Paed:Malignancy Flashcards
What is the most common malignancy in children?
Leukaemia followed by brain tumours
What are the general clinical presentations of malignancy in a child?
- localised mass (e.g. lymphadenopathy, ,organomegaly, soft tissue or body mass)
- consequences of disseminated disease (e.g. bone marrow infiltration causing systemic ill-health)
- consequences of pressure from a mass on local structures or tissue (airway obstruction secondary to enlarged lymph nodes in mediastinum)
What investigations are carried out if suspecting malignancy?
Radiology: xray, US, CT, MRI
Pathoogy: bone marrow biopsy/aspiration, tumour biopsy
Tumour marker studies:
- increased urinary catecholamine creation can confirm neuroblastoma
- increased alpha-fetoprotein can confirm germ cell tumour and liver tumour
What is the general management for cancer in children?
Chemo, radio, surgery, high-dose therapy with bone marrow rescue
What are the different types of leukaemia and their prevelances in children?
ALL 80%
AML 15%
CML 5%
What is the clinical presentation of leukaemia?
2-5years old General: malaise, anorexia, fever Bone marrow infiltration causes: - anaemia: pallor, lethargy - neutropenia: infection - thrombocytopenia: bruising, petechiae, nose bleeds and bone pain
Reticulo-endotehlia infiltration causes:
- hepatosplenomegaly
- lymphadenopathy
- superior mediastinal obstruction
Other organ infiltration:
- CNS: headaches, vomiting, nerve palsies
- Testes: testicular enlargement
What investigations are done in leukaemia?
FBC: low Hb + thrombocytopenia + evidence of circulating leukaemia blast cells
Bone marrow exam: confirm diagnosis and identify immunological + cryogenic characteristics
CXR: mediastinal mass characteristic of T-cell disease
Lumbar puncture
What are the 5 phases of chemo in leukaemia?
1) induction
2) consolidation + CNS protection
3) interim maintenance
4) delayed intensification
5) continuing maintenance
When is a haemopoietic cell transplantation used?
For high risk leukaemia pt in first remission or relapsed pt
What is the predominant type of brain tumour?
Infratentorial involving cerebellum, midbrain, brainstem
What are the different types of brain tumours?
Astrocytoma (40%) Medulloblastoma (20%) Ependymoma (8%) Brainstem glioma (6%) Craniopharyngioma (4%)
What is a craniopharyngioma?
A development tumour arising from the squamous remnant of the Rathke much. Not truly malignant but locally invasive and grows slowly in the suprasellar region.
What are clinical features of brain tumours?
Raised ICP Sx - nystagmus, ataxia, early morn headache worse on lying down, vomiting, papillodema, squint, personality/behavioural change Focal neurological signs Back pain Peripheral weakness of arms and legs Bladder/bowel dysfunction
What investigations are done for a brain tumour?
MRI scan
Magnetic resonance spectroscopy
Lumbar puncture (only with neurosurgical advice if suspicion of raised ICP)
Mx of brain tumour
Surgery: treat hydrocephalus, provide tissue diagnosis and attempt maximum reception
–> resection + VP shunt
Chemo + radio
What is lymphoma?
malignancy of the cells of the immune system
- non-hodgkins more common in childhood
- Hodgkins in adolescence
Clinical features of Hodgkins lymphoma
painless lymphadenopathy in neck
lymph nodes may cause airway obstruction
investigations for lymphoma
lymph node biopsy
radiological assessment of all nodal sites + bone marrow biopsy
mx of lymphoma
combo chemo + radio
PET scanning for monitoring
Causes of lymphadenopathy?
HIV infetion
Autoimmune conditions
Storage disorders
Malignancy
Clinical presentation of non-hodgkins lymphoma
Localised lymph node disease in head, neck or abdomen
Abdo disease: pain from intestinal obstruction, palpable mass + intersussception
What is a neuroblastoma?
Neural crest tube tumour arising from sympathetic tissue of the adrenal medulla
Median age of onset: 20 months
Clinical features of neuroblastoma?
Abdo mas, neck mass, chest mass
- pallor, weight loss, hepatomegaly, bone pain, limp, paraplegia, cervical lymphadenopathy, proptosis, periorbital bruising, skin nodules
What investigations are done for neuroblastoma?
Characteristic clinical + radiological features with raised urinary catecholamine levels suggest neuroblastome
- confirmatory biopsy
- bone marrow sampling
- MIBG scan
- bone scan
- bone marrow scan for neuroblastoma cells in ‘rosettes’
Mx of neuroblastoma
Surgery, radiation + chemo
Maybe bone marrow transplantation
What indicates poor prognosis of neuroblastoma?
- over expression of N-myx oncogene
- evidence of deletion of material on Chr 1
- again of genetic material on Chr 17q in tumour cells
What is nephroblastoma also known as?
Wilms tumour
What is wilms tumour?
Originates from embryonal renal tissue and commonest cause of renal tumour of childhood
What is clinical presentation of wilms?
80%: asymptomatic abdominal mass
Uncommon sx: abdo pain, anorexia, anaemia, haemturia, HTN
DD of nephroblstoma
hydronephrosis, PCK, renal cell carcinoma, neuroblastoma, lymphoma
Ix of wilms
US/CT/MRI: shows an intrinsic renal mass distorting the normal structure
Staging: lung mets
Mx of wilms
initial chemo then nephrectomy
What other congenital abnormalities might be assoc with wilms tumour?
sporadic anridia, hemihyperthrophy, genitourinary abnormalities
What is a retinoblastoma?
Malignant tumour of retinal cells and accounts for 5% of vision impairment in children
What’s the difference between bilateral and unilateral retinoblastoma?
Bilateral: hereditary
Unilateral: 20% hereditary, dominant incomplete penetrance on Chr 13, mostly present in first 3years
What are the clinical features of retinoblastoma?
White pupillary reflex instead of red
Squint
Ix for retinoblastoma
MRI + examine under anaesthetic
Tumours often multifocal
Tx for retinoblastoma
Depends on findings
- enuculation of eye
- chemo + radio
- laser treatment of retina
What are the different types of bone tumours?
Osteogenic sarcoma: more common
Ewing sarcoma: more often in younger children
Clinical features of bone tumours
Limbs most common site
Persistent, localised bone pain
Mass
Ix of bone tumours
Plain x-ray: destruction + variable periostel new bone formation MRI Bone scan Chest CT Bone marrow sampling
Mx of bone tumours
Chemo + surgery
Amputation
What is the most common soft tissue sarcoma in children?
rhabdomyosarcoma (originates from primitive mesenchymal tissue)
What are the clinical features of soft tissue sarcoma?
Head + neck
- proptosis, nasal obstruction or blood stained nasal discharge
Genitourinary tumours
- bladder, paratesticular structures or female genitourinary tract
- dysuria, urinary obstruction, scrotal mass, blood stained vaginal discharge
Mets
- lung, liver, bone, marrow
Ix of sarcoma
Biopsy
MRI
What are the types of malignant liver tumours?
liver tumours are rare!
Hepatoblastoma (65% - better prognosis)
Hepatocellular carcinoma (25%)
Clinical presentation of liver tumours
abdominal distension
mass
pain + jaundice (rare)
Ix of liver tumour
Elevated serum alpha fetoprotein (detected in nearly all heaptoblasoma and some hepatocellular)
Mx of liver tumour
Chemo, surgery, liver transplantaiton
What are germ cell tumours?
Arise from primitive germ cells which migrate from yolk sac endoderm to form gonads in the embryo. Can be benign or malignant.
Where are benign germ cell tumours usually found?
Sacrococcygeal region
Where are malignant germ cell tumours usually found?
Gonads
- v sensitive to chemo so good outcome
Ix for germ cell tumours
Serum markers alpha fetopretein and beta hCG confirm diagnosis
What is langerhans cell histiocytosis? (LCH)
A rare disorder characterised by an abnormal proliferation of histiocytes. A disorder of dendritic (antigen-presenting) cells.
How does LCH manifest?
bone lesions
- present at any age with pain, swelling or fracture
- xray: lytic lesion with well-defined border, often involving skull
diabetes insipidus
- may be assoc with skull disease, proptosis + hypothalamic infiltration
systemic LCH
- most aggressive form
- present in infancy with seborrheic rash and sub tissue involvement of gums, ears, lungs, liver, spleen, lymph nodes and bone marrow
