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Y5 Paediatrics > Malignant Disease > Flashcards

Malignant Disease Flashcards

(52 cards)

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1
Q

What is the most common solid organ tumour in childhood?

A

CNS → leading cause of childhood cancer deaths

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2
Q

What are the types of CNS tumours in childhood?

A
  • Astrocytoma (cerebellar) - 40%
  • Medulloblastoma (cerebellar) - 20%
  • Ependymoma - 8%
  • Brainstem glioma - 6%
  • Craniopharyngioma - 4%
  • Atypical teratoid/rhabdoid tumour
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3
Q

What are the features of astrocytoma?

A

Benign to highly malignant → most common = pilocytic astrocytoma

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4
Q

What are the features of medulloblastoma?

A
  • Arise from midline posterior fossa
  • Associated spinal metastases
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5
Q

What are the features of ependymoma?

A

Posterior fossa

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6
Q

What are the features of brainstem glioma?

A

Malignant tumours with poor prognosis

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7
Q

What are the features of craniopharyngioma?

A
  • Squamous remnant of Rathke pouch
  • Not truly malignant but locally invasive
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8
Q

What are the features of atypical teratoid/rhabdoid tumour?

A

Rare type of aggressive tumour occurring in young children

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9
Q

In which condition is pilocytic astrocytoma common?

A

Neurofibromatosis I (NF1)

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10
Q

What is the histopathology of pilocytic astrocytoma?

A
  • Piloid (hairy) cell
  • Rosenthal fibres and granular bodies
  • Slow growing with low mitotic activity
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11
Q

What is the most common mutation in pilocytic astrocytoma?

A

BRAF

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12
Q

What are the signs and symptoms of CNS tumours?

A
  • Headaches → worse in morning, coughing
  • Vomiting → on waking
  • Gait problems / Co-ordination problems / Clumsy
  • Irritability
  • Failure to thrive
  • Visual changes
  • Behaviour or personality change
  • Raised ICP
    • Papilledema = disc oedema, obscuration of margins, elevation, venous congestion, haemorrhages
  • Separation of sutures/tense fontanelle
  • Developmental delay
  • Increased head circumference
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13
Q

What is the management of benign intracranial hypertension?

A

LP with manometry → siphon off CSF to reduce intracranial pressure + monitor

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14
Q

What are the focal signs for intracranial hypertension?

A
  • Headache
  • Vomiting
  • Changed mental state
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15
Q

What are the focal signs for supratentorial?

A
  • Focal neurological deficits
  • Seizures
  • Personality change
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16
Q

What are the focal signs for subtentorial?

A
  • Cerebellar ataxia
  • Long tract signs
  • Cranial nerve palsies
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17
Q

What are the appropriate investigations for suspected CNS tumours?

A

MRI > CT / PET

  • Pilocytic astrocytoma = cerebellar; well circumscribed, cystic, enhancing
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18
Q

What is the management of CNS tumours in children?

A
  • MDT = paediatrician, neurologist, SN, OT, PT, SALT, psychology, radiologist, oncologist, CLIC Sargent
  • 1st line = Surgery - maximal safe resection to obtain and extensive excision with minimal damage to the patient
    • Resectability is dependent on the location, site and number of lesions
    • Craniotomy = debulking (subtotal and complete resections)
    • Open biopsies = inoperable but approachable tumours
    • Stereotactic biopsy = open biopsy not indicated
  • Radiotherapy → for low and high-grade gliomas, metastases
  • Chemotherapy → for high-grade gliomas (temozolomide)
  • Biological agents (EGFR inhibitors, PD-1 inhibitors etc.)
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19
Q

When is the peak incidence of leukaemia in children?

A

2-5 years → M > F

  • 80% = ALL
  • 20% = AML
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20
Q

What are the signs and symptoms of childhood ALL?

A
  • Bone marrow failure - anaemia, thrombocytopenia, neutropoenia
  • Local infiltration
    • Lymphadenopathy (± thymic enlargement)
    • Splenomegaly
    • Petechial rash on face and trunk
    • Hepatomegaly
    • Bone (causing pain)
    • Testes, CNS → are ‘sanctuary sites’ as chemotherapy doesn’t readily reach them
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21
Q

What are the appropriate investigations for childhood ALL?

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A
  • FBC and clotting studies – anaemia, neutropenia, thrombocytopaenia ± DIC, lumour lysis syndrome
  • Peripheral blood film – lymphoblasts
  • CXR – enlarged thymus
  • Bone marrow biopsy
    • >20% blasts in BM or peripheral blood
    • Immunological and cytogenic characteristics
22
Q

What is the management of Tumour Lysis Syndrome?

Study These Flashcards
A
  • Allopurinol
  • Hyperhydration
23
Q

What is the management of childhood ALL?

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A
  • Systemic chemotherapy
    • 2-3 years of therapy
    • Boys treated for longer because testes are a site of accumulation of lymphoblasts
  • CNS-directed therapy (e.g. intrathecal)
    • This is done in all patients even if initial LP is negative (6-8 treatments)
  • Molecular treatment
    • Imatinib (Tyrosine Kinase Inhibitor) for Ph +ve cases
    • Rituximab (monoclonal antibodies against CD20 for B-cell depletion)
  • Transplantation
  • Supportive care
    • Blood products
    • Broad-spectrum antibiotics
24
Q

What is the prognosis of ALL?

Study These Flashcards
A
  • Children = 5-year disease-free survival of 80%
  • Adults = 5-year disease-free survival of 30-40%
25
What are the poor prognostic markers for ALL?
* Age \<2 or \>10yo * T/B-cell surface markers * Non-Caucasian * Male sex
26
What forms of of lymphoma are most common in children?
* NHL = more common in childhood * HL = more common in adolescence - *more localised and contiguous spread*
27
What is an Ewing's sarcoma?
Primitive Neuroendocrine Tumour (PNET) * Malignant, small round blue-cell tumour
28
What are the signs and symptoms of Ewing's sarcoma?
* Mass or swelling and **Bone pain** * **Long bones** of arms, legs, chest, skull and trunk * Malaise * Fever * Paralysis → may precipitate osteomyelitis
29
What mutations are associated with Ewing's sarcoma?
* **t (11:22)** * EWSR1/FLI1 * q24; q12
30
What are the signs and symptoms of Ewing's sarcoma?
* X-Ray → bone destruction with overlying _onion-skin layers_ of periosteal bone formation * **Biopsy** → small round blue cells * CT/PET/MRI
31
What is the management of Ewing's sarcoma?
* Specialised sarcoma team management * **Surgery** → limb-sparing surgery ± amputation + chemotherapy + radiotherapy * Post-treatment → **OT, PT, dietician, orthotics/prosthetics, support**
32
What is the prognosis of Ewing's sarcoma?
Survival 5-year at 75% - *20-40% for metastasis*
33
What is retinoblastoma?
Malignant tumour of retinal cells. * Rare but accounts for 5% of severe visual impairment in children * Unilateral = 80% spontaneous, 20% hereditary * Bilateral (100% hereditary) * Autosomal dominant, chromosome 13 → encodes pRB (protein retinoblastoma) * Average age of diagnosis = 18 months
34
What are the signs and symptoms of retinoblastoma?
* **Red reflex -ve** → white pupillary reflex instead of normal red one * Squint
35
What are the appropriate investigations for suspected retinoblastoma?
* Ophthalmological assessment * MRI * Examination Under Anaesthesia
36
What is the management of retinoblastoma?
* **Unilateral = Enucleation** = removal of eye - *leaves eye muscles intact* * **Bilateral = Chemotherapy + Laser treatment to retina ± Chemotherapy** *(advanced disease)* * Prognosis * Most cured * Some may be visually impaired * Risk of secondary malignancy (sarcoma) in survivors of hereditary retinoblastoma
37
What is neuroblastoma?
Tumours arising from neural crest tissue in adrenal medulla and SNS. **most common extra-cranial tumour in children** * Spectrum of disease * Benign = ganglioneuroma * Malignant = neuroblastoma * Most common extra-cranial tumour in children * Most common \<5yo
38
What are the signs and symptoms of neuroblastoma?
* **Abdominal mass** - can be anywhere on 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 suppression, WL, malaise
39
What are the appropriate investigations for suspected neuroblastoma?
* Radiological findings * Raised urinary catecholamine metabolites (VMA/HVA) * Confirmatory biopsy from bone marrow and MIBG sampling
40
What is the management of neuroblastoma?
* **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 * Poor Prognosis * Metastatic disease cure rate = 40% * MYCN gene
41
What are the types of Hodgkin's lymphoma?
* Classical (subtypes exist) - *95%* * Nodular Lymphocyte Predominant HL (NLPHL) - *5%*
42
What are the sign and symptoms of Hodgkin's lymphoma?
* **Painless lymphadenopathy** (in neck) → *painful on drinking alcohol (in 10%)* * B symptoms (fever, night sweats, weight loss) → *uncommon even in advanced disease*
43
What are the appropriate investigations for suspected Hodgkin's lymphoma?
* LN biopsy – *Reed-Sternberg cells “Owl’s eyes”* * PDG-PET or CT staging – ***Ann Arbor Staging*** * Bloods *– prognostic markers* * FBC * ESR * LFTs * LDH * Alb * Immunophenotyping – CD30, CD15 *– diagnostic markers*
44
How is lymphoma staged?
Ann Arbor Staging
45
What is the management of Hodgkin's lymphoma?
* **Combination Chemotherapy** (ABVD) **± Radiotherapy** * *Adriamycin* * *Bleomycin* * *Vincristine* * *DTIC (Dacarbazine)* * PET scanning monitors response and guides therapy * Prognosis = 80% cured * In disseminated disease = 60%
46
What are the types of Non-Hodgkin's lymphoma?
* Common * **Diffuse Large B-cell** (30-40%) * **Follicular** (35%) * Interesting/uncommon * H. pylori MALToma * EATL * HIV-associated
47
What are the signs and symptoms of Non-Hodgkin's lymphoma?
* Painless lymphadenopathy ± compression symptoms * B symptoms (fever, night sweats, weight loss)
48
What are the appropriate investigations for suspected Non-Hodgkin's lymphoma?
* LN biopsy – *cytology, histology and immunophenotyping* * PDG-PET or CT staging – *Ann Arbor Staging* * Bloods *– prognostic markers* * FBC * ESR * LFTs * LDH * Alb
49
What is the management of Non-Hodgkin's lymphoma?
* Depends on type of NHL * Urgent chemotherapy * Monitor only * HSCT * Antibiotic eradication (H. pylori gastric MALToma) * Diffuse large B-cell lymphoma (30-40% of all NHLs): * Treated with 6-8 cycles of R-CHOP * *Rituximab* * *Cyclophosphamide* * *Adriamycin* * *Vincristine* * *Prednisolone* * Some eligible for HSCT
50
What is Burkitt's lymphoma?
A type of B-cell Non-Hodgkin's lymphoma * Prognosis = bad → fastest growing human tumour known
51
What are the types of Burkitt's lymphoma?
* **Endemic – EBV infection** * Most commonly in children living in malaria endemic regions - *chronic malaria may reduce EBV resistance* * Most common childhood cancer in Africa * Involves JAW or facial bones * **Sporadic – EBV infection** * Western world, associated with EBV infection * **Immunodeficiency – HIV infection** * Usually associated with HIV infection or immunocompromised post-transplant
52
What is the histopathology and molecular results of Burkitt's lymphoma?
* Hispathology * Arises from germinal centre cells * **Starry-sky appearance** * Molecular * C-myc translocation * (8;14, 2;8 or 8;22)

Y5 Paediatrics (26 decks)

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