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Malignancies Flashcards

1
Q

Are brain tumours more commonly primary or metastatic in children?

A

Brain tumours in children almost always primary rather than metastatic (contrast to adults)

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2
Q

What are the different types of brain tumour?

A

Astrocytoma (40%) = benign to highly malignant (Pilocytic Astrocytoma most common)

Medulloblastoma (20%) = arises in midline of posterior fossa, associated spinal mets

Ependymoma (8%) = posterior fossa

Brainstem glioma (6%) = malignant tumour, poor prognosis

Craniopharyngioma (4%) = developmental tumour arising from squamous remnant of Rathke pouch, not truly malignant but locally invasive

Atypical teratoid/rhabdoid tumour (ATRT) = rare type of aggressive tumour most commonly occurring in young children

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3
Q

What does an MRI and histopathology show in pilocytic astrocytoma?

A

MRI = cerebellar, well circumscribed, cystic, enhancing

Histopathology = piloid (hairy) cell, Rosenthal fibres and grabnular bodies, slow growing with low mitotic activity

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4
Q

What is the most common brain tumour in children?

A

Pilocytic astrocytoma

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5
Q

What are RFs for pilocytic astrocytoma?

A

Common in NF1
BRAF mutation in 70%

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6
Q

What are the S/S of a CNS tumour?

A
  • Headaches (worse in morning/coughing)
  • Vomiting (on waking)
  • Gait/coordination problems/clumsy
  • Visual changes
  • Irritability, FTT
  • Behaviour or personality change
  • Raised ICP > papilloedema (disc oedema, obscuration of margins, elevation, venous congestion, haemorrhages) > may take hours-weeks to develop so not always found at presentation
  • Separation of sutures/tense fontanelles, developmental delay or increased head circumference
  • Focal signs (depending on location of tumour)
    Intracranial HTN = headache, vomiting, changed mental state
    Supratentorial = focal neurological deficits, seizures, personality change
    Subtentorial = cerebellar ataxia, long tract signs, cranial nerve palsies
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7
Q

What are the investigations for a CNS tumour?

A

MRI > CT / PET (higher radiation)

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8
Q

What is the management for a CNS tumour?

A

MDT
Paediatrician, neurologist, SN, OT, PT, SALT, psychology, radiologist, oncologist, CLIC Sargent

1st line = surgery
Maximal safe resection to obtain extensive excision with minimal damage to pt

+/- Radiotherapy
Low and high-grade gliomas, mets

+/- Chemotherapy
High-grade gliomas

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9
Q

What is the difference between ALL and AML?

A

ALL (80%) = Malignant clonal disease characterised by proliferation of early B- and T-lymphocyte progenitors

AML (20%) = Malignant clonal disease characterised by a block in differentiation and unregulated proliferation of myeloid progenitor cells

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10
Q

What are the S/S of leukaemia?

A

BM failure

  • Anaemia (lethargy, pallor)
  • Thrombocytopaenia (easy bruising/bleeding, petechiae)
  • Neutropoenia (frequent/severe infection)

Local organ infiltration

  • Lymphadenopathy, hepatosplenomegaly, bone pain, gum hypertrophy, testes, CNS

Systemic

  • Malaise, weakness, pyrexia, B symptoms

Leukaemia cutis > petechial rash on face and trunk

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11
Q

What does ALL present with?

A
  • Anaemia
  • Neutropenia
  • Thrombocytopenia
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12
Q

What are the investigations for leukaemia?

A

Bloods

  • FBC > anaemia, neutropoenia, thrombocytopaenia, +/- DIC, high WCC
  • Clotting studies
  • Peripheral blood film > lymphoblasts (any abnormalities ie Auer Rods)

BM biopsy

  • > 20% blasts in BM or peripheral blood (type of blast – B/T-cell lineage tx differently)

CXR

  • Enlarged thymus

FBC may show tumour lysis syndrome > high K, LDH, PO4, uric acid > can cause renal failure

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13
Q

What is the management of leukaemia?

A

Immediate action:
Reduce Tumour Lysis Syndrome > Allopurinol + hyperhydration

(1) Hyperhydration (0.9% NaCl 5% Dextrose > no K)
(2) Allopurinol
(3) Cross-match for blood transfusion
(4) Platelet transfusion
(5) Transfer to specialist unit
(6) Bone marrow aspirate (from PSIS) > send for morphology, cytogenetics, immunophenotyping, minimal residual disease (MRD)
(7) Chemotherapy (2-3yrs, boys tx longer due to accumulation of lymphoblasts in testes)

Specific therapy:

  • CNS-directed therapy (e.g. intrathecal) > done in all pts even if initial LP is negative (6-8 treatments), can also be done by giving high-dose chemo so it penetrates BBB
  • Molecular treatment > Imatinib, Rituximab
  • Transplantation
  • Abx prophylaxis for PCP infection
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14
Q

What is the management of febrile neutropenia?

A

= Temp >38, neutrophil <0.5

  • Prone to gram -ve sepsis
  • A to E approach, sepsis 6
  • Need broad spectrum abx > piptazobactam / gentamicin
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15
Q

What are poor prognostic markers for leukaemia?

A
  • Age <2 or >10
  • Non-Caucasian
  • T/B-cell surface markers
  • Male
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16
Q

What is lymphoma?

A

Lymphomas are neoplasms of lymphoid cells, originating in lymph nodes or other lymphoid tissues (spleen, MALT), sometimes ‘anywhere’ (skin, CNS, testes, breast)

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17
Q

What are 3 types of paediatric-specific lymphoma?

A

Hodgkin’s (more localised, usually only one nodal site) > more common in adolescence

Non-Hodgkin’s (spreads contiguously to adjacent lymph nodes, involves multiple sites and spreads sporadically) > more common in childhood

Burkitt’s (a type of B-cell NHL)

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18
Q

What are the subtypes of Hodgkin’s lymphoma?

A
  • Classical (subtypes exist)
  • Nodular Lymphocyte Predominant HL (NLPHL)
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19
Q

What are the S/S of Hodgkin’s Lymphoma?

A
  • Painless lymphadenopathy (in neck) > can be painful when drinking alcohol
  • Fever, night sweats, weight loss
  • Others include pruritus, cough, dyspnoea with intrathoracic disease, SVC obstruction (blackouts, dyspnoea, feeling of fullness in head)

O/E > skin excoriations, non-tender firm lymphadenopathy, splenomegaly, facial oedema / raised JVP (SVC obstruction)

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20
Q

What are the investigations for Hodgkin’s lymphoma?

A

Bloods > FBC (low Hb, leucocytosis, eosinophilia, lymphopenia), high ESR / CRP, high LDH, high AST/ALT (if liver involved)

LN biopsy > Reed-Sternberg Cells (“Owl eyes”)

Imaging > CXR, CT (thorax, abdo, pelvis), PET scan > Ann Arbor Staging

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21
Q

What is the management for Hodgkin’s lymphoma?

A

Good prognosis > 80% cured

  1. Combination chemotherapy (ABVD) +/- radiotherapy
    - Adriamycin, bleomycin, vinblastine, dacarbazine
    - 2-4 cycles in stage 1/2, 6-8 cycles in stage 3
    - PET scanning monitors response and guides therapy
  2. Intensive chemotherapy and autologous SCT
    - Relapsed patients
22
Q

What are the S/S of Non-Hodgkin’s lymphoma?

A
  • Painless lymphadenopathy +/- compression sx > NO pain after alcohol
  • Fever, night sweats, weight loss

O/E > same as HL

23
Q

What are the investigations for Non-Hodgkin’s lymphoma?

A

Bloods > FBC, ESR, LFTs, LDH, Alb (prognostic markers)

LN/BM biopsy > cytology, histology, immunophenotyping

PET or CT staging > Ann Arbor Staging

24
Q

What is the management of Non-Hodgkin’s Lymphoma?

A

Depends on type of NHL

  • Urgent chemo
  • Monitor only
  • Abx eradication (H. pylori gastric MALToma)
  • T-cell NHL = chemo and CNS prophylaxis as for ALL
  • B-cell NHL = aggressive pulsed chemo regimen for stage IV disease using alkylating agents and methotrexate is improving survival
  • Surgery only indicated for emergency tumour obstruction of airways, bowel, or bladder
25
Q

What are the 3 variants of Burkitt’s lymphoma?

A

Endemic = EBV infection, most commonly in children living in malaria endemic regions, involves JAW or facial bones

Sporadic = EBV infection, western world

Immunodeficiency = HIV infection, pts immunocompromised post-transplant

26
Q

What does the histopathology and molecular analysis of Burkitt’s lymphoma show?

A

Histopathology

  • Arises from germinal centre cells
  • Starry-sky appearance

Molecular

  • C-myc translocation
27
Q

What is the prognosis of Burkitt’s lymphoma?

A

Fastest growing human tumour known
Decent cure rates

28
Q

Where does osteosarcoma most commonly occur?

A
  • Occurs at the end of lone bones (mainly KNEE)
  • Frequently develops in mandible (lower jaw) so caught on XRs during dental visits
29
Q

What are the S/S of osteosarcoma?

A
  • Relatively painless mass/swelling
  • Restricted movement
  • Bone pain > may come and go initially then becomes more persistent > may be worse at night
  • Fracture may occur after minor injury at site of weakened bone
  • RAPID metastasis to lung
30
Q

What are the investigations for osteosarcoma?

A
  • Bloods
  • Imaging > XR shows Codman triangle (elevated periosteum) and ‘sunburst’ pattern (soft tissue calcification), CT/MRI/PET
  • Biopsy > only definitive method to determine whether tumour malignant or benign
31
Q

What is the management of osteosarcoma?

A

> Specialised sarcoma team (London) mx

  • Surgery (limb sparing surgery +/- amputation) + chemo
  • Post-treatment > OT, PT, dietician, orthotics/prosthetics, support (Sarcoma UK)

> Poor prognosis

32
Q

What is Ewing’s sarcoma?

A

Primitive Neuroendocrine Tumour (PNET) / Malignant, small round blue-cell tumour

A rare type of cancer that affects bones or the soft tissue around bones

33
Q

Where does Ewing’s sarcoma most commonly occur?

A

Occurs most frequently in pelvis and long bones of arms, legs, chest, skull and trunk

34
Q

What age group does Ewing’s sarcoma most commonly affect?

A

Mainly seen in children and adolescents (median age 15)

35
Q

What are the S/S of Ewing’s sarcoma?

A
  • Mass or swelling
  • Severe bone pain
  • Malaise, fever, paralysis (may precipitate osteomyelitis)
  • FLAWS
36
Q

What are the 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
37
Q

What is Ewing’s sarcoma associated with?

A

Associated with t(11;22) translocation which results in an EWS-FLI1 gene product

38
Q

What is the management for Ewing’s sarcoma?

A

> Specialised sarcoma team (London) mx

  • Surgery (limb-sparing +/- amputation) + chemo (VIDE) + radiotherapy
  • Post-treatment > OT, PT, dietician, orthotics/prosthetics, support

> Prognosis = 5yr survival at 75%

39
Q

What is the difference between osteosarcoma and Ewing’s sarcoma?

A

OSTEOSARCOMA > forms bone
EWING”S SARCOMA > forms mesenchymal tissue

40
Q

What is retinoblastoma?

A

Primary malignant tumour of the immature cells of the retina

41
Q

What are the features of retinoblastoma?

A
  • Rare
  • Unilateral (80% spontaneous, 20% hereditary), bilateral (100% hereditary)
  • Autosomal dominant, chromosome 13, encodes pRB
  • Average age of dx > 18m
42
Q

What are the S/S of retinoblastoma?

A
  • Absence of red-reflex > replaced by white pupil (leukocoria)
  • Strabismus
  • Squint
  • Visual problems
43
Q

What are the investigations for retinoblastoma?

A
  • Fundoscopy
  • MRI may be needed
  • Genetic testing
44
Q

What is the management of retinoblastoma?

A

> > Aim is to cure but preserve vision

  • 1st line = Chemotherapy
  • Some patients require enucleation - gross anterior chamber involvement, glaucoma, orbital inflammation
  • Laser treatment of the retina
  • Radiotherapy may be used in more advanced cases

MOST PATIENTS ARE CURED (90%)
>Many will be visually impaired

45
Q

What are the complications of retinoblastoma?

A

Significant risk of secondary malignancy

46
Q

What is a neuroblastoma?

A

Tumour arises from neural crest tissue of adrenal medulla and SNS

47
Q

When does neuroblastoma present?

A

Most common <5yrs
Median onset = 20m

48
Q

How severe is neuroblastoma?

A

Spectrum of disease = benign (ganglioneuroma) > malignant (neuroblastoma)

49
Q

What are the S/S of neuroblastoma?

A
  • Abdominal mass (tumour can be anywhere on sympathetic chain)
  • Systemic sx = WL, pallor, hepatomegaly, bone pain, limp
  • Sx of spinal cord compression = paraplegia
  • Proptosis
  • If >2yrs = sx of metastatic disease (bone pain, BM suppression, WL, malaise)
50
Q

What are the investigations for neuroblastoma?

A
  • Radiological findings > calcification may be seen on AXR
  • Raised urinary catecholamine metabolites > vanillylmandelic acid (VMA) and homovanillic acid (HVA)
  • Biopsy = confirmatory from BM and MIBG sampling
51
Q

What is the management of neuroblastoma?

A
  • Localised primaries without metastatic disease > surgery
  • Metastatic disease > chemo + radiotherapy + surgery (high risk of relapse)
  • Spontaneous regression can occur in very young infants

Prognosis > cure rates in metastatic disease ~40%

Paeds (18 decks)

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