the clinical manifestations of Frasier syndrome?

A

Undermasculinisation of genitalia, FSGS and gonadoblastoma (also Wilms)

WT1 intron 9 variant

54
Q

Chromosome 13q deletion syndrome is associated with which oncological manifestation?

A

RB1 gene deletion - retinoblastoma and sarcoma

Also associated with intellectual disability, characteristic craniofacial abnormalities

55
Q

Trisomy 21 is associated with increased risk of which cancer?

A

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
Q

Klinefelter syndrome (47, XXY) is associated with which oncological anomaly?

A

Breast cancer, extragonadal germ cell tumors

57
Q

Xeroderma pigmentosum is associated with which oncological anomaly?

And mode of inheritance?

A

SCC, BCC and melanoma

Autosomal recessive; failure to repair UV-damaged DNA; XP gene mutations

58
Q

Fanconi anemia is associated with which cancer?

A

AML, MDS, ALL

Autosomal recessive, mutations in FANCX gene family

59
Q

Which cancers is Bloom syndrome associated with?

A

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
Q

Ataxia-telangiectasia is associated with which oncological anomaly?

A

Lymphoma, leukemia, less often central nervous system and other solid tumors

61
Q

What is the mode of inheritance of ataxia-telengectasia and other clinical findings?

A

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
Q

This syndrome is associated with increased risk of leukaemia and lymphoma - it is associated with microcephaly, characteristic facies, immunodeficiency; biallelic mutations in NBN gene

A

Nijmegen breakage syndrome

63
Q

This syndrome is associated with soft tissue carcinoma and melanoma - you also see with accelerated aging; autosomal recessive and mutations in WRN gene

A

Werner syndrome (progeria)

64
Q

X-linked recessive; WASP gene mutations and associated increased risk of leukaemia and lymphoma….

which syndrome?

A

Wiskott Aldrich syndrome

65
Q

X-linked agammaglobulinemia (XLA) is associated with increased risk of which cancer?

A

Lymphoproliferative disorders, colorectal cancer

66
Q

X-linked or autosomal recessive; mutations in IL2RG and ADA genes and increased risk of leukaemia/lymphoma - which syndrome?

A

SCID

67
Q

NF2 is associated with increased risk of which syndrome?

A

Bilateral acoustic neuromas, meningiomas

68
Q

Noonan syndrome:

Genetics involved?
Increased risk of which malignancy?

A

Malignancy - JMML, ALL, neuroblastoma, brain tumors

Genetics - Autosomal dominant; caused by RAS/MAPK pathway mutations (most frequently PTPN11)

69
Q

Which syndrome?

Autosomal dominant; mutations in PTCH1 or SUFU gene and increased risk of BCC and meduloblastoma

A

Gorlin-Goltz syndrome

70
Q

Apart from Wilms which other cancers is BWS associated with?

A

Wilms tumor, hepatoblastoma, neuroblastoma, rhabdomyosarcoma

71
Q

Von Hippel Landau syndrome:

Genetics?
Clinical features ? (malignancy)

A

Genetics - Autosomal dominant; mutations of tumor-suppressor VHL gene

Clinical features - Hemangioblastomas of the brain and retina, pheochromocytoma, renal cell carcinoma

72
Q

MEN1 is associated with hyperparathyroidism, ZES;

Caused by autosomal dominant; mutations in MEN1 tumor suppressor gene

What are the associated malignancies?

A

Parathyroid, pancreatic islet cell and pituitary tumors

73
Q

What are the associated malignancy with MEN2?

A

Medullary thyroid carcinoma, parathyroid tumors, pheochromocytoma

74
Q

Apart from colon cancer what are the two other malignancies associated with FAP?

A

Hepatoblastoma and thyroid cancer

75
Q

Autosomal dominant; mutations in BMPR1A and SMAD4 gene … which syndrome?

A

Juvenile polyposis

76
Q

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

A

Hereditary nonpolyposis colorectal cancer (HNPCC, Lynch syndrome)

77
Q

Which syndrome:

Autosomal dominant; mutations in APC or MLH1 gene

Increased risk of colorectal cancer, brain tumors (glioblastoma, medulloblastoma)

A

Turcot syndrome

78
Q

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?

A

Peutz-Jeghers syndrome

Associated with breast cancer, colorectal cancer

79
Q

Hereditary haemachromatosis is associated with which malignancy?

A

HCC

80
Q

Glycogen storage disease type 1 (von Gierke disease) is associated with which malignancy?

A

Hepatocellular carcinoma, liver adenomas

81
Q

What is the genetics and what are the common malignancies associated with Diamond Blackfan anaemia

A

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
Q

Which syndrome?

Increased risk of:
Pleuropulmonary blastoma (PPB), cystic nephromas, ovarian-Sertoli-Leydig tumors, multinodular goiter
A

Autosomal dominant; associated with mutations in DICER1 gene

DICER1 syndrome

83
Q

Autosomal dominant; mutations in ALK or PHOX2B gene is associated with _______

A

Neuroblastoma

84
Q

Which syndrome?

Autosomal dominant; mutations in the mitochondrial enzyme succinate dehydrogenase protein family (SDHA, B, C, or D)

Associated with Paraganglioma, pheochromocytomas

A

Hereditary paraganglioma-pheochromocytoma syndrome

85
Q

Hearing loss with _______ (chemo drug) is usually unilateral or bilateral and occurs in the 4,000 to 8,000 Hz range (high tone)

A

Cisplatin

86
Q

Which oncological issue has highest risk of secondary malignancy?

A

Retinoblastoma,
then Hodgkin,
then Soft tissue sarcoma

87
Q

Which childhood leukaemia is associated with DIC at presentation?

A

APML - Acute promyelocytic leukaemia

88
Q

Risk factor for subsequent papillary thyroid cancer?

A

Ionising radiation!

89
Q

Which congenital tumour is often present at birth affecting the kidneys?

A

Mesoblastic nephroma

Wilm’s in preschool to school age children

90
Q

Four ways to differentiate Wilms tumour and neuroblastoma?

A

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
Q

Is Wilm’s or Neuroblastoma most likely to metastasise to the lungs?

A

Wilms

92
Q

Which type of lymphoma commonly associated with intusucception?

A

Burkitts Lymphoma

Mature B cells

93
Q

Differentiating medulloblastoma and pilocytic astrocytoma?

A

Medulloblastoma is more solid; astrocytma is more cystic in appearance

94
Q

Osteosarcoma typically occurs at which location?

Ewings occur at which location?

A

Osteosarcoma - long bones, near the joint i.e. knee

Ewings - long bones and mid shaft

95
Q

15:17 translocation associated with ..

A

APML

good prognosis if you survive DIC

96
Q

Etoposide can have a common classic side effect …

A

Therapy related AML

97
Q

Rituximab targets CD_

A

CD 20

98
Q

Which biologic do you use for Burkitts Lymphoma?

A

Rituximab - CD 20

99
Q

Which CNS tumour is associated with multiple cranial nerve palsies?

A

Diffuse intrinsic pontine glioma

100
Q

Which cancer is associated with anti-NMDA receptor encephalitis?

A

Ovarian teratoma

101
Q

Where is the primary site of disease in a Non-Hodgkin lymphoma of B-Cell lineage?

A

Intestine

BPT exams (24 decks)

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