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FRACP Exam > Oncology > Flashcards

Oncology Flashcards

1
Q

5-fluorouracil

A

class: antimetabolite

mechanism: pyrimidine analogue

use: breast/GI

side effects: similar to MTX plus ataxia, esophagitis, stomatitis

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2
Q

6-Mercaptopurine

A

class: antimetabolite

mechanism: purine analogue

use: ALL

side effects: hepatotoxicity, mucositis, myelosuppression, pneumonitis

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3
Q

Acute GVHD

A

onset: 2-5wks but within 3 months

incidence: 30% HSCT from matched sibling, 60% unrelated donor

pathogenesis:

  1. recipient APC meets donor T cell and secrete cytokines
  2. donor T cells activated in response to cytokines and secrete TNF, IL2, IFN-g
  3. donor cytokines cause tissue famage and promote activation CD8 killer T cells

clinical:

  • maculopapular rash, anorexia, N/V/D
  • skin: erythema, pain, burning
  • GIT: mucosal ulceration, destruction of crypts, abdominal pain, PR bleeding
  • liver: jaundice

histology: endothelial damage and lymphocytic infiltrate, apoptotic bodies

management: prednisone (50% recover 20 days), MMF, monoclonal Ab

  • poor outcome if steroid resistant
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4
Q

Age of malignancies

A

Age <1 yr:

  1. Neuroblastoma
  2. Leukaemia
  3. CNS tumours
  4. Wilm’s tumour
  5. Lymphoma

Age 1-14yrs:

  1. Leukaemia
  2. CNS tumours
  3. Lymphoma
  4. Wilm’s/Germ cell/NB

Age 15-19yrs:

  1. Lymphoma
  2. Germ cell tumours
  3. Leukaemia
  4. CNS tumours
  5. Thyroid cancer
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5
Q

ALL

A

incidence: peak 2-3yrs, M>F

risk factors: genetic (T21, Bloom, Ataxia-telangiectasia, Fanconi anaemia), high BW, radiation, drugs (cyclophosphamide, eposfomide), advanced mat age, MZ twin with ALL (70%)

Indications HSCT: Philadelphia, extreme hypoploidy, Tcell with relapse, ALL early relapse, ALL poor early response

PreBALL relapse: consider different chemo of BMT

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6
Q

AML/ALL

clinical/diagnosis

A

clinical:

initial: anorexia, fatigue, malaise, low grade fever

BM failure: anaemia (pallor/hypoxia), thrombocytopaenia (purpura/bleeding), neutropaenia (fever>5days)

blastic infiltration: hepatosplenomegally, LN, bone pain, CNS sx, testicular pain, subcutaneous nodules, respiratory distress (mediastinal disease)

emergencies: leucostasis AML>ALL, TLS, mediastinal compression (T cell ALL), coagulopathy (APML), febrile neutropaenia

diagnosis:

blood film

  • ALL: monomorphic cells, large/small lymphoblasts with large nucleoli and minimal blue cytoplasm
  • AML: polymorphic lymphoblasts with Auer rods, more cytoplasm

bone marrow: >25% BM are lymphoblasts

CSF: worse prognosis with CSF infiltration

Immunophenotype: FLOW cytometry

Cytogenics aids in prognostics: FISH, SNP microarray, gene expression profile

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7
Q

Aneurysmal bone cyst

A

etiology: benign expansive vascular lesion which grow rapidly and destroy bone

clinical: F>M, adolescents, any bone (femur, tibia), localised pain, pathologic fractures, swelling

XR: aggressive, expansile, ltyic metaphyseal lesions with eggshell sclerotic rim

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8
Q

Anthracycline

Daunorubicin

Doxorubicin

A

class: antitumour antibiotics

effect: inhibits topoisomerase II

use: ALL, AML, NB, NHL, Sarcoma, Wilm’s

side effects: cardiotoxicity, dermatitis, diarrhoea, myelosuppression, red urine

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9
Q

Asparaginase

A

class: enzymes

mechanism: catalyses hydrolysis of asparaginase to aspartic acid

  • depletes asparaginase

use: ALL, AML

side effects: anaphylaxis, CNS depression, blood disoriders, pancreatitis

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10
Q

Beckwith-Wiedemann

Cancer

A

common tumours:

  • Wilm’s tumour up to 15%
  • hepatoblastoma

others: NB, adrenocortical carcinoma, rhabdomyosarcoma

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11
Q

Bleomycin

A

class: antitumour antibiotics

mechanism: binds to DNA and cuts

use: lymphoma, germ cell tumours

side effects: dermatitis, mucositis, pneumonitis/pulmonary fibrosis, Raynaud’s, stomatitis

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12
Q

Bone Marrow Transplant

A

allogenic: from another person

autologous: from oneself

preparative condition: myeloblative chemotherapy

  • high dose immunosuppression

Day 0: BMT infusion

  • IV donor cells
  • intrabone injection of cord blood cells into marrow

Day 0- 19: aplastic phase

Day 20: engraftment

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13
Q

Brain tumours

A

supratentorial:

  • pineoblastoma (PNET) (3%)
  • craniopharyngioma (10%)
  • choroid plexus carcinoma (4%)
  • germ cell tumours (germinoma/teratoma) (5%)

infratentorial:

  • cerebellar pilocytic astrocytoma (5%)
  • medulloblastoma (20%)
  • ependymoma (10%)
  • brainstem glioma (20%)
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14
Q

Brainstem glioma

A

incidence: 5-8yrs, 20% Paed BT

location: brainstem

features: cystic, well demarcated, non-infiltrating, non oedematous

clinical: long history, neck stiffness, CN symptoms, ataxia, N/V

associations: NF-1

treatment: resection, radiation, chemo

survival: 50%

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15
Q

Cell-cycle dependent chemotherapy

A

Phase-dependent drugs: kill cell at lower dose

  • prolonged dosing to ensure maximum cells receive insult at right time in cycle

G1: L-asparaginase (enzyme)

S drugs:

  • cytarabine: inhibits DNA polymerase
  • steroids: suppress transcription factors
  • antimetabolites:
  • Folinic acid antagonists- methotrexate
  • Pyrimidine antagonists- 5FU/ cytosine arabinoside
  • Purine antagonists-mercaptopurine/ 6MP/ thioguanine

G2 drugs:

  • topoismoerase inhibitors- etoposide
  • bleomycin

M phase: vinca alkaloids

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16
Q

Chemo side effects

A
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17
Q

Chemotherapy agents

-secondary malignancy-

A

1. Alkylating agents + cisplatin: cyclophosphamide, ifosfamide

2. Topoisomerase inhibitors: etoposide

3. Anthracyclines: daunorubicin, doxorubicin

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18
Q

Chemotherapy pharmacokinetics

A

1st order kinetics: kill constant fraction of tumour cells at any one time

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19
Q

Chlorambucil

A

class: alkylating agent

mechanism: interfers DNA crosslinking by alkylating guanine

use: bladder, ovarian, testicular cancer

side effects: infertility, neuropathy, ototoxicity, pneumonitis, renal impairment

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20
Q

choroid plexus carcinoma

(supratentorial)

A

incidence: 12-16yrs, 4% Paed BT

location: choroid plexus

features: aggressive, focal necrosis, oedema

associations: Li Fraumeni

clinical: FTT, macrocephaly, headaches, N/V

treatment: resection, radiation

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21
Q

Chronic GVHD

A

definition: persistance >3m post transplant

  • behaves like AI disease

incidence: 25% HSCT patients

risk factors: acute GVHD, HLA mismatch, HSV, peripheral SCT, female donor for male recipient, higher age, total body irradiation

pathophysiology:

  • autoantibody production with collagen deposits/fibrosis
  • due to persistent donor T cells not tolerant to recipient

clinical: limited or extensive

  • skin: lichen planus, scleroderma, malar rash
  • arthritis, joint contractures, obliterative bronchiolitis, bile duct degeneration

prognosis: skin/liver only is favorable

  • extensive disease has high morbidity/mortality
  • most resolve but may take 1-3yrs
  • lower life expectancy, metabolic syndromes, secondary malignancy

treatment: as for GVHD + AB for prolonged immunosuppression

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22
Q

Cisplatin

A

mechanism: inhibits DNA synthesis

use: osteosarcoma, NB, CNS tumours, germ cell tumours

side effects: delayed N/V, neuropathy, ototoxicity, renal impairement/uraemic syndrome, SIADH

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23
Q

Other Renal Tumours

A

clear cell sarcoma: 3% kidney tumours, 1-4yrs, good prognosis

rhabdoid tumour of kidney: 80%<2yrs, poor prognosis, high metastatic rate, 15% synchronous brain lesion, clinical fever/haematuria/hyperglycaemia

renal cell carcinoma: 20% mets at diagnosis, age 10 yrs

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24
Q

CML

A

99% due to Philadelphia chromosome t(9;22) translocation leading to BCR-ABL fusion protein

pathogenesis

initial chronic phase (3-4yrs): malignant proliferation mature WBCs and increased immature cells

  • associated splenomegally, anaemia, thrombocytopaenia

blast crisis: similar to ALL, increased risk TLS/hyperleukocytosis

  • fever, malaise, lethargy, anorexia

diagnosis: blood/BM, cytogenics (philadelphia chromosome)

management: imatinib (70% cure), HSCT (80% cure)

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25
Q

Coagulopathy

(cancer)

A

incidence: AML esp acute promyeloblastic leukaemia

clinical: DIC/fibrinolysis

treatment: FFP (maintain >1), platelets (maintain 30-50)

prognosis: 30% die haemorrhage in <2 weeks and often before starting treatment

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26
Q

Coloid cyst

A

etiology: rare developmental lesion with outer fibrous later and inner epi of mucin producing cells

location: 3rd ventrical

  • can block foramen of Munro causing hydrocephalus

clinical: symptoms raised ICP 30-60yrs

treatment: surgery +/- shunt

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27
Q

craniopharygioma

(supratentorial)

A

incidence: 5-14yrs, 10% Paed BT

location: suprasellar region (remnant Rathke’s pouch)

features: benign, solid, fluid filled cysts, calcification

clinical: long history of symptoms, visual sx, endocrine sx, headache, N/V

treatment: resection, radiation

survival: 90%

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28
Q

Cyclophosphamide

A

class: alkylating agent of the nitrogen mustard type

  • adds alkyl group to DNA interferes with DNA crosslinks

use: cancers (ALL, AML, Burkitt’s, NHL, Ewing’s sarcoma), autoimmune disorders, amyloidosis

side effects:

  • alopecia, N/V, BM suppression, stomach ache, haemorhagic cystitis, pulmonary fibrosis
  • AML, bladder cancer, haemorrhagic cystitis, infertility
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29
Q

Cyclophosphamide

A

class: alkylating agent

mechanism: interfers DNA crosslinking by alkylating guanine

use: ALL, AML, lymphoma, sarcoma

side effects: myelosuppression, hyperuricaemia, haem cystitis, pneumonitis/fibrosis, SIADH, secondary malignancy

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30
Q

Cytosine arabinoside

(ara-c)

A

class: antimetabolite

mechanism: pyrimidine analogue

use: ALL, AML, lymphoma

side effects: similar to MTX, conjunctivitis, neurotoxicity

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31
Q

Dactinomycin

A

class: antitumour antibiotic

mechanism: binds DNA inhibiting transcription

use: Wilm’s, rhabdo, Ewing’s

side effects: dermatitis, diarrhoea, myelosuppression, tissue necrosis on extravasation

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32
Q

Delayed onset emesis in chemotherapy

A

associated agents: cisplatin, doxorubicin + cyclophosphamide

treatment: corticosteroids, ondansetron, precipitant (NK1R antag)

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33
Q

Diencephalic syndrome

A

pathogenesis: syndrome caused by tumour located in the diencephalon juve above the brainstem in the hypothalamus (glioma or astrocytoma)

clinical: FTT and emaciation/weakness

  • behave in happy and outgoing manner
  • vomiting, visual changes, headache and pallor can develop

treatment: surgery, radiation, chemotherapy

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34
Q

ependymomas

(infratentorial)

A

incidence: 2-9yrs, 10% Paed BT

location: ependymal lining (cranial 90%, spine 10%)

features: slow growing, well demarcated, calcification, haemorrhage, ependymal rosettes

associations: NF2

clinical: ataxia/vertigo, papilledema, CN VI-X palsy, seizures, increased ICP, headache, N/V

treatment: resection, RTX, +/- chemo

survival: 50%

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35
Q

Etoposide

A

mechanism: topoisomerase inhibitor

use: ALL, lymphoma, germ cell, sarcoma

allergic reaction, constipation/diarrhoea, mycositis, secondary leukaemia

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36
Q

Ewing’s sarcoma

A

incidence: more common <10yrs

location: diaphysis of long bones of extremities: femur, tibia, humerus

associations: t(11:22), EWS gene

clinical: fever, weight loss, pain, swelling, mass

  • more systemic sx than osteosarcoma

histology: small round blue cells

XR: poorly marginated destructive lesion with permeative/moth eaten appearance in association with soft tissue mass and periosteal reaction (onion skinning)

treatment: chemo, RTX, +/- resection

prognosis: 60% survival

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37
Q

Febrile neutropaenia

A

neutropaenia: nadir 7-10 days post chemo

definition:

fever >38.5 or sustained >38

neutropaenia <0.5

  • consider <0.1 v.low, cell quality, duration of neutropaenia
  • high risk >7 days or altered BM, low risk <7 days AND normal BM

clinical: fever may be only sign of infection

  • look EVERYWHERE for source

organisms: bacteraemia most common

  • can rapidly progress to septic shock
  • due to gut translocation of flora in 80%

gram positive: S.aureus, S.pneumo, S.viridans, VRE, CONS

gram negative: Pseudomonas, Ecoli, Klebsiella, Enterobacter, Acinetobacter, ESBL

fungal: increased with prolong AB/neutropaenia (>5days)

  • candida (entry GIT), aspergillus (via lungs, assoc steroids), cryptococcus, fusarium
  • treatment: amphotericin

viral: HSV, VZV, resp viruses

management: AB <60 mins of fever

  • empiric: Amikacin/Gent, Tazocin/Timentin, Vancomycin
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38
Q

germ cell tumours (germinoma/teratoma)

(supratentorial)

A

incidence: 11-16yrs, 3-5% Paed BT

location: pineal, suprasellar

features: high bHCG/AFP

clinical: hormone sx, visual sx, headache

treatment: radiation, chemo

survival: 90%

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39
Q

Gonadal/Germ cell tumour

A

incidence: rare

pathogenesis:

  • GCT (most malignant gonadal): primordial germ cell
  • non germ cell gonadal tumour: coelomic epithelium

GCT types: teratoma/germinoma/endodermal sinus tumour, embryonal carcinoma

sacrococcygeal tumours: infant girl

testicular germ cell tumours: boys<4yrs

Non-GCT types (UNCOMMON): epithelial carcinoma (ovary), sex cord stromal tumours (ovary/testis)

associations: del Ch 1p/6q, gain 1q, T21, undescended testis/inguinal hernia/testicular atrophy

diagnosis: AFP/bHCG, imaging, pelvic exploration

management: resection, chemo, RTX

prognosis: 80% cure, >12yrs increased x4 risk death

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40
Q

Graft failure

A

etiology: rejection usually due to different btw major/minor MHC

definition:

primary failure: failure to reach neutrophil count 0.2x109 within 21 days

secondary failure: loss of peripheral blood counts after initial engraftment

causes:

  • residual recipient T cells causing rejection of donor cells
  • inadequate SC dose
  • infections: CMV, HHV-6

risk factors: HLA disparity, T cell/stem cell deplete graft, reduced intensity conditioning, prophylaxis with MTX/bactim

diagnosis: BMA/biopsy (persistence of host lymphocytes suggests immunologic rejection)

treatment: remove myelotoxic drugs, G-CSF, second transplant

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41
Q

Graft verus Host Disease (GVHD)

A

etiology: engraftment of donor lymphocytes with T cell activation again recipient MHC

risk factors: BMT, nonmatched donor, high T cell vol. received, disparities in HLA groups, disparities in sex chromosome (female donor recognise male recipient), advanced disease, cell source (peripheral blood>BM>CB)

prophylaxis for GVHD:

T cell deplete donor: but increased immunocompromise/systemic infection post tx

post transplant immunosuppressants: methotrexate, MMF, calcineurin inhibitors

pathophysiology:

  1. tissue damage
  2. T cell mediated immune response
  3. apoptosis
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42
Q

Growth fraction

A

definition: proportion of cells dividing at any one time

tumours: have high GF (1-10%)

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43
Q

Hepatic Sinusoidal Obstructive Syndrome

A

definition: severe syndrome with hepatic vein obstruction, portal HTN, 3rd spacing, IV hypovolaemia

pathophysiology:

  • fibrous obliteration of venules/veins with damage to surrounding hepatocytes/sinusoids with hepatic necrosis/haemorrhage
  • not associated thrombus formation

etiology: post HSCT/chemo/alkaloid toxins/radiation/liver tx

- high risk chemo drugs: alkylating agents, platinum complexes, thiopurines

incidence: <10% post HSCT, F>M

clinical: 3 weeks post HSCT

  • weight gain, hepatosplenomegally, RUQ pain, jaundice, ascites

diagnosis: high AST/ALT/conj bilirubin, prolonged PTT, renal insufficiency

management: avoid hepatic damage, diifibrotide, monitor

prognosis: often resolvied but can lead to progressive organ failure, mortality<4%

44
Q

Hepatoblastoma

A

incidence: <3yrs

associations: BWS, trisomy 18, trisomy 21, Acardia syndrome, Li-Fraumeni syndrome, Goldenhar syndrome, Von-Gierke’s dusease, FAP

pathology 2 types:

  • epithelial type (embryonal malignant cells): better outcome
  • mixed (mesenchymal/epithelial elements)

clinical: large asymptomatic mass right lobe liver

diagnosis: AFP (ALWAYS elevated), bilirubin/LFTs (normal), anaemia/thrombocytopaenia (common)

management: resection, chemo (Cisplatin/Vincristine/5-FU/Doxorubicin), liver transplant if unresectable

prognosis: 90% survival complete resection, 60% partial resection

45
Q

Hepatocellular carcinoma

A

incidence: adolescence

etiology: secondary to HepB/HepC infection

associations: tyrosinaemia, galactosaemia, GSD, alpha-1 antitrypsin deficiency, biliary cirrhosis

treatment: partial resection, chemotherapy

46
Q

High/Low grade gliomas

A

incidence: 40% all braintumours

etiology: arise supportive tissues (astrocytes/oligodendrocytes)

subtypes: astrocytoma, oligodendroglial, ependymoma

low grade: pilocytic astrocytoma, diffuse astrocytoma, oligodendroglioma, mixed oligoastrocytoma, ganglioglioma, subepenpendymal giant cell astrocytoma

  • tx: resection, +/- chemo/RTX

high grade: anaplastic glioma (astrocytoma/oligodendroglioma/oligoastrocytoma), diffuse intrinsic pontine glioma, GBM, pilomyxoid astrocytoma

  • tx: resection, chemo, RTX
47
Q

Hodgkin Lymphoma

A

incidence: rare, age 15-19yrs, 7% of ALL childhood cancers

pathology: Reed-Sternberg cells (<1% of LN)

  • large/multiloculated nuclei with prominent central nucleolus with surrounding clearance
  • arise from germinal B cells

etiology: EBV found in 25-50% Reed-Sternberg cells

histology:

  • classic HL (95%): nodular sclerosing, mixed cellularity, lymphocyte rich or deplete
  • nodular lymphocytc predominant (5%): malignant cells different from Reed-Sternberg

clinical: lethargy, malaise, anorexia, lymphadenopathy rubbery/firm/painless (cervical, supraclav, axillary), hepatosplenomegally, mediastinal mass (75%)

** B symptoms (<20% of children), weight loss >10% in 6/12, fevers >38 for <3/7, night sweats

diagnosis: tissue biopsy, BMA

treatment:

  • low risk (stage IA/IIA): OEPA (vincristine/etoposide/prednisone/doxorubicin)
  • intermediate risk (stage IB/IIB): same as above but more cycles
  • high risk (stage IIIB/IVB): more chemo BEACOPP (bleomycin, etoposide, doxorubicin, cyclophophamide, vincristine, procarbazine, prednisone), MBT

prognosis:

  • low risk: >95%
  • high risk: 90%

poor prognosis: males, IIB/IIIB/IV, bulky mediastinal disease, high WCC, anaemia

48
Q

Hypercalcaemia

(cancer)

A

incidence: leukaemia/lymphoma (osteolytic), alveolar rhabdomyosarcoma (osteolytic), Non-Wilm’s renal tumour

clinical: confusion → dehydration → renal failure

management: hyperhydration, frusemide, bisphosphonates

49
Q

Hypothalamic hamartoma

A

definition: most common brain lesion to cause precocious puberty

etiology: congenital malformation of ectopic neural tissue

pathogenesis: glial cells in lesion produce GF beta that activates GnRRH pulse

MRI: penduculated mass attached to tuber cinereum of the hypothalamus

clinical: gelastic seizures, precocious puberty

50
Q

Ifosfamide

A

class: alkylating agent

mechanism: interfers DNA crosslinking by alkylating guanine

use: lymphoma, Wilm’s, sarcoma, germ cell tumours

side effects: cyclo effects + cardiotoxicity, neurotoxocity, nephrotoxicity, SIADH

51
Q

Indications for HSCT

A

non-malignant disease

congenital BM failure: Fanconi’s anaemia

single lineage deficiency: sickle cell, thalassemia, Kausman

blood cell dysfunction: chronic granulomatous disease

immune deficiencies: SCID (all), CVID (some)

immune dysfunction: hyper inflammation (HLH)

metabolic disease: enzyme replacement (Hurler, Gaucher, metachromatic leujodystrophy)

acquired organ failure: aplastic anaemia

stem cell replacement: osteopretrosis, epidermolysis

52
Q

Indications for HSCT

A

malignant disease

AML: cytogenic/molecular high risk group or poor MRD

ALL: Philadelphia chromosome, extreme hypoploidy, poor MRD, early relapse, T cell relapse

myelodysplastic syndrome

myeloproliferative syndromes: JMML, polycysthaemia vera, CML

53
Q

Juvenile Myelomonocytic Leukaemia

JMML

A

incidence: the only myeloproliferative disease in childhood

clinical: rash, LN, splenomegally, haemorrhage

blood film: elevated WCC, anaemia, thrombocytopaenia

diagnosis: monocytosis>1x109/L, blasts<20%

  • increased HbF, WBC, neutrophils

cytogenetics: nil distinctive

associations: NF1 (10% cases), Noonan syndrome

treatment: HSCT

54
Q

Kaposiform Haemangioendothelioma

KHE

A

definition: locally aggressive vascular tumour

epidemiology: 50% at birth, otherwise <1yr

location: extremities, 10% don’t involve skins (retroperitoneum, mediastinum, internal organs)

clinical: raised subcutaneous mass with purpuric appearance and occassional telangiectasias, increased hair growth over tumour

complications: Kasabach-Merrit phenomenon

  • rapid enlargement of tumour with low platelets/fibrinogen, high D-dimer/fibrin

diagnosis: biopsy, MRI

management: excission, laser, chemo

prognosis: can undergo regression but necer completely resolve, long term oedema/pain/ortho issues

55
Q

Histiocytosis Syndrome Class I

Langerhan’s cell histiocytosis (LCH)

A

diseases: eosinophilic granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease

pathology: proliferation of cells from the monocyte lineage (including Langerhan’s cells with Birbeck granules) causing accumulation of granulomatous materials throughout the body

clinical: multisystemic deposits everywhere

  • malaise, lethargy, irritability
  • bone (80%): commonly the skull
  • skin: scaly, popular, seborrhoeic dermatitis, gingival infiltrates
  • LN, hepatosplenomegally
  • eyes: retroorbital infiltrates/proptosis
  • pituitary dysfunction

diagnosis: tissue biopsy, skeletal survey

treatment:

  • single system disease if benign: low dose RTX
  • multisystem disease: systemic multiagent chemo
56
Q

Histiocytosis Syndrome Class II

Haemophagocytic Lymphohistiocytosis (HLH)

A

pathology: non-malignant proliferative condition with accumulation of APC (macrophages) and uncontrolled activation of inflammatory cytokines

etiology:

  • AR (mutations in perforin or Munc 13-4 proteins)
  • infection associated: better outcome

clinical: <4yrs, fever, maculopapular rash, weight loss, irritability, severe immunodeficiency, hepatosplenomegally, LN, resp distress, aseptic meningitis

diagnosis: BM biopsy or clinical features plus

  • hyperlipidaemia, hypofibrinogenaemia, elevated LFTs/IL2/ferritin, pancytopaenia

treatment:

  • familial: chemo with IT MTX, fatal with relapse, HSCT cures 60%
  • infectious: treat cause
57
Q

LEAST myelosuppressive chemotherapy

A

Prednisone

Asparaginase

Bleomycin

Vincristine

58
Q

Leucostasis/Hyperleucocytosis

A

leucostasis: WCC>50

hyperleucocytosis: WCC>50 PLUS white cell plugs in microvasculature

incidence: occurs in AML>ALL and CML blastic crisis

  • present in 20% newly diagnosed AML

clinical:

  • CNS: altered GCS, headache, dizziness
  • resp: hypoxia, dyspnoea
  • fever in 80%

RISK OF HAEMORRHAGE

treatment: cytoreduction, prophylaxis for TLS, platelet transfusion

59
Q

Leukaemias

A

30% of paediatric cancers

ALL 77% (PreB 85%, T cell 15%, B cell <1%)

AML 11%

CML 2-3%

JMML (Juvenille myelomonocytic leukaemia) 1-2%

60
Q

Leukaemias

Cell morphology

A

ALL: blasts >25%

  • small to intermediate blasts
  • basophilic, rarely granules
  • B cells: CD19, T cells: CD3

AML: blasts>20%

  • larger/irregular blasts
  • auer rods, granules
  • myeloperoxidase
61
Q

Leukaemias

genetics

A

ALL:

  • hyperdiploidy, t(12:21): good
  • hypodiploidy, t(BCR ABL): bad

AML:

  • >3 abnormalieis, t(9:11, t(9:22): bad
62
Q

Li-Fraumeni syndrome

A

SBLA cancer syndrome

  • Sarcoma
  • Breast
  • Leukaemia
  • Adrenal gland

genetics: AD, abnormality tumour protein p53 gene

diagnosis: proband with a sarcoma <45yrs + a 1st degree relative with cancer <45yrs + a 1st/2nd degree relative cancer <45yrs OR sarcoma

lifetime cancer risk: 100%

63
Q

Long term effects post transplant

A

CAD: 75% at 5 yrs due to chronic rejection (vasculopathy)

hypertension: 60% 5yrs due to steroids/cyclosporin

neoplasm: 20% 6m-6yrs usually lymphoproliferative

renal dysfunction: 25% at 5yrs assoc MMF/sirolimus

osteoporosis: 100% due to steroids/calcineurin inhib

behavioural issues: 33% at 5 yrs

64
Q

Mediastinal compression

(cancer)

A

etiology: vena caval/bronchotracheal tree compression

incidence: T cell ALL

clinical: facial oedema, dyspnoea, wheeze, orthopnoea

  • must exclude pericardial effusion
65
Q

medulloblastoma

(infratentorial)

A

incidence: 5-9yrs, 20% Paed BT (most common malig)

location: ONLY in cerebellum

features: evolves weeks-months, 1/3 metastasise to CSF

clinical: altered GCS, ataxia/poor coordination, visual sx (CN VI), headache, N/V, increased ICP

associations: FAP, NBCCS

treatment: resection, RTX, chemo

survival: 50%

66
Q

Methotrexate

A

class: antimetabolite

mechanism: folic acid antagonist (inhibits DHFR)

use: ALL, lymphoma, medulloblastoma, osteosarcoma

side effects: hepatotoxicity, infertility, mucositis, myelosuppression

67
Q

Neuroblastoma

A

origin: arise from primitive sympathetic ganglion cells

sites: adrenal 40%, abdo 25%, thoracic 15%, pelvic 5%, cervical 5%

symptoms:

  • abdominal pain
  • proptosis/periorbital echymosses
  • Horner’s syndrome
  • opsoclonus myoclonus syndrome
  • bone pain
  • scoliosis
  • HTN
68
Q

Neuroblastoma

A

definition: embryonal cancer of peripheral SNS

epidemiology: 40% cancer in infancy, 90% <5yrs, M>F

etiology: 1-2% familial (Phox28/ALK genes)

locations: adrenals 40%, abdominal 25%, thoracic 15%, cervical 5%, pelvic 5%

pathology:

neural type cells

  • neuroblastoma: undifferentiated, poorly differentiated, most aggressive, small blue cells

schwann-type cells:

  • ganglioneuroblastoma (schwann cells/neuroblasts): intermediate malignant potential
  • ganglioneuroma (schwann cells/mature ganglion cells): benign
  • ganglionneuroblastoma nodular

clinical: mass related symptoms, fever, periorbital echymoses (orbital mets), proptosis, Horner’s, abdo pain, constipation, bladd/bowel dysfunction

paraneoplastic syndromes: opsoclonus-myoclonus ataxia sx, VIP secretion (diarrhoea), HTN

diagnosis:

  • urine catecholamine intermediates: neuroblastoma cells can’t synthesis catecholamines so intermediates (homovanillic acid, vanillymandelic acid, dopamine) accumulate

poor prognostic: MYCN protooncogene (25% tumours), chromosomal deletions, ALK mutations, age>18months, neuroblastoma, nodular neuroblastoma, high mitosis-karyorrhexis (MKI)

good prognosis: low MK, ganglioneuroma

treatment

  • low risk: surgery
  • intermediate risk: 96% survival, chemo (cyclo/carbo/cisplatin/etoposide/doxorubicin), surgery
  • high risk: <40% survival, induction chemo, surgery, SCT, radiation, immunotherapy
  • relapsed NB is incurable
69
Q

NF-1 cancer

A

genetics: 17q11 NF1 gene

types:

optic pathway gliomas (15% <6yrs): low grade pilocytic astrocytomas

brain tumours: astrocytomas, brainstem gliomas

soft tissue sarcomas: rhabdomyosarcoma, GISTs, MPNSTs, glomus tumours

other: JML, phaeochromocytoma

70
Q

NF-2 cancer

A

genetics: AD 22q12, NF2 gene

types:

bilateral vestibular schwannomas

intracranial meningiomas

spinal tumours

71
Q

Non Hodgkin Lymphoma

clinical/diagnosis/treatment

A

clinical:

  • rapid onset 1-3 weeks, enlarging non tender LN, compression of surrounding structures, CNS involvement
  • emergency: TLS, SC compression, SVC obstruction, hyperviscosity sx, hyperleukocytosis

diagnosis: anaemia, thrombocytopaenia, leukopaenia, increased uric acid/LDH

staging: CT +/- PET (neck, chest, abdo, pelvis), BMA, LP

treatment: chemotherapy, radiation rarely, NO surgery

prognosis: 90% survival

72
Q

Non-cell cycle dependant chemotherapy

A

non-phase dependent drugs:

  • inactivate DNA: procarbazine
  • alkylating agents: cyclophosphamide, ifosfamide
  • platinum analogues: cisplatin
  • anthracyclines
73
Q

Non-Hodgkin Lymphoma

epidemiology/pathology

A

epidemiology: 5th most common paediatric malignancy, median age 10yrs

genetics: de novo

etiology:

  • congenital: Wiskott-Aldrich, ataxia-telangiectasia, X-linked lymphoproliferative syndrome
  • aquired: HIV, EBV etc.

types:

Burkitt Lymphoma: highly aggressive B cell NHL

  • age 4-6yrs, M>F
  • translocation/deregulation of c-MYC proto-oncogene on chromosome 8
  • moth eaten appearance to LN
  • 3 variants: endemic 50% (Africa-New Guinea), sporadic 30% (Western), Immunodef (HIV)
  • endemic: ALL EBV receptor positive
  • sites: abdomen, head, neck

Diffuse Large B Cell Lymphoma

  • most common NHL
  • denovo transformation of other low grade B cell lymphoma
  • film: big cells, multiple nuclei, scant cytoplasm
  • location: neck, abdomen, mediastinum, B symptoms in 30%
  • TLS is UNCOMMON

Lymphoblastic T and B cell lymphoma/Leukaemia pre-T and pre-B ALL

  • age 12yrs, M>F
  • lymphoma if mass lesion and <25% blasts in BM
  • leukaemia if >25% blast BM with or without mass lesion
  • cytology: polymorphic lymphoblasts, CD7/CD3 positive, TCR rearrangements 30%
  • location: peripheral LN, neck
  • clinical: resp distress, SVC syndrome, >60% get BM infiltration

Anaplastic Large Cell Lymphoma

  • aggressive peripheral T cell lymphoma
  • assoc translocation of ALK (anaplastic lymphoma kinase gene on Ch2p23)
  • clinical: fever, painless LN, skin/subcutaneous involvement
74
Q

Osteochondroma

A

etiology: bony spur from external surface of bone

  • cartilaginous cap overlies the spur and causes growth

genetics: AD germline mutation tumour suppressor genes EXT1/EXT2

clinical: 2nd decade M>F, painless mass near a joint, functional problems, deformity, pathological fracture

locations: distal femur, knee, proximal humerus

XR: bony spur from surface of cortex away from joint

75
Q

Osteoid osteoma

A

pathology: small benign bone tumour

clinical: 5-20yrs, M>F, proximal femur/tibia, increasing/unremitting pain worse at night, limp, atrophy

XR: round/oval lucency in the metaphysis/diaphysis surrounded by sclerotic bone

76
Q

Osteosarcoma

A

incidence: most common bone cancer 10-16yrs

location: distal femur, metaphysis, vertebrae

etiology: associated rapid growth

  • pleomorphic spindle cell neoplasm with malignant osteoid bone

4 subtypes: osteoblastic, fibroblastic, chondroblastic, telangiectatic

associations: RB1, Li-Fraumeni, Rothmund-Thomson, Paget’s, fibrous dysplasia

clinical: pain, limp, swelling, soft tissue mass, joint effusion/warmth

  • assumed to be secondary to injury
  • 10% metastatic disease at presentation (lung, bone, LN)

diagnosis: histology (malignant cells produce osteoid), XR (sunburst appearance of secondary ossification in tissue), MRI

management: chemo (Cisplatin, Doxorubicin, MTX), resection

prognosis: 65-75% survival non metastatic

77
Q

Pilocytic astrocytoma

low grade glioma

(infratentorial)

A

incidence: MOST common, 20% paed BT, age 5-14yrs

location: cerebellum, 3rd ventricle

features: low grade, slow growing, well circumscribed, cystic

histology: tight fibrillary bundles around spongy areas

radiology: contrast enhancing nodule with cystic mass

clinical: torticollis, ataxia/poor coordination, visual sx, N/V, headache, FTT, increased ICP

treatment: resection, +/- chemo/RTX

prognosis: total resection 87%, partial 50-95%

78
Q

Pineoblastoma (PNET)

(supratentorial)

A

incidence: 1-12yrs, 3% Paed BT

location: pineal gland

features: highly malignant, large, lobulated, poorly demarcated

associations: RB1

clinical: hydrocephalus, headache, lethargy, neuroocular sx

treatment: resection, radiation, chemotherapy

79
Q

Pituitary adenoma

A

anterior: TSH, ACTH, FSH, LH, GH, prolactin

posterior: vasopressin/oxytocin

clinical: depends on adenoma and hormones prosuced

  • most common hormones: GH/prolactin
  • visual: bitemporal hemianopia, raised ICP
80
Q

Post HSCT infections

A

etiology: no neutrophils from donor and no/function of T cells remains low for several months

risks: low cell counts, immunosuppressants, GVHD

organisms:

- invasive fungal infections: aspergillosis, candida

- viral: CMV most common (1-4 months post)

- EBV lymphoproliferative disease: deficiency of T cells to attack EBV

treatment: IVAB

81
Q

Post transplant CMV infection

A

pathogenesis: common post renal tx due to immpaired T cell immunity

onset:

primary (sero +ve organ): 4-12 weeks post transplant

reactivation by immunosuppresants: more common, usuallly asymptomatic

clinical:

  • early: fever, decreased WCC, rash, joint pain, high ALT
  • GI: oesophagitis, gastritis/ulcers, gastroenteritis, pyloric stenosis, hepatits, pancreatitis, cholecytsitis, colitis
  • eye: CMV retinitis
  • late: impaired function of transplanted organ/fungal infection

prognosis: tissue damage may lead to renal rejection

  • 90% asymptomatic, 10% lead to death
82
Q

Post transplant lymphoproliferative disease

A

etiology: rare, malignant condition, associated CMV/EBV

clinical: fever, LN, GIT (abdo mass, pain, bleeding, obstruction, perforation, ascites), seizures, CNS abnormalities, mediastinal mass

83
Q

Posterior fossa syndrome

A

etiology: injury to the cerebellar vermis/dentate nuclei

clinical: impaired language production, emotional lability, inattention, difficulty initiating movement

onset: 1-2 days post op and resolve over weeks to months

84
Q

Pre B ALL

A

NCI standard risk (65%) cure rate 90%

  • age 1-9yrs AND WCC<50
  • favorable cytogenics: diploidy, hyperdiploidy (50-67 chromosomes), trisomies 4, 10, 17, TEL AMI t(12:21)

NCI high risk (25%) cure rate 75%

  • age <1yr OR** >10yrs **OR WCC >50
  • adverse cytogenies: t(9:22) Philadelphia with ABL-BCR fusion protein, t(4:11), t(1:19), iAMP21 amplification, MLL gene rearrangement, iKZF1 gene deletion, hypodiploidy of blasts (<45 chromosomes)
85
Q

Radiation Recall

A

definition: inflammation of skin in area of previous radiation

agents: dactinomycin, anthracyclines

pathogenesis: epidermal dysplasia, necrotic keratinocytes, fibrosis, vasodilation

management: dose reduction, CS

86
Q

Retinoblastoma

A

incidence: most common intraocular tumour childhood, <2yrs

etiology: sporadic and heritable

inherited: germ line mutation familial or sporadic

  • familial: RB1 gene mutational inactivation of both alleles: 1st mutation at birth then 2nd mutation in development causing multifocal/bilateral tumours, 90% penetrance
  • sporadic: both allele mutations arise sporadically in a single somatic cell of the retina
  • increased risk with family history/13q deletion

clinical: destruction of the eye and globe, metastases after 6 months, death within yrs

  • leukocoria, strabismus, decreased VA, ocular inflammation, orbital inflammation, hyphema, pupil irregulalities

diagnosis: opthalmoscopic examination, CT, MRI

management: enucleation, radiotherapy, radioactive plaques, cryotherapy, laser, photoablation, chemotherapy

prognosis: 93% survival

87
Q

Secondary malignancies

AML

A

prevalence: most secondaries are AML

clinical: pancytopaenia with BM infiltrate

bone marrow: large cells (myeloid), heterogenous, more cytoplasm, 2-5 nucleoli

agents:

  • alkylating agents (5yr latency)
  • topoisomerase inhibitors: latency 2 yrs, 1% risk, 70% mortality
88
Q

Secondary malignancy

A

etiology: secondary to chemotherapy/radiation used

risk factors:

chemo: etoposide, cyclophosphamide, carboplatin

cancers: HL (16% within 30 yrs dx), soft tissue sarcomas, breast cancer, thyroid cancer, AML, ALL (3% risk, more with cranial RTX), medulloblastoma (4% gliomas/astrocytomas), RB (40% risk at 50yrs)

89
Q

Soft tissue sarcoma

A

3% paediatric cancers, M>F 2:1, mean age 12 yrs

liposarcoma: arise precursors adipocytes, extremities/retroperitoneum, 3 types (well-diff, myxoid/round cell, pleomorphic liposarcomas)

leiomyosarcoma: SM differentiation, retroperitoneum/vein/uterus, cutaneous more indolent

synovial sarcoma: cell of unknown origin, resembles synovial cell

malignant peripheral nerve sheath tumour: ectodermal origina, 50% in NG1

angiosarcoma: uncommon, subcutaneous tissues head/neck/breast, assoc therapeutic radiation post 8-10yrs

solitary fibrous tumour: pleura/dura/pelvis, slow growing

treatment for ALL: surgery

90
Q

Spinal cord compression

(cancer)

A

incidence: leukaemia, lymphoma, NB, rhabdomyosarcoma, ewing’s sarcoma

management: URGENT imaging/histology, surgical decompression, dexamethasone, chemotherapy

91
Q

Syndromes associated acute leukamaemias

A
  • T21
  • NF-1
  • Bloom syndrome
  • Ataxia telangiectasia
92
Q

AML

A

11% paediatric leukaemias

incidence: adolescence, adults>kids

risk factors: T21, Bloom, Fanconi’s, Kostmann, Schwachmann-Diamond, Diamond-Blackfan, Li-Fraumeni, NF-1

diagnosis: >20% blasts in BM

  • classified via FAB: M0-M7

clinical as for ALL PLUS:

  • subcutaneous nodules, leukaemia cutis, blueberry muffin nodules, gingival infiltration, DIC, extramedullary disease (<1%), eye white plaques

prognosis:

  • age/WCC not strong indicator
  • good outcome if low MRD 2nd timepoint

cytogenic/molecular more prognostic

  • low risk: t(8:11), inversion 16, t(5:17)
  • intermediate risk: MLL rearrangement
  • high risk: MLL rearrangement, monosomy 7, abnormal 11q23, 8:16

treatment:

chemo: high risk relapse (56% survival)

  • used low risk group

BMT: high mortality, infertility, cardiac cx (63% survival)

  • used intermediate/high risk or relapse
93
Q

Teratoma

A

pathology: GCT can differentiate into any somatic cell type

  • benign but comtain malignant elements

location:

  • infants: sacrococcygeal, intracranial
  • adults: anterior mediastinum, retroperitoneum, suprasellar

types:

mature cystic teratoma (dermoid cyst) >95%

  • fully differentiated tissue from somatic cells (ectodermal, endodermal, mesodermal) with hair/sking/GIT

immature: partially undifferentiated/embryonal tissue

malignant: 15-30% malignant components, increased ALP/bHCG

clinical:

  • in utero: caudal mass in fetus
  • infancy: asymptomatic or obstruction bladder/rectum

treatment: resection, chemo

94
Q

Testicular relapse

A

incidence: 10% all boys ALL

clinical: painless swelling of one or both testicles

risk factors: T cell ALL highest rates

treatment: chemotherapy and local RTX

95
Q

Thymoma

A

definition: benign tumour arising from the thymus

incidence: adults 40-60yrs

associations: MG and other paraneoplastic sx

96
Q

Trisomy 21

acute leukaemia and transient myeloproliferative disorder

A

incidence: 20X more common than general population

type: ALL>AML

prognosis:

  • ALL: poorer outcome, very sensitive to methotrexate
  • AML: better outcome >80% long term survival

neonatal conditions: 10% of neonates transient leukmaemia, myeloproliferative disorder

  • increased WCC, blasts, anaemia, thrombocytopaenia, hepatosplenomegally
  • may require transfusions
  • 20-30% develop AML by 3 yrs
97
Q

Tuberous sclerosis

A

genetics: 9q34 TSC1, 16q13

types:

subependymal giant cell tumours: benign, slow, periventricular

cortical glioneural hamartomas: enlarged, atypical, disorganised neuronal/glial elemetics with astrocytosis

  • present in 90% TSC

diagnosis: MRI

98
Q

Tufted angioma

A

definition: rare vascular tumour

pathology: benign, tufts of capillaries within the dermis

clinical: infiltrated, firm, dusky red to violet plaques/nodules with ill-defined border, 1-10cm

  • usually over arms/legs/trunk

management: excision, laser

prognosis: can regress but never completely resolve

99
Q

Tumour Lysis Syndrome

A

incidence: starts post chemotherapy within 24 hours and lasts 4-5 days

risk factors: high proliferation, large tumour burden, high sensitivity to chemotherapy

  • common in lymphoma (Burkitt’s), ALL, AML
  • rare in solid tumours

predisposing factors: dehydration, oliguria, nephrotoxins

pathology: breakdown of cells producing

  • high levels phosphate (conc 4x normal in blast cells)
  • uric acid (from DNA break down)
  • potassium (from IC contents)

PLUS hypocalcaemia (bound to PO4), lactic acidosis, azotemia (increased protein metabolism with increased urea)

clinical: N/V/D, anorexia, lethargy, haematuria, CHF, cardiac dysrhythmias, seizures, cramps, tetany, syncope, death

diagnosis: 2 of more abnormal serum levels within 3-7 days chemo OR 1 serum marker and 1 clinical feature

management: prevention

  • low risk: allopurinol, NaBicarb
  • high risk: fluids, uric acid oxidase, NO NaBicarb

treatment: as above + electrolyte control, cardiac monitoring, dialysis

monitoring: urine output, urine urate/xanthine/hypothanthine/CaPO4 crystals, Ca/Mg/Ph 4-8hrly

100
Q

Turcot syndrome

A

genetics: FAP (APC gene)

definition: associated FAP/HNPCC with brain tumours

associations:

FAP: medulloblastoma

HNPCC: gliomas

101
Q

Vincristine

Vinblastine

A

class: vinca alkaloids

mechanism: mitotic arrest via spindle fibre inhibition

use: ALL, lymphoma, Wilm’s, Ewing’s, rhado, NB, brain tumours, histiocytosis

side effects: abdo pain, constipation/ileus, jaw pain, loss of ankle jerk, SIADH

102
Q

Von Hippel Lindau disease

A

genetics: AD, assoc mutation tumour supressor gene VHL chromosome 3

etiology: increased erythropoeitin/grow factors causing syndrome with benign and malignant tumours

clinical:

onset of tumour at childhood/adolescence:

  • cerebellar haemangioblastoma (assoc retinal angiomata)
  • clear cell RCC
  • pheochromocytoma
  • endolymphatic sac tumour of middle ear
  • serous cystadenomas
  • neuroendocrine tumours of the pandreas
  • papillary cystadenomas of the epididymis/broad ligament

management: early diagnosis/treatment, genetic counselling

103
Q

Wilm’s tumour

A

definition: neuroblastoma embryonal malignancy of kidney

incidence: 6% paed malignancies, F>M, 2-5yrs, bilateral 7%

etiology: sporadic, famlial 2% (WT1 (WAGR, Deny’sDrash), WT2, WTX (30%), FWT1, FWT2, Beckwith-Wiedermann (10% chance), Periman sx, NF1, Sotos sx

associations: isolated hemihypertrophy, aniridia, GU abnormalities

clinical: incidental finding, abdominal mass, abdo pain, haematuria, HTN (25%), anaemia, coagulopathy

diagnosis: CT/MRI (claw sign: focal necrosis/haemorrhage/hydronephrosis), PET

treatment: nephrectomy, chemotherapy (doxorubicin/cyclophosphamide/etoposide/carboplatin), post op radiotherapy (>stage 2)

prognosis: survival 90%

104
Q

Non-Hodgkins Lymphoma

A

MOST burkitts: highly aggressive B cell malignancy

three forms:

  • endemic: Africa
  • sporadic: developed countries
  • immunodeficiency related

clinical: rapidly growing tumour mass, TLS, LN

genetics: cMYC translocations

  • t(8:14), t(2:8), t(8:22)
105
Q

Risk Statification

A

FRACP Exam (23 decks)

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