Are brain tumours more commonly primary or metastatic in children?
Brain tumours in children almost always primary rather than metastatic (contrast to adults)
What are the different types of brain tumour?
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
What does an MRI and histopathology show in pilocytic astrocytoma?
MRI = cerebellar, well circumscribed, cystic, enhancing
Histopathology = piloid (hairy) cell, Rosenthal fibres and grabnular bodies, slow growing with low mitotic activity
What is the most common brain tumour in children?
Pilocytic astrocytoma
What are RFs for pilocytic astrocytoma?
Common in NF1
BRAF mutation in 70%
What are the S/S of a CNS tumour?
- 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
What are the investigations for a CNS tumour?
MRI > CT / PET (higher radiation)
What is the management for a CNS tumour?
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
What is the difference between ALL and AML?
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
What are the S/S of leukaemia?
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
What does ALL present with?
- Anaemia
- Neutropenia
- Thrombocytopenia
What are the investigations for leukaemia?
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
What is the management of leukaemia?
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
What is the management of febrile neutropenia?
= Temp >38, neutrophil <0.5
- Prone to gram -ve sepsis
- A to E approach, sepsis 6
- Need broad spectrum abx > piptazobactam / gentamicin
What are poor prognostic markers for leukaemia?
- Age <2 or >10
- Non-Caucasian
- T/B-cell surface markers
- Male
What is lymphoma?
Lymphomas are neoplasms of lymphoid cells, originating in lymph nodes or other lymphoid tissues (spleen, MALT), sometimes ‘anywhere’ (skin, CNS, testes, breast)
What are 3 types of paediatric-specific lymphoma?
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)
What are the subtypes of Hodgkin’s lymphoma?
- Classical (subtypes exist)
- Nodular Lymphocyte Predominant HL (NLPHL)
What are the S/S of Hodgkin’s Lymphoma?
- 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)
What are the investigations for Hodgkin’s lymphoma?
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
What is the management for Hodgkin’s lymphoma?
Good prognosis > 80% cured
- 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 - Intensive chemotherapy and autologous SCT
- Relapsed patients
What are the S/S of Non-Hodgkin’s lymphoma?
- Painless lymphadenopathy +/- compression sx > NO pain after alcohol
- Fever, night sweats, weight loss
O/E > same as HL
What are the investigations for Non-Hodgkin’s lymphoma?
Bloods > FBC, ESR, LFTs, LDH, Alb (prognostic markers)
LN/BM biopsy > cytology, histology, immunophenotyping
PET or CT staging > Ann Arbor Staging
What is the management of Non-Hodgkin’s Lymphoma?
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
