Oncology Flashcards

Question

Coagulopathy | (cancer)

Answer 1

**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

Answer 2

**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

Answer 3

**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%

Answer 4

**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

Answer 5

**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

Answer 6

**class:** antimetabolite **mechanism:** pyrimidine analogue **use:** ALL, AML, lymphoma **side effects:** similar to MTX, conjunctivitis, neurotoxicity

Answer 7

**class:** antitumour antibiotic **mechanism:** binds DNA inhibiting transcription **use:** Wilm's, rhabdo, Ewing's **side effects:** dermatitis, diarrhoea, myelosuppression, tissue necrosis on extravasation

Answer 8

**associated agents**: cisplatin, doxorubicin + cyclophosphamide **treatment:** corticosteroids, ondansetron, precipitant (NK1R antag)

Answer 9

**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

Answer 10

**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%

Answer 11

**mechanism:** topoisomerase inhibitor **use:** ALL, lymphoma, germ cell, sarcoma allergic reaction, constipation/diarrhoea, mycositis, secondary leukaemia

Answer 12

**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

Answer 13

**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

Answer 14

**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%

Answer 15

**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

Answer 16

**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

Answer 17

**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

Answer 18

**definition:** proportion of cells dividing at any one time **tumours:** have high GF (1-10%)

Answer 19

**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%

Answer 20

**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

Answer 21

**incidence:** adolescence **etiology:** secondary to HepB/HepC infection **associations:** tyrosinaemia, galactosaemia, GSD, alpha-1 antitrypsin deficiency, biliary cirrhosis **treatment:** partial resection, chemotherapy

Answer 22

**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

Answer 23

**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

Answer 24

**incidence:** leukaemia/lymphoma (osteolytic), alveolar rhabdomyosarcoma (osteolytic), Non-Wilm's renal tumour **clinical:** confusion → dehydration → renal failure **management:** hyperhydration, frusemide, bisphosphonates

Answer 25

**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

Answer 26

**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

Answer 27

**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

Answer 28

**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

Answer 29

**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

Answer 30

**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

Answer 31

**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

Answer 32

**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

Answer 33

Prednisone Asparaginase Bleomycin Vincristine

Answer 34

**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

Answer 35

_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%**

Answer 36

**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

Answer 37

**ALL:** - hyperdiploidy, t(12:21): good - hypodiploidy, t(BCR ABL): bad AML: - \>3 abnormalieis, t(9:11, t(9:22): bad

Answer 38

**SBLA cancer syndrome** - **_S_**arcoma - **_B_**reast - **_L_**eukaemia - **_A_**drenal 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%

Answer 39

**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

Answer 40

**etiology:** vena caval/bronchotracheal tree compression **incidence:** T cell ALL **clinical:** facial oedema, dyspnoea, wheeze, orthopnoea - must exclude pericardial effusion

Answer 41

**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%

Answer 42

**class:** antimetabolite **mechanism:** folic acid antagonist (inhibits DHFR) **use:** ALL, lymphoma, medulloblastoma, osteosarcoma **side effects:** hepatotoxicity, infertility, mucositis, myelosuppression

Answer 43

**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

Answer 44

**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

Answer 45

**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

Answer 46

**genetics:** AD 22q12, NF2 gene **types:** _bilateral vestibular schwannomas_ _intracranial meningiomas_ _spinal tumours_

Answer 47

**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

Answer 48

**non-phase dependent drugs:** - inactivate DNA: procarbazine - alkylating agents: cyclophosphamide, ifosfamide - platinum analogues: cisplatin - anthracyclines

Answer 49

**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

Answer 50

**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

Answer 51

**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

Answer 52

**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

Answer 53

**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%

Answer 54

**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

Answer 55

**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

Answer 56

**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

Answer 57

**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

Answer 58

**etiology**: rare, malignant condition, associated CMV/EBV **clinical:** fever, LN, GIT (abdo mass, pain, bleeding, obstruction, perforation, ascites), seizures, CNS abnormalities, mediastinal mass

Answer 59

**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

Answer 60

**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)

Answer 61

**definition:** inflammation of skin in area of previous radiation **agents:** dactinomycin, anthracyclines **pathogenesis:** epidermal dysplasia, necrotic keratinocytes, fibrosis, vasodilation **management:** dose reduction, CS

Answer 62

**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

Answer 63

**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

Answer 64

**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)

Answer 65

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

Answer 66

**incidence:** leukaemia, lymphoma, NB, rhabdomyosarcoma, ewing's sarcoma **management:** URGENT imaging/histology, surgical decompression, dexamethasone, chemotherapy

Answer 67

- T21 - NF-1 - Bloom syndrome - Ataxia telangiectasia

Answer 68

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

Answer 69

**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

Answer 70

**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

Answer 71

**definition:** benign tumour arising from the thymus **incidence:** adults 40-60yrs **associations:** MG and other paraneoplastic sx

Answer 72

**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

Answer 73

**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

Answer 74

**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

Answer 75

**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

Answer 76

genetics: FAP (APC gene) **definition:** associated FAP/HNPCC with brain tumours **associations:** _FAP:_ medulloblastoma _HNPCC:_ gliomas

Answer 77

**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

Answer 78

**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

Answer 79

**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%

Answer 80

**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)