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Paediatrics STG > Paediatric Oncology > Flashcards

Paediatric Oncology Flashcards

1
Q

Malignancy in General in Children

A

• 1 in 500 children develop cancer by 15yrs age; Leukaemia affects children at all ages, although there is an early childhood peak; Neuroblastoma and Wilms tumour almost always seen at first 6yrs of life
o Leukaemia is the most common malignancy, followed by Brain Tumours
• Hodgkin Lymphoma, Bone Tumours peak incidence at Adolescence and Early Adult Life

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2
Q

5 yr survival rate

A

Overall, 5yr Survival
about 75% for all forms of cancer; Most curative
o Improved Expectancy attributed mainly to Multiagent Chemotherapy, Supportive Care and Specialist MDT managemen

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3
Q

Malignancy in General in Children: Aetiology

A

Unclear; Most likely environmental factors and genetic susceptibility; Usually Sporadic but may be inherited, but specific gene mutation is unknown
o E.g. Bilateral Retinoblastoma due to mutated RB gene (Ch 13)
o Can also be associated with Syndromes (Down, Neurofibromatosis)
o Can be associated with Infection – EBV (Burkitt, Hodgkin, NPC), HBV (Hepatocellular Ca), HIV (Kaposi Sarcoma)

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4
Q

Malignancy in General in Children: Presentation

A

Can present as a Localised Mass, Consequences of Disseminated Disease (E.g. Bone Marrow Infiltration), Mass Effect on Neighbouring Structures or Tissues

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5
Q

Malignancy in General in Children: Investigation

A

Radiology (Anatomical, Functional/Nuclear Medicine); Tumour marker studies for specific malignancies (Urinary Catecholamines for Neuroblastoma, Alpha-Fetoprotein for Germ Cell and Liver Tumours)

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6
Q

Malignancy in General in Children:Pathology

A

BM Aspirations for Leukaemias, Biopsy for most Solid Tumours; IHC for differentiate tumour types; Confirmation of Diagnosis and Prognostication

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7
Q

Malignancy in General in Children: Prognosis

A

Survival suggests Teenagers and Young Adults have poorer outcomes than Children`

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8
Q

Malignancy in General in Children: Chemotherapy

A

Primary Curative, Neoadjuvant, Adjuvant
o Limited by risk of Irreversible Damage to normal tissues, particularly BM; BM Transplant can be used post-intensive chemotherapy as a Rescue therapy
o Allogenic or Autologous Transplant; Allogenic Transplant principally used in management of High-risk, or Relapsed Leukaemia; Autologous Transplant may be used in treatment with Solid Tumours where prognosis is poor with conventional

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9
Q

Malignancy in General in Children: Targeted Therapy

A

Tyrosine Kinase inhibitors, Monoclonal Antibodies

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10
Q

Malignancy in General in Children: Radiotherapy

A

Electron Beam, Proton Beam, Intravenous therapy (e.g. Radioactive Iodine)
o Risk to damage higher in children due to growing tissues
o Adequate protection, optimised positioning and immobilisation
o Cranial RT for children under 3yrs associated with severe Neurocognitive damage

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11
Q

Malignancy in General in Children: Surgery

A

Frequently in multiple stages; Biopsy, more extensive surgery typically occurs after Neoadjuvant treatments

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12
Q

Complications from Treatment: Infection

A

• Infection secondary to Immunosuppression – Fever and Neutropaenia should be managed as Neutropaenia sepsis unless proven
o Opportunistic organisms include PCP, Disseminated Fungal, CoNS
o Atypical presentation of viral infections (Measles, VZV) may be life-threatening; Prompt administration of IVIg, VZIg may confer some protection
o Live Vaccines during CTx up to 6-12/12 contraindicated

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13
Q

Complications from Treatment: Bone Marrow Suppression

A

Anaemia requiring Transfusion, Thrombocytopaenia

requiring blood product support; May be part of Rescue

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14
Q

Complications from Treatment: GI Effects

A

Mouth ulcers (Common, painful, severe; might limit feeding), Nausea, Vomiting (Only partially prevented by Antiemetic drugs)
o Can result in significant nutritional compromise
o Gut mucosal damage causes diarrhoea, may predispose to Gram negative infections

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15
Q

Complications from Treatment: Drug-specific Side Effects

A

Cardiotoxicity (Doxorubicin), Renal Failure and Otoxicity (Cisplatin), Haemorrhagic Cystitis (Cyclophosphamide), Neuropathy (Vincristine)

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16
Q

Complications from Treatment:Fertility

A

Preservation techniques e.g. Moving organ out of radiotherapy field, Sperm banking, Cryopreservation of Ovarian Cortical tissue

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17
Q

Acute Leukaemia

A

• ALL accounts for 80% Leukaemias in Children; Remainder mostly AML, ANLL; CML and other Myeloproliferative Disorders are rare

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18
Q

Acute Lymphoblastic Anaemia

A

• ALL incidence peaks 2-5yrs; Presents with symptoms from Disseminated Disease from Marrow Infiltration and other organs; Insidious over several weeks

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19
Q

Acute Lymphoblastic Anaemia: Clinical Features

A

Anaemia, Thrombocytopaenia, Evidence of circulating Leukaemic Blast Cells
o Marrow Examination essential to confirm Diagnosis, Identify
Immunological and Cytogenetic features; Prognostication
o Clotting Screen – 10% of patients with Acute Leukaemia have DIC at diagnosis; Haemorrhagic or Thrombotic Complications
o LP – Identify disease in CSF; CXR – Mediastinal Mass characteristic of T-cell disease

20
Q

Management of ALL: Prognosis

A

Prognosis based on Age, WBC Count at presentation, Cytogenetics of Leukaemic Cells, Rate and Completeness of Response to Treatment

21
Q

Management of ALL: Before Starting Treatment

A

Correct Anaemia and Thrombocytopaenia, Treat infections; Hydration and Prophylaxis against Lysis Syndrome (Allopurinol, or Urase Oxidase if higher risk); PCP Prophylaxis with Co-Trimoxazole

22
Q

Management of ALL: Induction Chemotherapy

A

Induction Chemotherapy – Combination Chemotherapy and Steroids; Remission in 95%
o Aims to eradicate Leukaemic Blasts and Restore Normal Marrow Function

23
Q

Management of ALL: Intensification

A

Given to consolidate remission; Improves cure rate at the expense of toxicity

24
Q

Management of ALL: CNS Treatment

A

Intrathecal CTx; Additional Induction doses if evidence of disease

25
Q

Management of ALL: Maintenance

A

Up to 3yrs from Diagnosis

26
Q

Management of ALL: Relapse

A

Relapse – High Dose Chemotherapy ± Total Body Irradiation with BM Transplant

27
Q

Brain Tumours in Children

A

In children, almost always Primary Brain Tumours; 60% Infratentorial; Most common Solid Tumour in children, Leading cause of Childhood Cancer deaths

28
Q

Causes of brain tumours in children

A 
o Astrocytoma (40%) varying from Benign to Glioblastoma Multiforme
o Medulloblastoma (20%) – Arises from Midline of Posterior Fossa; 20% have Spinal Mets due to speeding via CSF
o Ependymoma (~8%) – Mostly Posterior Fossa; Behaves like Medulloblastoma
o Brainstem Glioma (6%) – Malignant; Poor prognosis
o Craniopharyngioma (4%) – Arises from Squamous Remnant of Rathke pouch; Not truly malignant, but locally invasive and grows to Suprasellar region
29
Q

Presentation of Brain Tumour

A

Presents as Focal Neurological symptoms, or effects of Raised ICP; Spinal tumours can present with Back Pain, Peripheral Weakness, Incontinence depending to level of lesion
o Supratentorial/Cortical – Seizures, Hemiplegia, Focal Neurology
o Midline – Visual Field Defects, Pituitary Disorders
o Cerebellar and Fourth Ventricle – Truncal Ataxia, Coordination Difficulties
o Brainstem – CN Defects, Pyramidal Tract signs, Cerebellar Signs
o Persistent Back Pain in Children warrants MRI Investigations

30
Q

Brain Tumours in Children: Management

A

MRI and LP for staging; LP contraindicated if raised ICP suspected
Surgery is first line for operable tumours – Treating Hydrocephalus, Tissue Diagnosis and Tumour Debulking; Radiotherapy and Chemotherapy depending on tumour type

31
Q

Hodgkin Lymphoma: Presentation

A

More common in adolescents; Painless Lymphadenopathy (Most frequently in the neck); Firm, Larger LN, may obstruct neighbouring organs
o Long history of systemic symptoms (Sweating, Weight Loss, Fever, Pruritus) are uncommon even in advanced disease

32
Q

Hodgkin Lymphoma: Management

A

LN Biopsy, Radiological staging and BM Biopsy
o Combination Chemotherapy ± Radiotherapy; PET scanning to monitor treatment response, and guide further management
o 80% Cure rate; Even in Disseminated disease, 60% Cure

33
Q

Non Hodgkin Lymphoma: Presentation

A

T-cell Malignancies can present either as Leukaemia (15% of ALL) or NHL; Mediastinal Mass with varying degrees of BM Infiltration
o Mediastinal mass might cause SVC Obstruction
• B-cell Malignancies present more commonly as NHL with localised LN disease in Head and Neck or Abdomen (Palpable Mass, Obstruction or even Intussusception

34
Q

Non Hodgkin Lymphoma: Management

A

Investigations involve Biopsy, Radiology of Nodal Sites, BM Examination and LP
• Managed with Combination Chemotherapy; >80% Survival rate

35
Q

Burkitt’s Lymphoma

A

B-cell NHL; Endemic (most common in EBV-infected regions), Sporadic, or Immunodeficiency Related (HIV Infection, or Immunosuppression post-transplant)

36
Q

Neuroblastoma

A

• Arise from Neural Crest Tissue in Adrenal Medulla and Sympathetic Nervous System; Sometimes spontaneous regression can occur in very young patients

37
Q

Neuroblastoma Presentation

A

Abdominal Mass in most, but tumour can be anywhere along Sympathetic chain in Neck to Pelvis; Paravertebral tumours can invade adjacent Intervertebral Foramen, causing Cord Compression; Symptoms include Bone Pain, BM Suppression, Weight Loss, Malaise, Hepatomegaly

38
Q

Neuroblastoma Investigations

A

Raised Urinary Catecholamine Metabolites (VMA, HVA); Confirmatory Biopsy, BM Examination, MIBG Scanning

39
Q

Neuroblastoma Management

A
  • Surgery can be curative for Localised Primary without Metastatic Disease
  • Metastatic disease in older children – Chemotherapy (High dose with Stem Cell Rescue), Surgery and Radiotherapy; High risk of relapse, ~40% Cure
  • Immunotherapy, Maintenance therapies in development in High-risk Disease
40
Q

Wilms Tumour: Nephroblastoma

A

Arises from Embryonal Renal Tissue; Most common Renal Tumour of Childhood; >80% present before 5yrs, rarely seen >10yrs age

41
Q

Wilms Tumour: Presentation

A

Large Abdominal mass, often found incidentally in otherwise well child; Haematuria, Abdominal Pain, Anorexia, Anaemia or Hypertension can also be present

42
Q

Wilms Tumour: Investigations

A

Ultrasound, CT/MRI show Intrinsic Renal Mass distorting normal structure; Staging assesses Metastases (If present, Typically Lung), Initial Tumour Resectability and Contralateral Kidney

43
Q

Wilms Tumour: Management

A

Initial Chemotherapy followed by Delayed Nephrectomy with Histological assessment for further treatment; Radiotherapy for advanced disease
o 5% have Bilateral disease; Careful management to preserve renal function
• Good prognosis, 80% Cure Rate; 60% for Metastatic disease (15% of Nephroblastoma) but relapse has poor prognosis

44
Q

Sarcoma

A

Connective Tissues such as Muscle and Bone; Rhabdomyosarcoma most common type of Soft Tissue Sarcoma; Arises from Primitive Mesenchymal Tissue, Most commonly in Head and Neck; Metastatic disease in 15% of patients at presentation
o Biopsy, Full radiological examination; Surgery is challenging due to misleading tumour margins, and should not be attempted unless clear
o 65% Overall Cure

45
Q

Bone Tumours

A

Uncommon before Puberty; Osteosarcoma > Ewing Sarcoma, but Ewing seen more in younger children; Male preponderance
o Limbs most common; Persistent Localised Bone Pain preceding Mass
o Plain film XR, MRI, Bone Scan; CT chest for Lung Mets, BM sampling
o Neoadjuvant Combination Chemotherapy; Radiotherapy can be used in Ewing; Amputation can sometimes be avoided by En-bloc Resection plus Prosthesis

46
Q

Retinoblastoma

A

Malignant tumour of Retinal cells; 5% of Severe Visual Impairment; All Bilateral and 20% Unilateral due to RB mutations (Ch 13; AD with incomplete penetrance)
o Most present within first years of life; Children from affected families screened
o Most common presentation by detection of White Reflex, or with Squint
o MRI, Examination under Anaesthetic; Enucleation of the eye for more advanced disease; Local laser treatment ± Neoadjuvant Chemo
o Significant risk of other malignancy (especially Sarcoma) among survivors of Hereditary Retinoblastoma

47
Q

Liver Tumours

A 
Liver tumours (Hepatoblastoma 65%, Hepatocellular Carcinoma 25%); Abdominal Distention or mass; Pain and Jaundice rare; Elevated AFP in nearly all HB and some HCC
o Chemotherapy, Surgery; Liver Transplant if Inoperable

Paediatrics STG (17 decks)

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