Pediatric Oncology 2019 Flashcards

Question

AML What is the prognosis of AML? What is the remission rate from chemo alone? What is the remission rate after BMT? Why do you BMT these guys and not ALL?

Answer 1

1. Remission in 80% of patients from chemo alone 2. matched-sibling BMT after remission = 60-70% long-term survival; 3. graft-versus-leukemia effect from allogeneic transplantation (not seen in ALL)

Answer 2

Reactive lesion of bone Xray = cavernous spaces filled with blood and solid aggregates of tissue, mostly femur, tibia & spine Biopsy necessary to confirm diagnosis; treated with excision; recurrence rate 20-30%

Answer 3

**Extra S/E of doxorubicin** Hair loss, Hand/foot syndrome, u Skin discolouration

Answer 4

1. Definitive = divert CSF, or remove block 2. Emergent = dexamethasone to reduce oedema - Can “hold” until gets to a neurosurgeon

Answer 5

Common with aggressive tumours Tumour may invoke oedema Blockage of drainage channels – acute hydrocephalus -Severe hydrocephalus gives cerebral oedema too

Answer 6

**Pathology** 1. Classic 2. Large cell/anaplastic 3. Nodular/desmoplastic **Molecular** Group 1 – WNT Group 2 – SHH Group 3 Group 4

Answer 7

**Medulloblastoma** **Presents:** 1. Headache, Vomiting, Ataxia 2. Cranial nerve palsies & Developmental regression

Answer 8

**Emergency!!** 1. Ophthalmology, 2. neuroimaging (head MRI), 3. neuroendocrine evaluation, 4. lumbar puncture

Answer 9

**Increased ICP** – lethargy, irritability, macrocephaly, headache, papilledema, vomiting **Supratentorial/cortical** – subtle personality/speech changes, motor weakness, sensory changes, seizures, reflex abnormalities, infants can present with early handedness(\<1yr is pathologic) **Infratentorial** – abnormal gait, torticollis, blurred vision, diplopia, nystagmus

Answer 10

**Brainstem** – gaze palsy, multiple cranial nerve palsies, UMN defects (hemiparesis, hyperreflexia, clonus) **Optic pathway** – decreased visual acuity, Marcus Gunn pupil (RAPD), visual field defect **Suprasellar** – neuroendocrine deficits, e.g. DI, galactorrhea, abn puberty, hypothyroidism

Answer 11

**Diencephalic syndrome:** FTT, emaciation, increased appetite, euphoria **Pineal region**: Parinaud syndrome: paresis of upward gaze, nystagmus, eyelid retraction **Spinal cord:** long nerve tract motor and/or sensory deficits, bowel/bladder deficits, back pain

Answer 12

astrocytoma (Juvenile pilocytic and Diffuse), PNET (medulloblastoma), Ependymoma, Craniopharyngioma

Answer 13

**1. Gene alteration disrupts normal genetic repair:** Ataxia telangiectasia = lymphoma Bloom syndrome = leukemia; Xeroderma pigmentosa = skin cancer; **2. Dysfunctional cellular growth:** Beckwith-Weidemann = Wilms tumor, hepatoblastoma, rhabdo, adrenal tumor; MEN = Hepatic tumors; Neurofibromatosis = optic glioma, CNS tumors & rhabdomayosarcoma **3. Inactivation of tumor suppressor gene:** Familial retinoblastoma & Li Fraumeni = osteosarcoma

Answer 14

- May have a role in certain malignancies such as CML, AML, ALL, gliomas, and breast cancer. - These tumor-initiating cells have self renewal and proliferative properties similar to nonmalignant stem cells.”

Answer 15

1. **Viruses** **EBV –** Burkitt’s , Hodgkin + T-cell lymphoma **HBV –** Hepatocellular carcinoma **HCV –** Hepatocellular carcinoma, splenic lymphoma **HPV –** Cervical cancer **HHV-8 –** Kaposi sarcoma 2. **Genomic imprinting** (selective inactivation of ½ gene alleles) à Wilms tumor – loss of normal inactivation of maternal gene 3. **Telomerase** (enzyme that adds telomeres on xsomes = “immortal” cell lines

Answer 16

1) Mutation that converts **Proto-oncogenes into oncogenes.** These mutations can be: a. _Amplification_, e.g. N-MYC in neuroblastoma b. _Point mutation_, e.g. 10q RET in MEN2 c. _Translocation_, e.g. Philadalphia xsome t(9:22) BCR/ABL in ALL 2) Mutation that **disables Tumor suppressor genes**

Answer 17

Cancer is a complex of diseases arising from alterations that can occur in a wide variety of genes. Alterations in the following normal cellular processes can result in a malignant phenotype: 1. signal transduction, 2. cell cycle control, DNA repair, 3. cellular growth and differentiation, 4. translational regulation, senescence and apoptosis (programmed cell death)

Answer 18

MLH1, MSH2, MSH6, PMS2 non-polyposis Colorectal carcinoma, endometrial, gliomas, ovary, ureteric

Answer 19

NF1 gene – complex mutations Benign nerve sheath tumours, gliomas, MPNST, plexiform neurofibroma

Answer 20

Germline p53 mutation Adrenocortical carcinoma, sarcomas, breast cancer, brain tumours

Answer 21

1. PTCH mutation – SHH pathway mutation \*GROUP 2\* 2. Naevoid basal cell carcinoma syndrome Basal cell carcinomas Medulloblastoma Odontogenic cysts Skeletal and cutaneous abnormalities

Answer 22

1. leukoencephalopathy after high-dose methotrexate therapy; 2. infertility in male patients treated with alkylating agents (e.g., cyclophosphamide); 3. myocardial damage caused by anthracyclines; 4. pulmonary fibrosis caused by bleomycin; 5. pancreatitis caused by asparaginase; 6. renal dysfunction due to ifosfamide, nitrosourea, or platinum agents; and 7. hearing loss from cisplatin

Answer 23

Mtx Alkylating agent Anthracycline Bleomycin Asparaginase Ifosfamide Cisplatin

Answer 24

Cisplatin: **Immediate side effects** Nausea/vomiting Acute renal tubular dysfunction + Hypomagnesaemia **Long-term side effects** Hearing loss Renal impairment with low GFR Electrolyte disturbances with low Mg, âK

Answer 25

1. 2-3% of all childhood leukemia; 2. 99% w Philadelphia chromosome t(9:22) BCR-ABL 3. 3-4 yrs of chronic phase (high WBC, low Hb, high Plt, splenomegaly) leading to a “blast crisis” phase 4. Treated with busulfan, hydorxyurea & interpheron-alpha; stem-cell transplant the only cure

Answer 26

20x increased frequency of acute leukemia; ALL\>AML except first 3 years of life prognosis \>80% long-term survival. ALL prognosis slightly worse than others, AML prognosis slightly better. More sensitive to methotrexate & other metabolites – higher risk of toxicity

Answer 27

1. Occurs in 10% of neonates with Down syndrome ; lasts up to 1 year 2. High WBC, blasts, anemia, thrombocytopenia, hepatosplenomegaly. 3. Management: usually supportive w transfusions 4. Chance of leukemia: 20-30% develop acute leukemia by 3 yrs old

Answer 28

Second decade, Highest risk during adolescent growth spurt Undifferentiated **bone sarcoma** that may arise from **soft tissue**

Answer 29

Other small round cell tumors – neuroblastoma, rhabdomyosarcoma, Non-Hodgkin lymphoma histiocytosis, osteomyelitis

Answer 30

Onion skin

Answer 31

**Chemotherapy** +/- surgery +/- radiotherapy

Answer 32

Pain, swelling, limitation of motion, localized tenderness Spinal cord compression if tumor is paraspinal or vertebral Often have systemic symptoms – fever, weight loss

Answer 33

Lungs, Lymph nodes, bone marrow

Answer 34

**Long** bones – diaphysis **Axial** skeleton & **chest** wall

Answer 35

1. Commonest oncological emergency 2. Commonest non-cancer cause of death 3. Reason for increased risk: breakdown” of infective protection mechanisms * Low neutrophils/phagocytes * Impaired mucosal barriers * Indwelling catheters * Impaired immune response

Answer 36

**Gram positive (most common)** Staph aureus & epidermidis, Strep viridans **Gram negative (can be life threatening)** Pseudomonas E coli Klebsiella **Fungal** Candida Cryptococcus

Answer 37

1. Patients are at particular risk if the ANC is \< 500 but \< 1000 also incur some risk 2. Lack of neutrophils leads to a loss of the inflammatory response and fever may be the only manifestation of infection and the need for empiric abx

Answer 38

Patients without an identifiable source and persistent fever after **\> 5 days** with progression of symptoms, the addition of an antifungal agent is generally warranted

Answer 39

Occurs in 40% of children \> 2yrs old; represent ossification defect, usually discovered incidentally Occasional pathologic fractures; xray = lucency, may be multilocular, long axis runs parallel to bone Spontaneous regression after skeletal maturity; excision if \>50% of bone diameter

Answer 40

1. Px with **HR K+ or HOCa+ -**cardiac monit & lytes q 4hrly - **K+ \> 7-7.5 o**r or widening of QRS complex requires immediate intervention.9,10 Standard therapies to lower the potassium **Rx for HR K+:** a. loop diuretics, insulin and glucose, inhaled bagonists, polystyrene sulfate, and calcium gluconate for symptomatic hyperkalemia or electrocardiographic changes. b. hemodialysis. 2. **Hyperphosphatemia** is treated with phosphate binders. 3. The presence of **secondary hypocalcemia** can be life-threatening and generally necessitates the use of ***hemodialysis***. **Asymptomatic hypocalcemia should not** be treated with calcium administration because of the risk of increasing calcium phosphate deposition in the rena

Answer 41

1. 2% of childhood leukemia cases occur \< 1 year 2. ALL:AML ratio 2:1 3. Poor prognosis – hyperleukocytosis, organomegaly, CNS disease, leukemia cutis (subcut nodules), diffuse pulmonary infiltration 4. Intense chemotherapy + stem cell transplant

Answer 42

-Urate oxidase oxidizes uric acid to the water-soluble and readily excreted metabolite **allantoin**. - Rasburicase (urate oxidase) reduces uric acid levels without increasing the levels of uric acid precursors, and therefore **does not risk xanthine nephropathy.**

Answer 43

1. 3rd most common type of childhood cancer; 2. 6% of childhood cancers; peak in adolescence; rare in children \< 10 years 3. Increased risk with immunodeficiency, - acquired (HIV) & - congenital (e.g. Ataxia-Telangiectasia)

Answer 44

1. Unknown 2. genetic (risk 100-fold for monozygotic twin) 3. infectious (EBV, CMV, HHV-6) factors 4. Post chemo and radiotherapy

Answer 45

1. Painless, non-tender, firm cervical **lymphadenopathy**; 2. **B symptoms** (fever, wt loss, night sweats) **3. Mediastinal adenopathy** common - - airway compromise, - SVC syndrome, - pleural/ pericardial effusion

Answer 46

Reed Sternberg cells (owls eye cell)

Answer 47

1. Chest x-ray 2. excisional lymph node biopsy -Reed-Sternberg cell– clonal B cells

Answer 48

**Types:** 1. nodular, 2. lymphocyte-rich, 3. mixed cellularity, 4. anaplastic large cell, 5. lymphocyte-depleted

Answer 49

**Anterior Mediastinum**: 1. Lymphoma, 2. Thymoma, 3. Thyroid **Middle Mediastinum** 1. Lymphdenopathy – 2. lymphoma, metastatic disease, 3. Germ cell tumour **Posterior Mediastinum** 1. Neuroblastoma, neurofibroma, 2. Ewings, 3. Rhabdomyosarcoma

Answer 50

**Unfavorable**: 1. tumor bulk, 2. stage at diagnosis, 3. B symptoms = 90% survival 4. Relapse \> 12 mos after therapy = 60-70% survival **Favorable** **prognostic factors** + early-stage disease = 95% survival

Answer 51

**Ann Arbor Staging - Lymphoma** **Stage 1** Single nodal group **Stage 2** 2 or more nodal groups on SAME side of diaphragm **Stage 3** 2 or more nodal groups on BOTH sides of diaphragm **Stage 4** Involvement of extra-lymphoid tissues **B- Symptoms** Present vs Absent

Answer 52

1. Combined **chemotherapy** & **radiotherapy** **Chemo eg:** **ABVD”** (A) Doxorubicin Bleomycin Vincristine Dacarbazine 2. Autologous **stem cell transplant** in those who never achieve remission or relapse after \< 12mos

Answer 53

**Epidemiology** - 60% of all lymphomas in children & adolescents; 10% of all malignancies - \>70% present with advanced disease. RFs include immune deficiency, viral infection, syndromes. - Represents 50% of all pediatric malignancies in equatorial Africa (endemic Burkitt’s lymphoma) - Usually in younger children **Etiology**: EBV present in 95% of cases of endemic (African) Burkitt’s lymphoma

Answer 54

1. Burkitt 40% (small nocleaved B cell), 2. Lymphoblastic 30% (TCell), 3. Diffuse large B/T-cell 20%, 4. Anaplastic large cell 10%

Answer 55

**Masses**: head & neck, intrathoracic, mediastinal, abdominal, cutaneous. Can spread to marrow. **Site specific findings:** SVC syndrome, airway compression, intussusception, spinal cord compression, TLS

Answer 56

90-95% survival for localized disease; 60-90% with advanced disease

Answer 57

**Systemic & intrathecal chemotherapy** **Surgery** for diagnostic biopsy; rarely for debulking large masses

Answer 58

- Get baseline analyte values prior to Rx: uric acid, electrolytes, LDH, and creatinine, **HIGH RISK:** every 4 to 6 hours **INTERMEDIATE RISK:** every 6-8 hours **LOW RISK:** every 24 hours N.B. 1. uric acid levels may remain normal in patients with an emerging TLS ON ALLOPURINOL 2. For **rasburicase**, laboratories must develop protocols to collect uric acid samples in prechilled tubes that are placed immediately on ice and kept on ice throughout rapid transport to the laboratory for immediate run on the instrument to prevent rapid in vitro uric acid breakdown, and a spuriously low assay result.

Answer 59

**Epidemiology** 3rd most common pediatric cancer ~8%; 90% diagnosed by 3yrs old Most common neoplasm in infants - 1/3 of cases

Answer 60

1. Abdo and chest CT 2. BMA w/ biopsy (bilateral!) 3. 95% have elevated urine HVA & VMA 4. Bone scan/MIBG scan 5. Mass biopsy

Answer 61

**Other small round cell tumors** – 1. rhabdomyosarcoma, 2. Ewing sarcoma, 3. Non-Hodgkin lymphoma

Answer 62

Can develop at any site of sympathetic nervous tissue, S&S reflect location & extent of tumor 1. Thoracic: dyspnea/horner syndrome 2. Abdominal: abdo pain, vomiting, palpable mass 3. Pelvic: constipation and urinary retention 4. Paraspinal: back pain, limp, leg weakness, hypotonia Where can neuroblastoma develop?

Answer 63

Funny **METS** to _bone marrow_ and _skin_: 1. Periorbital bruising (raccoon eyes), 2. blue berry muffin spots (skin nodules) **Paraneoplastic syndrome:** 1. Opsoclonus-myoclonus, 2. intractable watery diarrhea, 3. hypertension, 4. sweating

Answer 64

1. Embryonal cancer of the peripheral sympathetic nervous system; variable neural differentiation from undifferentiated (neuroblastoma) to mature ganglion (ganglioneuroma) 2. Amplification of _n-MYC gene,_ _11q and 1p deletions_ associated with poor outcome

Answer 65

**Factors include** 1. amount of stroma, 2. degree of differentiation, 3. presence of enlarged nucleoli, 4. mitosis index **Depends on stage:** stage 1 \> 90%; stage 2 80%, stage 3 50%; stage 4 30%; stage 4S (\<1yr) \>80%

Answer 66

Surgery, chemotherapy, radiotherapy 4S = special! 1. \<1year of age, 2. localized primary tumour w/ spreading _limited_ to **liver, skin, bone marrow.** Very good prognosis!

Answer 67

Locally destructive, progressively growing bone lesion, prefers vertebrae Insidious onset of dull, aching pain; may have neurological deficits at presentation **Biopsy** necessary to confirm diagnosis; treated with excision

Answer 68

Arise in metaphysis of long bone, esp distal femur, proximal humerus, proximal tibia Bony, painful mass; Xray = stalks or broad-based projections from bone surface Malignant degeneration in 1% of adults, surgical resection if symptomatic

Answer 69

**Unremitting, gradually increasing pain** that is worse at night, relieved by analgesic **Most common sites** proximal femur & tibia; vertebral lesion = scoliosis or neurological deficits **Xray** = round or oval lucency surrounded by ring of sclerosis **Surgical resection;** some resolve spontaneously with skeletal maturity

Answer 70

Malignant spindle cell tumour. Second decade, Highest risk during adolescent growth spurt **Etiology**: hereditary retinoblastoma, Li-Fraumeni

Answer 71

1. **Radiology**: a. _Xray_ = **sunburst pattern** (ossification of soft tissue) & **Codman triangle** (cortical elevation) - **primary site** **b.** _MRI primary site & CT chest_ **u** _Bone scan_ 2. **Biopsy** of tumor & metastatic workup to **lung + bone marrow** 3. **Blood work up:** Normal CBC and chemistry, but may have elvated **LDH & ALP**

Answer 72

Lytic bone lesion ddx = histiocytosis, Ewing sarcoma, lymphoma, bone cyst

Answer 73

**Indicative of highly aggressive or advanced tumour** 1. Local invasion 2. Lymphoid spread -Locoregional, distant 3. Haematogenoous spread u Distant sites (e.g. lungs, brain, bone marrow) 4. CNS tumours & CSF dissemination

Answer 74

1. Pain, limp, swelling most common. May be attributed to sports injury or growing pains 2. Deep bone pain that causes nighttime awakenings; May also see decreased ROM, joint effusion, tenderness, warmth Normal CBC and chemistry, but may have elvated LDH & ALP Locations: long bone metaphysis – 50-80% are in proximal tibial / distal femur

Answer 75

75% survival in non-metastatic disease; 25% survival in metastatic (lung) disease

Answer 76

**1. Neo-adjuvant chemo (**Cisplatin, Doxorubicin, Methotrexate (high dose)..then 2. complete **surgical resection** (ESSENTIAL) (may need amputation) ....then more chemo Note: this tumour is more chemo resistant \*\*NOT radiosensitive

Answer 77

**Ends** of long bones (usually solitary) **Pelvis**, others

Answer 78

1. Growth delay (cranial or vertebral irradiation) 2. Endocrine (midbrain irradiation) 3. Pulmonary or cardiac insufficiency (chest) 4. Strictures and adhesions (GI) 5. Infertility (pelvic

Answer 79

1. Dermatitis of adjacent tissues 2. Mucositis anywhere from mouth to anus 3. Somnolence (Cranial irradiation) 4. Alopecia

Answer 80

Opthalmology, MRI to r/o intracranial tumor

Answer 81

**BILATERAL:** Rare, bilateralinvolvement highest if presents \<1yr old 25% bilateral/hereditary **UNILATERAL**: 60% unilateral/non-hereditary; 15% unilateral/hereditary;

Answer 82

Hereditary form due to 2-hit hypothesis: 1st inherited germ cell mutation then 2nd somatic mutation Increased risk in Li-Fraumeni

Answer 83

**Primary goal** = cure; **secondary goal** = preserving vision 1. **Enucleation** (eyeball resection), 2. **chemotherapy** + **focal therapy** (laser or cryo), 3. **radiation** 4. All 1st-degree relatives need to be **screened**

Answer 84

95% cure rate; poor prognosis if metastatic

Answer 85

Leukocoria– white papillary reflex Strabismus; with advanced disease = orbital inflammation, hyphema, pupil irregularity

Answer 86

Cataract, retinopathy of prematurity, visceral larva migrans, coloboma

Answer 87

1. If RB1 gene carrier – depending on the mutation 1**0-100% risk of retinoblastoma**, and 2. increased risk for other tumours (**osteosarcoma, glioma, glioblastom multiforme, melaninoma, lung, bladder**) and need to have optho exams _until 9 yo_

Answer 88

Requires high index of suspicion Biopsy required, cytogenetics to determine prognosis

Answer 89

1. Neo-adjuvant chemotherapy then 2. complete resection if possible, 3. followed by XRT & chemo

Answer 90

Other small round cell tumors – neuroblastoma, Ewing sarcoma, Non-Hodgkin lymphoma

Answer 91

1. retroperitoneal & other 35% 2. Head & neck 25%, 3. genitourinary tract 22%, 4. extremities 18%, **Mass** that causes displacement or obstruction of normal structures – symptoms are specific to site Orbit = proptosis, middle ear = chronic otitis media, larynx = hoarseness, bladder = hematuria

Answer 92

80-90% survival with complete resection; 70% with incomplete resection, 25% with disseminated

Answer 93

Arise from striated skeletal muscle

Answer 94

3.5% of childhood cancers, increased risk in neurofibromatosis

Answer 95

K+ is an ICF electrolyte regulated through renal excretion. TLS HR K+ ...**cardiac dysrhythmias** and **sudden death**. Ca+ stabilizes the cardiac membrane, Low Ca+ exacerbates HR Ca+- induced cardiotoxicity and cardiac dysrhythmias. HR Ca+ \> 7.0 mEq/L(hyperkalemic emergency) and can be potentiated by **ongoing TLS**, **Low Ca+,** and/or **renal failure**

Answer 96

1. Maintaining a **high urine output with adequate hydration** - most important aspect of TLS prevention and management, because this improves renal perfusion and glomerular filtration, and minimizes acidosis, all of which serve to prevent the precipitation of uric acid and calcium phosphate crystals in the renal tubules. 2. **intermediate-to-high risk** & for established TLS: hyperhydration with NS to maintain equal fluid balance and a high urine output. 3. **K+, PO4, and Ca+** should NOT be added to the hydration solution. 4. **Monitored Volume status** to prevent volume overload, particularly in patients with renal failure or cardiac dysfunction, and diuretics may be necessary to maintain urine output.

Answer 97

Preferred prophylactic agent for patients at high risk for TLS and the treatment of choice for established TLS. **Benefits:** 1. rapidly reduces uric acid levels and breaks down deposits of uric 2. Reduces PO4 + serum creat levels 3. **does not** require renal dosing; however, there is a risk for **Problems:** 1. severe hypersensitivity reactions (anaphylaxis) particularly with repeat dosing 2. contraindicated in pregnant/lactating F & G6PD Def'y , (severe hemolytic anemia) - Screen for G6PD

Answer 98

AKI - independent predictor of short + long-term TLS mortality **Mecnms:** **Crystal-dependent** and **crystal-independent mechanisms.** 1. **Crystal-dependent mechanisms** - obstructive uropathy. - Decreased urinary outputcan result in volume overload and cardiac failure, which can exacerbate crystal precipitation. Urine alkalinization to \> pH 6.5 not in use now **2. Crystal-independent mechanisms** -loss of autoregulation, renal vasoconstriction, and local inflammation -Tumor lysis–induced hypercytokinemia can result in hypotension, systemic inflammation, and multiorgan failure

Answer 99

1. The risk appears to be cumulative, increasing by about 0.5% per year, resulting in approximately a 12% incidence at 25 yr after treatment. 2. Patients should be examined annually, with particular attention to second malignancies.”

Answer 100

1. Safe storage and labelling 2. Appropriate handling and protective equipment 3. Careful identification of patient and dosage (double checked) AND ROUTE 4. Appropriate administration

Answer 101

Allopurinol blocks uric acid synthesis leading to accumulation of the uric acid precursors **xanthine** and **hypoxanthine**, of which hypoxanthine is more soluble and more easily excreted than uric acid.

Answer 102

_Hyperuricemia_ with _hyperphosphatemia_, can lead to acute kidney injury. **Mecanism:** **-**Purine containing nucleic acids released into the serum are catabolized to **uric acid** and, when present in excess, surpass the renal tubular capacity for uric acid excretion. -Uric acid is poorly soluble in water, and the solubility is markedly reduced in the physiologically acidic environment of the distal tubules and collecting system. **Thus, uric acid crystals readily precipitate** when concentrated in the renal circulation, leading to renal tubule obstruction and obstructive uropathy, with compromised glomerular filtration and reduced urine output.

Answer 103

**Timing:** 1. Within 12 to 72 hr \> initiation of cytotoxic therapy - Can occur 72 hrs 2. Can occur before Rx **Symptoms include:** 1. nausea, vomiting, diarrhea, anorexia, 2. weakness, lethargy and syncope. 3. hematuria, 4. muscle cramps, tetany, **Cplxns:** 1. Cardiac dysrhythmias, 2. seizures, 3. sudden death

Answer 104

**Deftn:** Acute, life-threatening disease among children that is associated with the initiation of cytoreductive therapy in the treatment of malignancy. **Characteristic findings:** _Raised:_ uric acid, phosphate, K+, urea, creatinine _Low_**:** Ca+, **Cplxns:** cardiac arrhythmia, seizures, renal failure, and sudden death.

Answer 105

1. Alpha-fetoprotein: Hepatoblastoma, germ cell tumours 2. Beta-HCG: Germ cell tumours 3. Urine catecholamines: Neuroblastoma, Phaeochromocytoma

Answer 106

Usually occur b/w 3-12 yrs, fluid-filled lesion, mostly asymptomatic until pathologic fracture Xray = solitary, centrally located lucency, proximal humerus or femur Treatment with aspiration and injection of bone marrow or steroids

Answer 107

Special side effects: (vincristine, vinblastine, vindesine) Vincristine Vinca alkaloid Severely neurotoxic Severe vesicant – extravasation damage Intrathecal administration ( WILL DIE....) Severe demyelination Severe encephalopathy Severe pain..extravasation burn.. Death, usually

Answer 108

1. **Antimetabolites:** MTX, 6 MP, Ara C 2. **Alkylating agents:** cyclophosphamide, ifosphamide, cisplatin, carboplatin 3. **Antibiotics:** Anthracyclines (Doxorubicin, daunomycin), Dactinomycin, Bleomycin **4. Plant alkaloids;** Vincristine, vinblastine, etoposide VP 16 **5. Miscellaneous:** Prednisone, PAG Asparaginase Alkylating agents u e.g. u Anthracyclines u e.g. doxorubicin u e.g. Methotrexate u Antibiotics u e.g. bleomycin u Enzymes u e.g. asparaginase u Microtubule inhibitors u e.g. vincristine u Nucleoside analogues u e.g. 6-mercaptopurine u Platinum agents u e.g. u Small-molecule inhibitors u e.g. imatinib u Topoisomerase inhibitors u e.g. etoposide

Answer 109

1. refractory volume overload 2. **AKI:** oliguria or anuria, 3. persistent **HR K+, HR uric acid** despite above measures 3. hyperphosphatemia-induced **symptomatic hypocalcemia**, **and** a calcium phosphate product of greater than 70 mg2/dL N.B. Prophylactic hemodialysis in patients at risk for TLS may be appropriate in the setting of **preexisting renal disease** or **acute renal injury at presentation**, and further studies are needed

Answer 110

"Preferred prophylactic in low-to-intermediate risk patients" * *Limitations:** 1. As it it does not break down preexisting uric acid, **urate nephropathy** can develop in the 3 days it takes to have a therapeutic effect. SO is **NOT** the preferred agent in the presence of hyperuricemia. Furthermore, xanthine nephropathy must be considered in patients who develop TLS while receiving appropriate prophylactic therapy with allopurinol. 2. **allopurinol is excreted by the kidneys** and must be dose reduced or discontinued in patients with renal insufficiency. 3. **hypersensitivity** reactions have occurred. Asian populations have a higher frequency of the HLAallele, which is associated with severe adverse cutaneous reactions with allopurinol use. 4. because allopurinol **reduces purine degradation,** **chemotherapeutic agents, such as 6-mercaptopurine** **and azathioprine,** must be dose reduced by 50% to 70% when concurrently administered. N.B. Any patient who develops TLS while receiving allopurinol prophylaxis should be switched to rasburicase

Answer 111

- Tumor cells have higher (PO4) than their normal cells - In TLS renal tubular excretion of (PO4) becomes saturated. - excess serum phosphate - Excess (PO4) binds to Ca+-- **low (Ca)** + **Ca PO4 deposition** - - **Low (Ca) Sx:** - Arrythmias, seizures, Nephrocalcinosis -renal tubulular nephrolithiasis and **cause or further provoke an obstructive** **uropathy.**

Answer 112

**Epidemiology** 6% of pediatric cancers, usually between 2-5 years; unilateral 90% **Etiology**: Most sporadic; 1-2% AD; _increased risk in syndromes_ 1. Beckwith-Wiedemann 2. Denys-Drash – Glomerulopathy/Pseudohermaphrodism 3. Neurofibromatosis 4. Sotos 5. WAGR- (W-ilms, A-niridia, G- GU anomalies, R- MR,

Answer 113

Complex mixed embryonal tumor

Answer 114

1. **CT scan:** Diagnostic imaging to confirm _intrarenal origin_ & determine _local_ & m_etastatic spread_ ( claw sign) 2. **Biopsy** if diagnosis uncertain (Look at both kidneys!) - risk of biopsy!!

Answer 115

Nephrectomy, chemotherapy, radiotherapy

Answer 116

**1.** **Renal specific Sx**: a. Hematuria 25%, hypertension (renal ischemia), b. Mass effect: - **Abdominal** mass, generally discovered fortuitously while parent bathing child - r**apid abdominal enlargement** (hemorrhage into renal pelvis), 2. develop acquired **bleeding** diasthesis (VWD) 3. **polycythemia**

Answer 117

1. Depends on **DNA content;** 2. S**taging:** \>60% survival across all stages; \>90% survival if stages 1-3

Pediatric Oncology 2019 Flashcards

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