Childhood Cancers Flashcards

Question

What are the clinical features in the Chronic Phase of CML?

Answer 1

Chronic phase lasts for approximately 3 years if untreated. Hyperleukocytosis with weakness, fever, night sweats, bone pain, respiratory distress, priapism Left upper quadrant pain (splenomegaly) +/- hepatomegaly

Answer 2

Characterized by progressive splenomegaly, thrombocytopenia, and increased percentage of peripheral and bone marrow blasts(10-19%)

Answer 3

Bone marrow shows greater than 30% blasts and clinical picture is indistinguishable from acute leukemia. Two-thirds of blast crisis is myeloid Patients in blast crisis will die within a few months.

Answer 4

Used to be hematopoietic stem cell transplant. Now, hydroxyurea/busulfan is given for cytoreduction Then imatinib (Tyrosine kinase inhibitor - has reduced the yearly risk of CML progression. Works on BCR-ABL)

Answer 5

Lymphomas: Non-Hodgkin and Hodgkin's Lymphoma Non-Hodgkin's Lymphoma: Lymphoblastic, Small Non Cleaved Cell (SNCC) and Large Cell Lymphoblastic - B cell and T cell. For SNCC - Burkitt's and High Grade B cell. For Large Cell - Anaplastic large cell and diffuse B cell large cell lymphoma.

Answer 6

Non-Hodgkin's lymphoma

Answer 7

Malignant solid tumour of B or T lymphocytes

Answer 8

Between the ages of 4 to 9 and peak age at 5yrs. Rare below 2 yrs and above 16yrs.

Answer 9

Males are affected more than females 2:1 Prognosis is good with minimal therapy in early stages. Symptoms at presentation depends on location of the tumour

Answer 10

Neck/jaw mass, involving nasopharynx, sinuses, Intra-oral extension Painless or painful Proptosis Teeth displaced - Called Dental Anarchy Swallowing +/- breathing difficulty (because nasopharynx is affected)

Answer 11

Abdominal mass +/- ascites Abdominal pain Nausea and vomiting, constipation +/- bowel obstruction Urinary retention Neurological symptoms including paraplegia from pressure on spinal cord

Answer 12

Cough, Stridor, Breathlessness Symptoms relating to anterior mediastinal mass +/- pleural effusion +/- SVC obstruction Lymphadenopathy Hepatosplenomegaly

Answer 13

Age: 5-10 y (Endemic), 6-12 y(Sporadic Sex: M>F (Endemic), M>F (Sporadic) Disbn of Dx: Africa, Brazil, Turkey (Endemic). North America, Europe (Sporadic) Annual Incidence: 10 in 100,000 (Endemic), 0.2 in 100,000 (Sporadic) Tumor sites: Jaw, Abdomen, CNS, CSF (Endemic) and Abdomen, marrow, lymph nodes, ovaries (Sporadic) Histopathologic features -Both have Starry sky appearance Presence of EBV DNA in tumor cells - 95% (Endemic), 15% (Sporadic)

Answer 14

Rapidly growing tumour of the jaws or abdomen in a high risk group (by age or location) Doubling time of 24hrs A longer history makes the suspicion less A diagnosis can only be confirmed by biopsy and histology

Answer 15

If Burkitt's lymphoma is found at these sites prognosis is not good/ there's an increased risk of relapse. Testes, Breasts, Thyroid gland, Skin, Epidural space, Bone Pancreas

Answer 16

Blood tests FBC – Rule out leukaemia (Blood film comment). Hb, WBC and Platelet count. Renal and liver function tests ( Renal function deranged with NHL and tumor lysis syndrome) LDH – raised in NHL (Highly proliferative Ca. that releases LDH on breakdown) Uric acid – raised in high tumour burden HIV screening Radiology/imaging CXR : mediastinal mass (which poses a risk of SVC syndrome) Abdominal and/or neck USS CT scan (extent of mass) MRI – not critical Echo - 'cause of use of cardiotoxic drugs (look at ejection fraction before giving drugs)

Answer 17

Use least invasive method to obtain tissue Ideally a piece of solid tissue (or Needle biopsy) Pleural or ascitic fluid (check for malignant cells) Bone marrow aspirates + trephines - for diagnosis and staginng Lumbar Puncture – CNS status CNS +ve if blasts/lymphocytes in CSF or cranial nerve palsy or intracerebral mass or intraspinal mass Histopathology Scattered diffusely among the tumour cells are giant phagocytic macrophages Macrophages have retracted cytoplasm thereby creating empty spaces between the cell and adjacent tissue Characteristic starry-sky pattern - for Burkitt's

Answer 18

A A single extra-abdominal tumour site B Multiple extra-abdominal tumour sites AR Completely (>90) resected intra-abdominal tumour (seen intraop and resected) C Intra-abdominal tumour without involvement of other sites D Intra-abdominal and extra-abdominal tumour sites

Answer 19

I A single tumor (extranodal) or single anatomic area (nodal) with the exclusion of mediastinum or abdomen. II A single tumor (extranodal) with regional node involvement. Two or more nodal areas on the same side of the diaphragm. Two single (extranodal) tumors with or without regional node involvement on the same side of the diaphragm. A primary GI tract tumor, usually in the ileocecal area, with or without involvement of associated mesenteric nodes only.

Answer 20

III Two single tumors (extranodal) on opposite sides of the diaphragm. Two or more nodal areas above and below the diaphragm. All the primary intrathoracic tumors (mediastinal, pleural, thymic). All extensive primary intra-abdominal disease. All paraspinal or epidural tumors regardless of other tumors site(s). IV Any of the above with initial CNS or bone marrow involvement.

Answer 21

Cycle 1/Prephase – Oral medication with Cyclophosphamide, Prednisolone and Allopurinol Alternate cycles of Vincristine, Doxo, Cyclophosphamide, Prednisolone, IT with Cytarabine, Vincristine Cyclophosphamide, Prednisolone, IT Give every 3 weeks Minimum of 6 cycles and max of 10 cycles Give in small doses to prevent TLS. Hydrate patient

Answer 22

Tumour burden Bone marrow involvement CNS involvement Presentation above age 13yrs. Overall response rate to chemotherapy is about 90% Relapse shortly after remission (3months) has poor prognosis

Answer 23

Wilm's tumor/Nephroblastoma

Answer 24

At 1-5 years with peak incidence of 3-4 years Median age of presentation - 3 years 6 months in unilateral disease and 2 years 6 months in bilateral Common type - Sporadic (1% familial)

Answer 25

Palpable mass in abdomen - flank mass that doesn't cross the midline) Hypertension - from hyper reninemia Hematuria Weight loss Obstipation - difficulty defecating Urinary tract infection Diarrhea Previous trauma Other S & S-nausea abdo pain

Answer 26

Occur in 12-15% of cases WAGR syndrome – wilm’s tumour, aniridia, genitourinary malformations and mental retardation Denys-Drash Syndrome- wilm’s tumour, early renal failure with mesangial sclerosis and pseudohermaphroditism Beckwith-wiedemann syndrome-visceromegaly with hemihypertrophy, macroglossia, abdominal wall defects etc. Wilm’s, hepato, neuro, rhabdo and adrenocortical carcinoma.

Answer 27

Thrombocytosis usually present Bleeding diathesis due to presence of acquired von Willebrand disease – prolonged bleeding time, decreased FVIII levels Hypertension Erythropoietin increased, associated with males, older age and low clinical stage. Polycythemia

Answer 28

Headache. Dizziness or lightheadedness. Chest pain. Weakness. Bloody stools Numbness or tingling of the hands and feet. Skin bruising easily. Bleeding from places like the nose, mouth and gums. Bleeding in the stomach or intestinal tract Blood clots in arteries and veins, most often in the hands, feet, and brain Swollen lymph nodes

Answer 29

Locally – grow into the renal sinus or ureter Contiguous spread through renal vein into IVC and rarely R atrium Regional lymph node involvement Haematogenous spread Lungs(80%) Liver(15%) Rarely bone, bone marrow or brain

Answer 30

Stage I ... confined to one kidney Stage II ... through capsule Stage III ... residual tumour (after biopsy) Stage IV ... metastatic disease Stage V ... bilateral disease (you'll need to stage both kidney's separately)

Answer 31

History –Fhx of cancer, congenital defects p/e congenital abnormalities FBC Urinalysis and renal function LDH, uric acid, clotting screen (because of acquired von willebrand disease) Echo - if you want to give doxorubicin for mets disease Abdominal USG/CT/MRI (better delineation) Chest x-ray/CT scan (CT better for chest, can pick up small nodules)

Answer 32

Neoadjuvant/Adjuvant chemotherapy Surgery (primary or delayed) for all - In Ghana, Chemotherapy, Surgery, Radiotherapy if indicated, Chemotherapy Chemotherapy Stage I or II: Vincristine + Dactinomycin Stage III or IV: Add DOXORUBICIN RENAL BED RADIOTHERAPY for local Stage III PULMONARY RADIOTHERAPY for lung metastases

Answer 33

Nephroblastomatosis – Nephrogenic rests that are potential precursors of Wilms Tumour. Congenital mesoblastic nephroma (In children <3 months) Clear cell sarcoma of the kidney- 3-5yrs. Metastasize to the bone, lung, liver and brain Rhabdoid tumour of the kidney. (presents by 1 yr, Poor prognosis with mortality of 80%). Patients have hypercalcemia Renal cell carcinoma – mostly adolescents

Answer 34

Abnormally persistent clusters of embryonal cells, representing microscopic malformations (dysplasias) of the developing kidney.

Answer 35

It is a malignant tumor of the embryonic neural retina. Starts as an intraocular growth occurs, prior to invasion of structures within the globe or spread to metastatic sites.

Answer 36

Children <5 years of age

Answer 37

Most common intraocular malignant tumour Occurs 1 in 20,000 live births Annual incidence is 10-14 per million under 5yrs of age 11% of cancers in 1st year of life 3% of cancers diagnosed in children < 15yrs Average age at diagnosis is 18mths

Answer 38

Leukocoria. Strabismus - squint Proptosis Decreased visual acuity. Inflammatory changes in eye. Hyphema. Vitreous hemorrhage, resulting in a black pupil.

Answer 39

3 overlapping methods Laterality- unilateral (2yrs) or bilateral (1yr), Trilateral Focality - unifocal or multifocal Genetics- hereditary(40%) or non-hereditary(60%)

Answer 40

Well recognized syndrome that occurs in children under the age of 5 years Usually consists of bilateral hereditary retinoblastoma associated with an intracranial neuroblastic tumour of the pineal gland Occurs in about 5 – 15% of children with familial, multifocal or bilateral retinoblastoma

Answer 41

Eliciting red reflexes in the eye as part of the well child check ups Siblings of children with retinoblastoma should be screened by ophthalmology at regular intervals at least through age 3 yrs. Siblings of patients should be screened for mutations in the RB1 gene.

Answer 42

CT, or MRI scan of brain and orbit Due to concern about rupturing the tumor and causing both intraocular and extraocular spread, surgical biopsies are not performed for confirmation.

Answer 43

FBC BUE and Creatinine Lumbar puncture if there is radiographic or clinical suspicion of CNS disease (CSF Cytology) Bone marrow biopsy when there is abnormal blood counts Histopathologic evaluation if enucleation is performed (usually after chemotherapy to reduce size of tumor)

Answer 44

Single used or combined modality approach is not uncommon: Systemic chemotherapy External beam radiotherapy Local ophthalmic therapy(laser Ry or Cryotherapy) Intra-arterial chemotherapy, using an interventional neuro-radiology approach Enucleation.

Answer 45

OSTEOSARCOMA SOFT TISSUE SARCOMAS MELANOMA LEUKAEMIA LYMPHOMA BREAST CANCER

Answer 46

Malignant tumour of peripheral sympathetic nervous system that develops from immature nerve cells(neural crest)

Answer 47

Neuroblastoma Commonly arises in and around adrenal glands More common in boys than girls

Answer 48

Sympathetic chain Neck Thorax - posterior mediastinal neuroblastoma Retroperitoneum - of adrenal medulla origin//Paraspinal ganglion origin Pelvis Adrenal gland

Answer 49

Constitutional Symptoms: Anorexia, Weight loss, Malaise, fever Pain (most common), due to local spread &/or metastatic disease Abdominal lump Respiratory compromise: from mediastinal mass Esp in young infants with massive hepatomegaly Horner's syndrome(neck mass), spinal cord compression, Cytopenias (marrow involvement), Blueberry muffin sign (skin involvement). Bowel/bladder dysfunction (pelvic mass), opsoclonus-myoclonus syndrome (truncal ataxia and cerebellar encephalopathy) Proptosis/ecchymoses: Orbital mets

Answer 50

Pepper syndrome – Neuroblastoma of adrenal gland with metastases in the liver Hutchinson syndrome – Limping and irritability due to skeletal metastases Horner syndrome – Miosis, ptosis , anhidosis

Answer 51

FBC, LDH, LFT, RFT, Uric acid 24hr Urine VMA and HVA - specific investigation Xray – calcified abdominal or posterior mediastinal mass USG CT or MRI Bone Scan MIBG- meta-iodobenzylguanidine scan Bone marrow aspirate Biopsy

Answer 52

Chemotherapy – COJEC (Cisplatin, Oncovin/Vincristine, Carboplatinum, Etoposide, Cyclophosphamide) Surgery Radiation High dose chemotherapy/radiation/stem cell transplant Cis-retinoic acid - to consolidate/maintain KEDO - In pallative setting Cyclophosphamide, Vincristine, Doxorubicin

Answer 53

50-60% of paediatric brain tumours occur in the posterior fossa (below the tentorium) Tumours with similar histology can arise in different areas of the CNS so it’s most useful to consider them according to cell of origin. Untreated brain tumours are fatal regardless of whether the histology is low grade (“benign” / “dysplastic”) or high grade (“malignant”)

Answer 54

Embryonal (primitive neuroectodermal tumours) Medulloblastomas / Supratentorial PNETS Pinealoblastomas (Atypical Teratoid / Rhabdoid Tumours) Glial Tumours Glioblastomas Astrocytomas Oligodendrogliomas Ependymomas Craniopharyngioma Germ Cell Tumour Pituitary Adenoma

Answer 55

Radiation therapy is the only known exogenous cause! Increased incidence with certain syndromes Neurofibromatosis 1: low grade gliomas (especially optic pathway) Tuberous Sclerosis: subependymal giant astrocytomas Sturge-Weber: meningeal and cortical angiomas Familial syndromes: Li Fraumeni … gliomas Gorlin / Turcot’s medulloblastomas

Answer 56

INFRATENTORIAL OR SUBTENTORIALLY Cerebellar signs (ataxia) Cranial nerve palsies (esp. bulbar) Hemiplegia Headaches (nausea and vomiting) - anything obstructing v4 SUPRATENTORIALLY Focal Neurology ex Hemispheric lesions Hemianopia and Hormonal dysfunction ex tumours occuring in the Suprasellar region

Answer 57

Hydrocephalus VP shunts were associated with multiple complications Less of a problem with the advent of the third ventriculostomy Steroids for relief of raised ICP and occasionally focal neurology Dexamethasone at minimum effective dose Bulbar Palsy May require NG tube feeding or occasionally justify a PEG Physio / Occupational Therapy / Neurodevelopmental rehab is crucial ADEQUATE ANALGESIA in the palliative care setting!

Answer 58

Surgery Shunting, Surgical excision - Gross total excision Radiotherapy Chemotherapy

Answer 59

Any physical or psychological outcome that develops or persists beyond 5 years from the diagnosis of cancer

Answer 60

*Chemotherapy, radiation therapy and surgery may all cause late effects *Some late effects of therapy identified during childhood or adolescence resolve without consequences, while other late effects become chronic and progress to become adult medical problems

Answer 61

Skeletal maturation Linear growth Emotional & social maturation Intellectual function Sexual development Cancer effects Recurrent primary cancer Subsequent neoplasms

Answer 62

Cardiac\Endocrine/GI & Hepatic/Genitourinary/Musculoskeletal/Neurological/Pulmonary Fertility & Reproduction Fertility Health of Offspring Sexual functioning

Answer 63

Mental Health Education Employment Health Insurance Chronic symptoms Physical/Body Image

Answer 64

Neurocognitive IQ and performance reduced Endocrine GH and thyroid hormone def Infertility Precocious puberty ACTH deficiency Obesity , GH def with hypothalamic obesity Neurologic Hearing loss Neuropathy Ataxia and motor defects Second cancers Leukaemias and brain tumours Vascular, Cerebro Moyamoya and microhaemorhages

Answer 65

a group of malignant diseases in which genetic abnormalities in a haemotopoietic cell give rise to an unregulated clonal proliferation of cells; resulting in a disruption of normal marrow function and ultimately marrow failure.

Answer 66

Leukemia - accounts for 31% of all malignancies that occur in children less than 15 years.

Answer 67

Acute Lymphoblastic leukemia

Answer 68

Peak incidence between 2-5years

Answer 69

Down Syndrome, NF 1, Bloom Syndrome, Ataxia Telangectasia 25% blasts in bone marrow

Answer 70

Proliferation of immature lymphoid cells or lymphoblasts.

Answer 71

General systemic effects Fever, lassitude and pallor. Haematologic effects from bone marrow invasion Anaemia – pallor, irritability, decreased activity, Neutropenia - fevers Thrombocytopenia – petechiae, ecchymosis, epistaxis, DIC

Answer 72

Lymphoid System Infiltration Lymphadenopathy, splenomegaly and hepatomegaly. Testicular involvement Renal involvement- haematuria, hypertension and renal failure. GI manifestations- bleeding Bone and joint involvement- bone pain. Leukaemic infiltration of the periosteum, bone infarction or expansion of marrow cavity by leukaemic cells.

Answer 73

CNS involvement Raised ICP(headache, morning vomiting, VI nerve palsy. Focal neurologic signs- convulsions, ataxia, dysmetria etc Hypothalamic syndrome (polyphagia, excessive wt gain, hirsutism) Chloromas of the spinal cord-back pain, numbness, weakness. CNS haemorrhage- leukostasis, leukothrombi-infarcts-haemorrhage (coagulopathy)

Answer 74

Temp > 37.50C on 2 occasions 30mins apart or > 38.30C with Neutropenia: ANC (Absolute neutrophil count) < 1500 cells / mm3 Mild Neutropenia: 1000-1500 cells / mm3 Moderate Neutropenia: 500-999 cells / mm3 Severe Neutropenia: < 500 cells / mm3 Profound Neutropenia: <100 cells/ mm3

Answer 75

All persons should perform hand hygiene before entering and after leaving the patient’s room “Neutropenic diet” (ie, well-cooked foods) Oral hygiene (toothbrushing(soft) at least twice per day; oral rinses at least four times per day; Daily showers or baths Daily inspection of skin sites that may be portals of infection (eg, perineum, sites of intravascular access) Avoidance of rectal procedures (eg, thermometry, enemas, suppositories, PR examinations) Prevent constipation in patients. Antimicrobial stewardship

Answer 76

idiopathic in most cases (interaction between environmental factors (e.g. viral infection) and host genetic susceptibility. Inherited in 10-15% of cases (RB gene mutation – bilateral Retino) Childhood syndromes – Down syndrome and Leukaemia, NF1 and gliomas Infection related –EBV – Burkitts lymphoma, Hodgkins lymphoma, Nasopharyngeal Carcinoma. Kaposi Sarcoma – HIV and Human herpes 8 virus Burkitts lymphoma – HIV and Malaria Others – Radiation (leukemia), chemotherapy, commercial pesticide use (farming, Burkitt's) etc Malaria - Burkitt's Lymphoma

Answer 77

Early warning signs for Cancer S: Seek help for persistent signs I: Eye - White spot, new onset squint, impaired vision, persistent reddening, bulging eyeball L: Lumps - swelling/lumps in any part of the body U: Unexplained: Fever>2 weeks, pallor, tiredness, easy bruising, loss of weight A: Aches - Persistent breaks in bones and joints, bones that break easily. N; Neurological - Change in walking, balance or speech, headache for more than a week with or without vomiting, enlarging head.

Answer 78

VMA and HVA for neuroblastoma. High α-fetoprotein (αFP) in germ cell tumours and liver tumours Biopsy for solid tumours and BMA for Leukaemia

Answer 79

Targeted drug therapy eg Imatinib - Tyroxine Kinase inhibitor - In patients with CML, Rituximab - CD20 inhibitor for Burkitt's lymphoma Immunotherapy eg Car-T Cell Therapy for Leukemia

Answer 80

Adjuvant: short course of high dose, usually combination drugs given after rad. or surg. Neoadjuvant: adjuvant drugs used pre- or perioperative period Palliative: given to improve quality of life or control symptoms if cure is not possible Salvage: curative high dose given when symptoms have recurred or treatment has failed with another regimen

Answer 81

Adjuvant: short course of high dose, usually combination drugs given after rad. or surg. Neoadjuvant: adjuvant drugs used pre- or perioperative period Palliative: given to improve quality of life or control symptoms if cure is not possible Salvage: curative high dose given when symptoms have recurred or treatment has failed with another regimen

Answer 82

Anaemia Thrombocytopenia Bleeding Neutropenia -can lead to infection Undernutrition Aloepecia

Answer 83

Mucositis Nausea/vomiting Diarrhoea Cystitis Sterility Neuropathy Alopecia Cardiotoxiciy Local reaction Renal failure Myelosupression Phlebitis