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Paediatrics, Year 5 > Malignant Disease > Flashcards

Malignant Disease Flashcards

1
Q

What is the most common CNS tumour in childhood?

A

Pilocytic Astrocytoma (GRADE-1)
common in Neurofibrimatosis 1

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2
Q

What is the histopathology of Policytic Astrocytoma?
With what gene mutation is it usually associated with?

A
  1. Slow growing and low mitotic activity
  2. Piloid cell (hair cell)
  3. Rosenthal Fibers

70% is associated with BRAF mutation

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3
Q

What are the presenting Symptoms of Policytic Astrocytoma?

A
  1. Headaches
  2. Balance issues, co-ordination problems
  3. Vomiting when walking
  4. Visual changes
  5. Failure to thrive-irritability- behavioral changes
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4
Q

What are the signs found with Policytic Astrocytoma?
What is the appropriate modality for investigation?

A

Papilldema: due to an increased ICP
* disc oedema, obscuration of margins, venous congestions, haemmorrhages

MRI> CT/PET (too much radiation)

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5
Q

What is the appropriate management of Policytic Astrocytoma?

A

There needs to be an MDT approach which will include: Surgery, Councelor, padiatrician, neurologists and , CLIC Sargent (Cancer and Leukemia in children social worker)

1st line: Surgery (depends on the type and location of tumor)

2nd line: Radiotherapy for low and high grade; gliomas and metastases

3rd line: Chemotherapy used for high grade gliomas

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6
Q

What are the most common Leukemias in children?
What is the most common age for incidence of Leukemia?

A
  • 80% ALL (85% are B-lineage, 15% T-cell linaege)
  • 20% AMM

2-5yrs old, more common in Males

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7
Q

What are the symptoms of Leukemia in children?

A
  1. BM failure (thrombocytopenia, aneamia, Neutropenia)
  2. Hepatosplenomegaly
  3. Lymphadenopathy
  4. Leukaemia cutis: petechial rash: face and trunk
  5. Tumor lysis syndrome: high K+, LDH, PO2-4, Uric acid
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8
Q

What are the appropriate management for Tumor Lysis Syndrome?

A

Allopurinol and hyperhydration

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9
Q

What are the appropriate investigations for Leukemias?

A

FBC and clotting studies: anaemia, thrombocytopenia and neutropenia (may show Tumor Lysis Syndrome)

Blood film: lymphoblasts

CXR: enlarged thymous

BM biopsy

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10
Q

What is the appropriate management of ALL ?

A

MDT: hollistic approach to treating the patient (paeds, haemoligists, councelor)

  1. Systemic Chemotherapy: 2-3 years of chemo, males might need a longer course as lymphoblasts accumulate in the testes
  2. CNS directed therapy: even if there are no cells in the CNS treatment is administered (6-8 session), where intrathecal chemotherapy in given
  3. Mollecular treatment: Imatinib, Rituximab
  4. Transplantation
  5. Supportive care: blood products, Antibiotics
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11
Q

What is the prognosis of ALL for children and adults?
What are some poor prognostic factors?

A

for children: 80% 5-year survival
for adults 30-40% 5-year survival

Poor prognostic factors:
1. <2yrs old
2. >10yrs old
3. Non-caucasian
4. Males
5. T or B cell surface markers

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12
Q

What are the different types of Lymphoma in children?

A
  1. Hodgkin’s Lymphoma(HL): more common in children, more localised and spreads contiguously (more common in children)
  2. Non-Hodgkin’s Lymphoma (NHL): involves multipe sides and spreads sporadically (more common in adolescents)

If a specific type of cell called a Reed-Sternberg cell is seen, the lymphoma is classified as Hodgkin’s.

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13
Q

What are the signs and symptoms of Hodgkin’s Lymphoma?

A
  1. Painless lymphadenopathy
  2. B symptoms (fever, night sweats, weight loss)
  3. Painful on drinking alcohol
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14
Q

What are the appropriate investigations for Hodgkin’s Lympoma and Non-hodgkin’s lymphoma?

A

LN biopsy
Bloods: FBC, ESR, Alb, LFT’s
PDG-PET

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15
Q

What is the appropriate Staging for Hodgkin’s and non-hodgkin’s lymphoma?

A

Ann-Arbor staging

  1. I: one group of lymph nodes
  2. II: more than one group of lymph nodes but on the same side of the diaphragm
  3. III: nodes are on more than one side of the diaphragm
  4. IV: extranodular metastases
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16
Q

What is the prognosis for Hodgkin’s Lympoma?

A

80% cured (in disseminated disease, this falls to 60%)

17
Q

What is the management of Hodgkin’s lymphoma?

A

Combination Radiotherapy (ABVD)+ Chemotherapy
1. Adriamycin
2. Bleomycin
3. Vincristine
4. DTIC (Dacarbazine)

Pet-scan to monitor for progress

18
Q

What is the presentation of Non-hodkin’s lympoma?

A

Can be low or high grade which describes the speed and aggresivness of onset

Painless lympadenopathy
B-cell symptoms: fever, nigh sweats, weight loss

19
Q

What is the management of Non-hodgkin’s lymphoma?

A

(1) Urgent chemotherapy
(2) Monitor only
(3) Antibiotic eradication (H. pylori gastric MALToma)

20
Q

What type of malignancy is Burkitt’s lymphoma and what are the different variants?

A

It is a subtype of B-cell Non-hodgkin’s Lymphoma

There are 3 variants:
1. Endemic – EBV infection (chronic malaria may reduce EBV resistance):
Most commonly in children living in malaria endemic regions
Most common childhood cancer in Africa
Involves JAW or facial bones
2. Sporadic – EBV infection: Western world, associated with EBV infection
3.Immunodeficiency – HIV infection

21
Q

What is the histopathology of Burkitt’s Lymphoma?

A
  • Arises from germinal center cells
  • Starry-sky appearances
22
Q

What is the most common primary bone malignancy of chilhood?

A

Osteosarcoma: more common in Males, long bones (60-75% in knee)

23
Q

What are the symptoms of Osteosarcoma, where does it commonly metastasize?

A
  • Relatively painless, mass swelling, restricted movments
  • commonly metastasized to lungs
24
Q

What are the appropriate investigations for Osteosarcoma?

A

(1) X-ray: bone destruction and formation
There are soft tissue calcification (sunburst appearance)
Elevated periosteum: Codman’s Triangle
(2) Biopsy
(3) CT/PET/MRI

25
Q

What is the appropriate management of Osteosarcoma?

A
  1. Specialist Sarcoma team
  2. Surgery (limb sparing surgery/ amputation)
  3. Chemotherapy
  4. Post-treatment: OT, PT dietician
26
Q

What is the difference between Osteosarcoma and Ewing’s Sarcoma?

A

Osteosarcoma: forms bone
Ewing’s Sarcoma forms mesenchymal tissue

27
Q

What type of tumor is Ewing’s sarcoma?
What’s the commonest age and which bones are usually affected?

A

It is a bone tissue malignancy, Primitive Neuroendocrine Tumour (PNET)

  • <25y, median 15y
  • Long bones of arms, legs, chest, skull and trunk
28
Q

What are the main Symptoms of Ewing’s Sarcoma?

A

Mass or swelling and bone pain

Malaise, fever, paralysis (may precipitate osteomyelitis)

29
Q

What are the appropriate investigations for Ewing’s Sarcoma?

A

XR (bone destruction with overlying onion-skin layers of periosteal bone formation)

Biopsy (small round blue cells)

CT/PET/MRI

30
Q

What is the appropriate management for Ewing’s sarcoma?

A

o Specialised sarcoma team (London) management
o Surgery (limb-sparing surgery ± amputation) + chemotherapy (VIDE) + radiotherapy
o Post-treatment  OT, PT, dietician, orthotics/prosthetics, support
o Prognosis  survival 5-year at 75% (20-40% for metastasis

31
Q

What is the aetiology of Retinoblastoma?
What are the different types?
What is the most common age ?

A

RARE but accounts for 5% of severe visual impairment in children

  1. Unilateral (80% spontaneous, 20% hereditary) or
  2. billateral (100% hereditary)

Autosomal dominant, chromosome 13–> encodes pRB (protein retinoblastoma)

Average age of diagnosis = 18 months

32
Q

What are the signs of Retinoblastoma and what are the appropriate investigations?

A

Signs & symptoms:

  • Red reflex absent (i.e. a white pupillary reflex instead of normal red one)
    Squint

Investigations:

  • MRI and EUA
33
Q

What is the appropriate management of Retinoblastoma and what is the prognosis?

A

Management:

  1. Enucleation (removal of eye, leaving eye muscles intact)
    2.** Chemotherapy (bilateral)** + laser treatment to retina ± chemotherapy (advanced disease)

Prognosis:

  • Most cured, some may be visually impaired (>90% survive to childhood)
  • Risk of secondary malignancy (sarcoma) in survivors of hereditary retinoblastoma
34
Q

What type of tumor is a neuroblastoma?
Which is the commonest age?

A

Neuroblastoma arises from neural crest tissue in adrenal medulla and SNS
Most common extra-cranial tumour in children
Most common in bellow 5 years of age

35
Q

What are the signs and symptoms with Neuroblastoma?

A
  • **Abdominal Mass **(tumour can be anywhere in the sympathetic chain)
  • Systemic symptoms: WL pallor, hepatomegaly, bone pain, limp
  • Symptoms of spinal cord compression
  • Over 2yo–> symptoms of metastatic disease (bone pain, BM supression, WL, malaise)
36
Q

What are the appropriate investigations for Neuroblastoma?

A

o Radiological findings
o Raised urinary catecholamine metabolites (VMA/HVA)
o Confirmatory biopsy from BM and MIBG sampling

37
Q

What is the appropriate management for Neuroblastoma?
What is the prognosis ?

A

Spontaneous regression can occur in very young infants

Localised primaries without metastatic disease–> surgery alone

Metastatic disease–> chemotherapy + radiotherapy (with autologous stem cell rescue) + surgery (High risk of relapse )

Prognosis–> cure rates for children with metastatic disease is around 40%

Paediatrics, Year 5 (6 decks)

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