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Pediatrics > Childhood Cancers > Flashcards

Childhood Cancers Flashcards

1
Q

Chemo agents + Long term effects

A

Doxorubicin- Cardiotoxic
Cisplatinum - Nephrotoxic and Ototoxic
Carboplatinum -nephrotoxic
Vincristine - Neurotoxic
Etoposide - Secondary malignancy
Dactinomycin - Hepatotoxic ( Veno - occlusive disease of liver)
Bleomycin - Pulmonary toxicity (fibrosis)

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2
Q

Treatment of Tumor Lysis

A

Preventative: Hyperhydration - 2.5 L/m2 of IV fluid, allopurinol or urate oxidase (Rasburicase)

Phosphate excretion: Aluminum hydroxide

Treat symptomatic hypercalcemia
Dialysis if progressive renal failure, K>6mEq/l, PO4 >6mg/dl, Oliguria, Anuria and volume overload, symptomatic hypocalcemia, hypertension

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3
Q

Hyperleukocytosis

A

WCC >100 x 10^9/l
More common in AML than ALL

Clinically significant hyperleukocytoses
WCC> 200x10^9/l
AML - >50x 10^9/l

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4
Q

Signs and Symptoms of Hyperleukocytosis

A

CNS - Vision loss, diplopia, delirium, stupor, papilledema, coma
RS - Orthopnea , Dyspnea, Tachypnea, Hypoxia
Genitourinary- Oliguria, anuria, priapism
DIC, retinal hemorrhages, renal vein thrombosis

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5
Q

Management of patients with Hyperleukocytosis

A

Supportive care: Hyper-hydrate with 2.5-3 L/m2 of fluid, prevent TLS, give allopurinol/rasburicase.

If patient is asymptomatic, do chemotherapy

If patient is symptomatic or TLC >300,000/mm3 (AML) or >100,000mm3 (ALL)- Chemotherapy + Leukapheresis

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6
Q

Management of patients with Hyperleukocytosis

A

Coagulopathy correction
Asymptomatic patients: HB>6-7 g/dl: No RBC transfusion
Hb<5-6g/dl or signs of Congestive heart failure: RBC transfusion

Symptomatic
Platelets <20000/mm^3 - Platelets transfusion

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7
Q

Management of Hyperleukocytosis

A

Hyper-hydration - N/S (0.9%) at 3l/m2 IV. Monitor urine output
Start allopurinol 100mg/m2/day in divided doses
Platelet transfusion if platelet count <20 and reduce risk of IntraCranial Hemorrhage

Whole blood transfusion only if symptomatic anaemia or Hb<6. avoid packed cells - causes hyper viscosity
FFP transfusion with Vit K if coagulopathy present
Exchange blood transfusion (Leukopheresis)
Start steroids for acute leukemia, NHL
Hydroxyurea in CML/AML
Start chemotherapy

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8
Q

Management of Febrile Neutropenia

A

Blood cultures
Urinalysis and culture
Culture of septic lesions
CSF culture if there’re signs of meningism
CXR if respiratory signs
Give Ceftriaxone and Gentamycin - 1 line
Meropenam - 2 line
Vancomycin- 3 line

If there’s prolonged FN >96 hours - invasive fungal infection
Or viral infection or TB

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9
Q

Good prognostic indicators for ALL

A

Age - 1 to 9
WBC x10^9 - 10
Immunophenotype - Pre-B cell
Genetics - Hyperploidy, DNA index >1.16
CNS Status - CNS 1
Race - Caucasians
Sex - Female
Organomegaly - Absent
Mediastinal mass - Absent
Response to early treatment- Rapid
MRD( end of induction) - <0.01

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10
Q

Intermediate Prognostic factors for ALL

A

Age - <10^a
WBC x10^9 - >/= 50^a
Immunophenotype - T cell
Genetics - Diploid
CNS Status - CNS 2^a
MRD( end of induction) - 0.01 to 0.99%

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11
Q

Unfavorable prognostic indicators for ALL

A

Age - <1 and MLL+
Genetics - Hypoploidy <44 DNA<1.16
CNS Status - CNS 3
Race - Black
Sex - Male
Organomegaly - Present
Mediastinal mass - Present
Response to early treatment- Slow
MRD( end of induction) - <1%

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12
Q

Treatment of ALL

A

Start with good counselling about condition and treatment (takes 2 years)
Supportive care – packed cells, platelet transfusion.
High fever/Febrile neutropenia and possible septicaemia-antibiotics with Blood cultures.
Allopurinol 10mg/kg/day
Fluid intake of 2-3L/m2/day
Manage hyperleukocytosis
Support to prevent and ameliorate tumour lysis syndrome.

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13
Q

Differential Diagnosis of ALL

A

AML
Aplastic anaemia
Myelofibrosis
Infectious mononucleosis
Juvenile idiopathic arthritis
Osteomyelitis
Infiltration of the bone marrow- neuroblastoma, rhabdomyosarcoma, Ewing sarcoma

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14
Q

Laboratory Investigations for ALL

A

FBC and blood film comment (Blasts in peripheral blood)
Bone marrow aspirates and trephine- Morphology (Blast >25%), histochemistry, immunophenotyping, cytogenetics.
Chest x-ray - Mediastinal mass
Blood chemistry – LFTs and RFT
CSF – blasts and cells (Lumbar Puncture)
Coagulation profile
HIV screen

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15
Q

Treatment Strategies of ALL - Induction and Consolidation

A

Induction: usually in Haematologic malignancies. A combination of high dose drugs to induce complete response when initiating a curative regimen(Vinc, Doxo, L-Aspar, Steroid, IT MTX/Ara C - MAVDAS)

Consolidation: given after induction has achieved a complete remission. Prevents re-proliferation of leukaemic cells. Repeated to increase cure rate or prolong survival. (Ara C, Cyclo, Doxo, Etop - CADE)

Ara-C is cytarabine
IT-MTX - Methotrexate

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16
Q

Treatment Stategies for ALL - Delayed Intensification and Maintenance

A

Intensification: after complete remission is achieved, same agents used in induction or diff agents are given at high doses to effect better cure rate or longer remission (Repeat Induction and Consolidation)

Maintenance: Combination, low dose given on long term basis in remission to prevent re-growth of residual cancer cells( 6MP, MTX, Vinc, steroids for 2 – 3yrs. Females - 2 y. Males - 3 y
MMVS

6MP - Mercaptopurine

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17
Q

Acute Complications from ALL Treatment

A

Tumour lysis syndrome
Renal failure
Sepsis
Bleeding - ‘cause of low thrombocytes from treatment
Thrombosis
Encephalopathy
Seizures
Typhilitis- neutropenic enterocolitis

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18
Q

Chronic Complications of ALL Treatment

A

Secondary malignancy
Short stature
GH deficiency
Learning disability
Cognitive defects
Neuropathy

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19
Q

Presenting features of AML

A

Leukemia Cutis
Gingival hypertrophy
Chloroma

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20
Q

Predisposing factors to AML

A

Down’s syndrome
Fanconi anaemia
Diamond-Blackfan syndrome
MDS and myeloproliferative syndrome
Ionizing radiation treatment
Chemotherapy- Cyclo, ifosfamide, etoposide, chlorambucil.

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21
Q

When is the prognosis for AML poor?

A

WBC > 100,000/mm3
2o AML - For example following treatment of ALL with etoposide

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22
Q

When is prognosis for AML good?

A

Down syndrome
M3 Subtype

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23
Q

How is AML treated?

A

*Ara-C, Doxorubicin. Etoposide (CED), Triple Intrathecal Therapy (Methotrexate/Hydrocortisone/Cytarabine)
*All Trans Retinoic Acid for M3
*High dose cytarabine
*Stem cell transplant for relapse

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24
Q

What is the cytogenic abnormality in CML?

A

Cytogenic Abnormality - Presence of Philadelphia Chromosome, which results from the translocation of chromosomes 9 and 22 (t(9;220) resulting in BCS-ABL fusion protein.

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25
Q

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

A

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

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26
Q

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

A

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

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27
Q

What are the clinical features of the blast crisis of CML?

A

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.

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28
Q

What is the first line treatment of CML?

A

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)

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29
Q

Describe the classification of Lymphomas

A

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.

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30
Q

What is the commonest childhood cancer in Tropical Africa?

A

Non-Hodgkin’s lymphoma

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31
Q

What is NHL?

A

Malignant solid tumour of B or T lymphocytes

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32
Q

When do over 90% of patients with NHL present?

A

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

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33
Q

Which groups of people are more affected? And when is the prognosis good? What is unique about the symptoms

A

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

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34
Q

Clinical Presentation of Burkitt’s Lymphoma of Jaw (Endemic)

A

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)

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35
Q

Clinical Presentation of Abdominal B cell Burkitt’s Lymphoma (Sporadic)

A

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

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36
Q

Clinical Presentation of T cell Lymphoblastic Lymphoma

A

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

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37
Q

Differentiation Between Endemic and Sporadic Burkitt’s Lymphoma

A

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)

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38
Q

Clinical Features of Burkitt’s Lymphoma

A

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

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39
Q

Sanctuary Sites for Burkitt’s Lymphoma -

A

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

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40
Q
A

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)

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41
Q

Other Investigations for NHL

A

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

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42
Q

Staging of Burkitt’s Lymphoma

A

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

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43
Q

ST Judes Staging System for NHL - Stages 1 and 2

A

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.

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44
Q

St Judes Staging for NHL - Stage III and IV

A

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.

45
Q

Treatment of Burkitt’s Lymphoma - Prephase

A

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

46
Q

What factors are considered important for prognosis

A

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

47
Q

What is the most common primary renal tumor of childhood?

A

Wilm’s tumor/Nephroblastoma

48
Q

At what age is Wilm’s tumor mostly diagnosed? What is the common type -

A

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)

49
Q

Signs and symptoms of Wilm’s tumor

A

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

50
Q

Associated Congenital anomalies of Wilm’s tumor

A

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.

51
Q

Paraneoplastic syndromes associated with Wilm’s Tumor

A

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

52
Q

Symptoms of Thrombocytosis

A

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

53
Q

Patterns of Spread of Wilm’s Tumor

A

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

54
Q

Wilm’s tumor staging

A

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)

55
Q

Investigations for Wilm’s tumor

A

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)

56
Q

Wilm’s Tumor Treatment

A

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

57
Q

What are the other renal tumors of childhood?

A

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

58
Q

What are nephrogenic rests?

A

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

59
Q

What is retinoblastoma and how does it grow?

A

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.

60
Q

What age group is affected by retinoblastoma?

A

Children <5 years of age

61
Q

Incidence of Retinoblastoma

A

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

62
Q

What are the signs and symptoms of Retinoblastoma?

A

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

63
Q

How is retinoblastoma classified?

A

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

64
Q

Trilateral retioblastoma

A

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

65
Q

How is Screening for Retinoblastoma done

A

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.

66
Q

How is Diagnosis of Retinoblastoma made?

A

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.

67
Q

Investigations for Retinoblastoma

A

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)

68
Q

Modalities of treatment of Wilms Tumor

A

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.

69
Q

Secondary Malignancies of Retinoblastoma

A

OSTEOSARCOMA
SOFT TISSUE SARCOMAS
MELANOMA
LEUKAEMIA
LYMPHOMA
BREAST CANCER

70
Q

What is a neuroblastoma?

A

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

71
Q

Most common cancer in infancy//Commonly arises where//Common in which groups of people

A

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

72
Q

Sites for neuroblastoma

A

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

73
Q

Clinical Presentation of neuroblastoma

A

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

74
Q

Other syndromes associated with Neuroblastoma

A

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

75
Q

Investigations for Neuroblastoma

A

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

76
Q

Treatment of Neuroblastoma

A

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

77
Q

Give some facts about brain tumors

A

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”)

78
Q

How are brain tumors classified?

A

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

79
Q

Aetiology of Brain Tumors

A

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

80
Q

Clinical Presentation of Brain Tumors

A

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

81
Q

Management of patients with Brain Tumor

A

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!

82
Q

Management Principles for Brain Tumor

A

Surgery
Shunting,
Surgical excision - Gross total excision
Radiotherapy
Chemotherapy

83
Q

Define Late Effects of Cancer

A

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

84
Q

What causes late effects of cancer? What is the prognosis of these effects

A

*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

85
Q

Long Term Growth and Development effects, Cancer effects

A

Skeletal maturation
Linear growth
Emotional & social maturation
Intellectual function
Sexual development

Cancer effects
Recurrent primary cancer
Subsequent neoplasms

86
Q

Long term effects of Cancer on organs/Fertility and reproduction

A

Cardiac\Endocrine/GI & Hepatic/Genitourinary/Musculoskeletal/Neurological/Pulmonary

Fertility & Reproduction
Fertility
Health of Offspring
Sexual functioning

87
Q

Long term effects of cancer on Psychosocial health

A

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

88
Q

Long Term Effects of Radiation

A

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

89
Q

Leukemia definition

A

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.

90
Q

What is the most common malignant neoplasm in childhood?

A

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

91
Q

What is the most common hematopoietic tumor of childhoood?

A

Acute Lymphoblastic leukemia

92
Q

Peak incidence of ALL

A

Peak incidence between 2-5years

93
Q

Predisposing syndromes of ALL + % of blasts in bone marrow

A

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

94
Q

How does ALL occur?

A
95
Q

How does ALL occur?

A

Proliferation of immature lymphoid cells or lymphoblasts.

96
Q

Symptoms and Signs of ALL - General and hematologic effects

A

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

97
Q

Symptoms and Signs of ALL - Lymphoid system infiltration

A

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.

98
Q

Signs and Symptoms of ALL - CNS Involvement

A

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)

99
Q

What is Febrile Neutropenia? Describe the types

A

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

100
Q

What are the infection Precautions for febrile neutropenia?

A

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

101
Q

Aetiology for Childhood Cancers

A

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

102
Q

SILUAN Signs

A

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.

103
Q

Tumor Markers and Pathology for Childhood cancer

A

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

Biopsy for solid tumours and BMA for Leukaemia

104
Q

Targeted drug therapy and Immunotherapy

A

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

105
Q

Treatment strategies in Chemotherapy

A

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

106
Q

Treatment strategies in Chemotherapy

A

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

107
Q

Short Term Side effects of chemotherapy

A

Anaemia
Thrombocytopenia
Bleeding
Neutropenia -can lead to infection
Undernutrition
Aloepecia

108
Q

Side effects of Chemotherapy

A

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

Pediatrics (12 decks)

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