Oncology Flashcards
Risk factors for ALL?
- Prenatal radiation exposure
- Postnatal exposure to high doses of radiation
- Down syndrome
- Ataxia- telangiectasia
- Fanconi anaemia
- Neurofibromatosis
- Bloom syndrome
Top 4 most common childhood malignancies?
Leukaemias (25%)
CNS tumours (17%)
Neuroblastomas (7%)
NHL (6%) and Wilms (6%)
Key pathology finding in Hodgkins Lymphoma
Reed-Sternberg cells, large multinucleated cells that divide rapidly and live longer than normal cells
Histologic subtypes of Non-Hodgkin Lymphoma
- Burkitt lymphoma
- Lymphoblastic lymphoma
- Diffuse large B cell
- Anaplastic large cell
Common brain tumour associated with NF1?
OPTIC GLIOMAS
Meningiomas
Ependymomas
Common brain tumour associated with NF2?
BILATERAL VESTIBULAR SCHWANNOMA
regional glioma
meningioma
glioma
schwannomas (cranial and peripheral nerve)
Common brain tumour associated with tuberous sclerosis?
Subependymal giant cell tumours
What is rasburicase
Used in patients with TLS with high rate levels or those at high risk of TLS
It is an enzyme that degrades uric acid
AFP is a tumour marker for which cancers?
Hepatoblastoma and HCC
Yolk sac tumours
Immature teratomas
embryonal carcinomas
Beta hCG is a tumour marker for which tumours
Choriocarcinomas
Seminomas
Some dysgerminomas
Tumour markers for neuroblastoma
Urine catecholamines (VMA and HMA)
Neuron specific enolase (NSE)
Cancers associated with Beckwith-Wiedemann Syndrome?
Hepatoblastoma
Wilms Tumour
Cancers associated with Gorlin Syndrome
- BCC
- Medulloblastoma
PTCH gene mutation
Staging of Wilms Tumour
- Stage 1: limited to 1 kidney
- Stage 2: extends beyond the kidney but can be completely excised
- Stage 3: confined to abdomen and local lymph nodes
- Stage 4: haemogenous spread - commonly to lungs
Stage 5: bilateral kidney involvement (5-10%)
Which cancers are associated with Li Fraumeni syndrome?
Sarcomas
Breast cancer
Adrenocortical cancer
Brain cancer
ALL
Lymphoma (esp HL)
PTEN hamartoma syndrome is associated with what clinical features and cancer predisposition?
- Acral keratosis (papules on hands/feet)
- Facial papules
- Hamartomas tumours of the outer hair sheath follicle
increased risk of THYROID, breast, endometrial, kidney, and colorectal cancers.
Cancers associated with ataxia-telangiectasia?
- Leukaemia and lymphoma
- Breast cancer
Cancers associated with HNPCC (Lynch Syndrome)
- Colorectal
- Endometrial and ovarian
Cancers associated with familial adenomatous polyposis? (APC gene mutation)
- Colorectal
- Hepatoblastoma
- Thyroid
Cancer associated with Noonan Syndrome?
Myeloproliferative disorder/ Juvenile Myelomonocytic Leukaemia (JMML) - A rare, agressive leukaemia
Management of Philadelphia chromosome positive ALL?
Need addition of tyrosine kinase inhibitor (eg imatinib)
Poor prognostic indicators in ALL?
- Age <1 or >10 at diagnosis
- Philadelphia chromosome t(9;22)
- T cell leukaemia
- Extramedullary disease (ie CNS or testicular disease)
- Measurable residual disease at end of induction (>0.01% blasts)
- Mature B cell leukaemia
- WCC>50 at presentation
(Hyperdiploidy, rapid response to treatment, trisomies of chromosome 4 and 10 and T(12;21) translocation are GOOD prognostic factors)
Chloromas (green-hued skin lesions), subcutaneous nodules (“blueberry muffin”) and infiltration of gingiva are distinguishing features of which cancer?
AML
Pathognomonic blood film finding in AML?
Auer rods (inside peripheral blood cell blasts)
Common condition seen in early infancy in T21?
Transient abnormal myelopoeisis (circulating blasts, often associated with hepatomegaly)- resolves spontaneously in most cases
Side effects of cyclophosphamide?
- Haemorrhagic cystitis (give Mesna)
- Anaphylaxis
- secondary malignancies
- Myelosuppression
- Infertility
Side effects of doxorubicin /anthracyclines?
Cardiomyopathy + arrhythmia
Red urine
Tissue necrosis with extravasation
Myelosuppression
Which chemotherapy agent commonly causes ototoxicity?
Platinums (eg cisplatin, carboplatin)
Which chemo agent commonly causes constipation?
Vincristine
Which chemo agent commonly causes peripheral neuropathy?
Vincristine
Which chemo agent commonly causes diarrhoea?
Irinotecan
Which chemo agents cause pulmonary fibrosis?
Bleomycin
Busulfan
Main side effects of methotrexate?
Myelosuppression
Mucositis
Hepatitis
Stomatitis
High dose - renal toxicity, neurocognitive effects
Long term: osteopenia
Which chemo agents need genetic testing performed to ensure toxic dosing not given?
Mercaptopurine and azathioprine
Thiopurine S- methyltransferase (TMPT) gene encodes one of the metabolising enzymes of thiopurines (azathioprine, mercaptopurine). Low levels of this enzyme -> accumulation -> severe myelosuppression
5 Features of veno-occlusive disease?
- Weight gain >5% above baseline
- Tender hepatomegaly
- Ascites
- Rising bilirubin from baseline on 3 consecutive days
- refractory thrombocytopenia
Highest risk in first month post bone marrow transplant
Management of veno-occlusive disease?
Defibrotide (a single stranded polydeoxyribomucleotide which has local anti-thrombotic, anti-ischaemic, anti-inflammatory activity)
Supportive care: diuretics, analgesia, electrolyte replacement
Mixed linear leukaemia is commonly seen in which type of leukaemia?
Infant ALL. Rare disorder with much poorer outcome
Acute promyelomyelocytic leukaemia
- May present with DIC
- If survive induction, often very good prognosis
- Manage with retinoid acid +/- arsenic
What are CAR-T cells?
Engineered autologous T cells
- T cells taken out, reprogrammed using a viral vector to recognise cancer cells/ other cells expressing a particular antigen - infused after lymphodepleting chemotherapy
Used in B cell malignancy (eg B cell lymphoma, CD19+ ALL)
Which type of HSCT has the highest rates of GVHD?
Peripheral blood stem cells > bone marrow > cord blood
Order of preference for Allogenic HSCT?
- Matched sibling donor (Marrow >PBSCs)
- Matched unrelated donor - BM
- Matched unrelated donor - cord
- Mismatched familial haploidentical donor- BM
- Matched unrelated donor - PBSC
- Miamatched unrelated donor - cord > marrow > PBSC
Acute GVHD affects which organs?
Skin
Liver
Intestines (diarrhoea)
Risk factors for Acute GVHD
HLA mismatch
Unrelated donor
Sex mismatch
Total body irradiation
High T cell numbers in donor
Low concentration of recipient immunosuppressive meds
Management of acute GVHD
- Optimise immunosuppression
- Increase ciclosporin
- Steroids
- Wet wraps
Poor prognostic factors in hyperleukocytosis (WCC >100)?
- WCC >200
- Age <1 or >10yrs
- AML (due to size of blasts)
Associated with neuromuscular and Resp complications, DIC and tumour lysis syndrome
Features of posterior reversible encephalopathy syndrome (PRES)?
Headache
HTN (75%)
Confusion
Convulsions
Vision loss
MRI; bilateral hyperintense densities in parieto-occipital region on T2
Which chemotherapies are associated with increased risk of secondary AML/ myelodysplastic syndrome?
Etoposide
Alkylating agents (eg cyclophosphamide)
