Paediatric oncology Flashcards
Name 5 oncological emergencies
- Sepsis/febrile neutropenia
- Raised ICP
- Spinal cord compression
- Mediastinal mass
- Tumour lysis syndrome
Tumor lysis syndrome definition [2]
4 features on blood investigations
Metabolic derangement [1] due to rapid death of tumor cells [1]
Hyperphosphatemia, hypocalcemia
Hyperuricemia, hyperuricosuria
Tumor lysis syndrome treatment [6]
Hyperhydrate and diuresis Monitoring: ECG, phosphate Allopurinol, urate oxidase Phosphate binders Correction of hyperkalemia Dialysis
Treatment of hyperkalemia [3]
Calcium gluconate
Salbutamol
Insulin
Febrile neutropenia [2]
Neutrophils < 0.5 x 109/L and Fever > 38.0°
Spinal cord compression
Presentation in children [6]
Management [3]
Muscle weakness Spine tenderness Sphincter disturbance Sensory disturbance Back pain Gait disturbance
Dexamethasone
Chemotherapy
Surgery - debulking, laminectomy, lminotomy, laminoplasty
How to prevent tumour lysis syndrome? [1]
Pre-chemotherapy hydration measures
Define purpura
Differentials of purpura [6]
Bleeding into skin from small blood vessels to produce a non-blanching rash.
Differentials
• Meningococcal septicaemia
• ALL
• Viral infection e.g. measles, enteroviruses
• Vasculitis e.g. Kawasaki, Henoch Schonlein Purpura
• Idiopathic thrombocytopenic purpura
• Congenital bleeding disorders
Investigations [4]
FBC
Blood film
Coagulation screen
Von Willebrands factor screen
Idiopathic thrombocytopenia purpura Ax [2] Sxs Ix [3] Complication
Ax:
- Type II Hypersensitivity
- Follows viral infection
Sxs: platelet type bleeding
FBC & blood film
Bone marrow biopsy
CT head
Cx: intracranial haemorrhage
ITP
What would you see on FBC & blood film?
Management [3]
FBC & blood film - isolated thrombocytopenia
Mx:
- Avoid NSAIDs, contact sports
- Acute: gradual resolution over 3m, supportive tx optional, splenectomy (severe)
- Chronic: anti-D, RITUXIMAB, splenectomy
Generalized lymphadenopathy
Differentials [5]
Viral infection Bacterial infection Parasitic: toxo Autoimmune JIA Malignancy ALL
Examination of lymphadenopathy. What features of the LN would indicate:
Normal [3]
Malignancy [6]
Infection [4]
o Normal: bilateral anterior cervical nodes up to 2cm, axillary nodes up to 1cm, inguinal nodes up to 1.5cm
o Malignancy: supraclavicular (chest or abdo malignancy), epitrochlear (Hodgkin lymphoma), firm, non-tender, matted together or rubbery
o Infection: erythema, warmth, tenderness, fluctuance
Chronic non-specific lymphadenitis
Define [2]
Causative organisms [3]
Ix [2]
Chronic inflammation of LN in response to pathogens. Enlarged painless LN
Causative organisms:
- Cat scratch disease (bartonella hernselae)
- HSV, toxoplasmosis
- TB, atypical mycobacterium
Ix: FNAC or biopsy
Chronic non-specific lymphadenitis - Cat scratch disease
Sxs [3]
Mx
Fever, headache
Regional lymphadenopathy
Malaise
Mx: conservative
Henoch Schonlein Purpura
Define
Features [6]
IgA mediated small vessel vasculitis
Features:
- Palpable purpuric rash, localized edema
- Over buttocks and extensor surfaces of arms/legs
- 1-3d post infection
- Abdominal pain
- Polyarthritis
- IgA nephropathy eg haematuria, renal failure
HSP
Tx [2]
Prognosis [2]
Treatment
- analgesia for arthralgia
- treatment of nephropathy is generally supportive
Prognosis
- usually excellent, HSP is a self-limiting condition, especially in children without renal involvement
- around 1/3rd of patients have a relapse
Purpura NAI
Thrombocytopenic bleeding normally on legs or head in crying babies; be suspicious of NAI or severe congenital coagulation disorder if anywhere else
Abdominal mass differentials
Neuroblastoma
Wilms tumour
Neuroblastoma
Epidemiology
Ax [2]
Sxs [6]
Ep: 0-5y/o (most common solid tumour in under 5’s)
Ax:
- embryonal neoplasm derived from sympathetic neuroblasts
- most commonly adrenal in origin but can be anywhere along sympathetic chain
Sy/Si:
- abdominal mass
- lethargy, weight loss
- hypertension
- metastasis (lymph nodes, scalp, bone
- spinal cord compression
- bruising and swelling of eye
Neuroblastoma
Ix [5]
Mx [2]
Ix:
- FBC (thrombocytopenia)
- raised urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels
- CT chest abdo pelvis (MRI if suspect spinal lesions)
- MIBG radioisotope scan (specific to catecholamine)
- biopsy
Mx:
- chemotherapy e.g. (CYCLOPHOSPHAMIDE and DOXORUBICIN)
- RT, surgery
Wilms tumour
Ax
RF [3]
Ax: nephroblastoma Risk factors: - Bethwith-Wiedemann syndrome - WAGR syndrome - hemihypertrophy
Wilms tumour
Sxs [3]
Ix [5]
Sy/Si:
- abdominal mass
- painless haematuria, flank pain
- anorexia, weight loss, hypertension
Ix:
- FBC, U&E, urinalysis, CT chest abdo pelvis, AVOID transcutaneous needle biopsy
Wilms tumour
Mx [3]
Prognosis
Mx:
- Nephrectomy
- Chemotherapy
- RT if andvanced
Prognosis: 90% 5y survival
What is the most common cancers in children
Leukaemia - 33%
Brain tumour - 25% but higher mortality (posterior common)
Extra-cranial
Give examples of extra cranial cancers [6]
Lymphoma - Hodgkin's / non-Hodgkin's Neuroblastoma Nephroblastoma (Wilms), Hepatic Retinoblastoma Malignant Bone Germ cell - teratoma secrete ALP
What age group is most likely to get cancer
Peak at 0-4 = highest rate
Another peak at 18-22
What are people with Down’s likely to get
Leukaemia
What suggests immediate referral to hospital (same day) [2]
Unexplained petechiae
HSM
What suggests urgent referral (48 hour) [3]
Repeated attendance with same problem
New neuro symptoms
Abdominal mass
What suggests a referral (2 week) [6]
Rest pain, Back pain Unexplained lump Lymphadenopathy Unexplained and persistent symptoms Extreme fatigue, Extreme weight loss Changes in mole
How does febrile neutropenia present [4]
Fever (sometimes low temp)
Rigors
Drowsiness
Shock - tachy, tachypnoea, hypotension, prolonged cap refil, reduced UO, metabolic acidosis
How do you manage febrile neutropenia [5]
IV access Broad spectrum antibiotics ABC - oxygen / fluids Inotropes Support of PICU
What cancer can present with spinal cord compression [3]
Almost all tumours
Ewing’s sarcoma involving vertebral body
Medullablastoma
Neuroblastoma + germ cell
What causes Superior vena cava syndrome [5]
Tumour pressing on vena cava or medisitunum
- Lymphoma
- Neuroblastoma
- Germ cell
- Thrombosis
Signs of SVCS
Facial, neck and upper thoracic plethora Oedema Cyanosis Distended veins Ill Anxious Reduced GCS
Signs of superior mediastinum syndrome
Dyspnoea especially flat Orthopnoea Tachypnoea Cough Wheeze, Stridor
How do you investigate SVSC / SMS [3]
Mx [6]
CXR
CT chest
ECHO
- Keep upright and calm
- Urgent biopsy
- FBC / pleural aspirate / GCT marker (germ-cell tumour)
- Steroid
- Chemotherapy, RT
- Thrombolytic therapy
Retinoblastoma
Ax & inheritance [3]
Presentation [5]
Ix [3]
Heritable (40%) Autosomal dominant of RB gene on 13q14
Non-inheritable 60%
Strabismus Leukocoria (white pupil) Decreased vision Proptosis No red light reflex
Ix:
- Examination under anaesthesia with maximally dilated pupil
- Bidimensional ocular USS & MRI
- Blood and tumor sample testing for RB1 gene
Retinoblastoma
What are 5 interventions?
What is the 5y survival rate in UK?
- Chemotherapy
- Enucleation - eye removal
- External beam RT
- Ophthalmic tract brachytherapy
- Cryotherapy and Transpupillary thermotherapy (TTT)
5 yr survival 99%
Retinoblastoma - what are 3 drugs used in chemotherapy
Chemotherapy:
- Carboplatin
- Vincristine
- Etoposide
If heritable retinoblastoma, what follow up is needed? [2]
o MRI surveillance: develop new tumours after dx so 6 monthly for 5y
o Parental and sibling screening: counselling and pre-symptomatic mx; must consider germline mosaicism as still a risk of having more children with retinoblastoma
What to do when a child with an unexplained enlarged abdominal mass presents to the clinic?
Arrange paediatric review with 48 hours
