Curriculum standards

Knowledge guide

LG14: Oncological conditions

Key presentations and conditions

Advanced Trainees will have a comprehensive depth of knowledge of these presentations and conditions.

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Less common or more complex presentations and conditions

Advanced Trainees will understand these presentations and conditions.

Advanced Trainees will understand the resources that should be used to help manage patients with these presentations and conditions.

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Epidemiology, pathophysiology, and clinical sciences

Advanced Trainees will have a comprehensive depth of knowledge of the principles of the foundational sciences.

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Investigations, procedures, and clinical assessment tools

Advanced Trainees will know the scientific foundation of each investigation and procedure, including relevant anatomy and physiology. They will be able to interpret the reported results of each investigation or procedure.

Advanced Trainees will know how to explain the investigation or procedure to patients, families, and carers, and be able to explain procedural risk and obtain informed consent where applicable.

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Important specific issues

Advanced Trainees will identify important specialty-specific issues and the impact of these on diagnosis and management and integrate these into care.

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Presentations

Abdominal mass
Abnormal movement issues, such as:
- limp
Asymptomatic finding on surveillance investigations
Bowel / Bladder dysfunction
Cytopaenias, such as:
- anaemia
- pancytopenia
- thrombocytopenia
Hyperleukocytosis
Lymphadenopathy
Pain, such as:
- bone
- joint
Raised intracranial pressure
Significant family history
Skin lesions, such as:
- atypical rashes
- nodules
- petechiae
Spinal cord compression
Systemic symptoms:
- anorexia
- fever
- weight loss
Thoracic mass, such as:
- mediastinal
Tumour lysis syndrome

Conditions

Central nervous system tumours:
- atypical teratoid rhabdoid tumour
- choroid plexus carcinoma
- craniopharyngioma
- embryonal tumours of the central nervous system
- ependymoma
- high-grade glioma, including diffuse midline glioma
- intracranial germ cell tumour
- low grade glioma
- medulloblastoma
Leukaemia:
- acute lymphoblastic leukaemia
- acute myeloid leukemia and subtypes
- juvenile myelomonocytic leukaemia
- other rare leukaemia, such as:
  - acute undifferentiated leukaemia
  - chronic myeloid leukaemia
Lymphoma:
- Hodgkin
- non-Hodgkin
Non-central nervous system solid tumours:
- endocrine tumours:
  - adrenocortical
  - phaeochromocytoma
  - thyroid cancers
- germ cell tumours
- hepatoblastoma
- hepatocellular carcinoma
- histiocytic disorders, such as Langerhans cell histiocytosis
- metastasis
- nephroblastoma
- neuroblastoma and other peripheral nerve cell tumours
- neuroendocrine tumours of childhood
- renal cell carcinoma
- retinoblastoma
- sarcomas:
  - Ewing sarcoma / primitive neuroectodermal tumours
  - osteosarcoma
  - rhabdomyosarcoma
  - soft tissue sarcoma
  - undifferentiated sarcoma
Non-malignant haematological conditions:
- anaemia and polycythaemia
- bone marrow failure / dysfunction, such as aplastic anaemia
- haematologic and thrombotic emergency conditions
- haemolytic disorders, such as:
  - hemolytic uremic syndrome
  - thrombotic thrombocytopenic purpura
- haemophilia
- histiocytic disorders, such as hemophagocytic lymphohistiocytosis
- lymphopenia and lymphocyte subsets
- myelodysplasia and myeloproliferation
- neutropoenia and neutrophilia
- other common bleeding disorders, such as von Willebrand disease
Stem cell transplant and cellular therapies:

complications, such as:
- graft-versus-host disease (GvHD)
- sinusoidal obstruction syndrome (SOS)
- veno-occlusive disease (VOD)
conditioning regimens
donor lymphocyte infusion
donor selection
GvHD prophylaxis
indications

Thrombocytopaenia and thrombocytosis

For each presentation and condition, Advanced Trainees will know how to:

Synthesise

recognise the clinical presentation
identify relevant epidemiology, prevalence, pathophysiology, and clinical science
take a comprehensive clinical history
conduct an appropriate examination
establish a differential diagnosis
plan and arrange appropriate investigations
consider the impact of illness and disease on patients and their quality of life when developing a management plan

Manage

provide evidence-based management
prescribe therapies tailored to patients’ needs and conditions
recognise potential complications of disease and its management, and initiate preventative strategies
involve multidisciplinary teams

Consider other factors

identify individual and social factors and the impact of these on diagnosis and management

Conditions

Adult cancers presenting in childhood, such as:
- gastrointestinal tumours
- nasopharyngeal carcinomas
All paediatric cancers can present with non-specific and undifferentiated symptoms, and in atypical locations
Lymphoproliferative diseases and malignancies related to immunodeficiency and infection, such as post-transplant lymphoproliferative disorders
Non-malignant conditions that are treated by oncologists as may benefit from similar pathways, such as neurofibromatosis lesions
Other leukaemia, such as:
- chronic subtypes, such as chronic myeloid leukaemia
- other variants
Other rare solid tumours, such as rhabdoid
Premalignant haematological conditions, such as:
- myelodysplasia
- myeloproliferative disorders
Skin cancers, such as:
- Kaposi sarcoma
- melanoma

Presentations

Horner syndrome
Malignant bone fractures
Paraneoplastic syndromes, such as opsoclonus-myoclonus-ataxia syndrome
Pruritis
Skin nodules
Undifferentiated symptoms

For each presentation and condition, Advanced Trainees will know how to:

Synthesise

recognise the clinical presentation
identify relevant epidemiology, prevalence, pathophysiology, and clinical science
take a comprehensive clinical history
conduct an appropriate examination
establish a differential diagnosis
plan and arrange appropriate investigations
consider the impact of illness and disease on patients and their quality of life when developing a management plan

Manage

provide evidence-based management
prescribe therapies tailored to patients’ needs and conditions
recognise potential complications of disease and its management, and initiate preventative strategies
involve multidisciplinary teams

Consider other factors

identify individual and social factors and the impact of these on diagnosis and management

Anatomical biochemistry, cellular and molecular biology, genomics, immunology, pathology, and pharmacology of cancer, such as cancer immunology, including biologics and immunotherapy
Epidemiology, including (but not limited to) age, geographic, and sex distribution, incidence, and prevalence of cancers, along with predisposing factors, if known
Genetic disorders, genetics of malignancy, malignancy associations, and underlying syndromes
Pathophysiology of premalignant and malignant diseases, and non-malignant haematological diseases, including genetics and neoplasia development, and role of chronic inflammation and tumour microenvironment

Investigations

Appropriate aspirates, biopsies, and tissue samples:
- fine needle versus trucut versus excisional biopsies (image guided or open)
- importance of biobanking
- research or clinical trials samples
- role of second opinions and central review
- samples for anatomic pathology / morphology
- tissue samples (fresh / frozen) and their role in diagnosis / prognosis:
  - cytospin
  - fluorescence in situ hybridization / polymerase chain reaction
  - immunohistochemistry
  - molecular pathology and cytogenetics:
    - karyotyping and gene panel analysis
    - next-generation sequencing or whole exome sequencing
    - precision medicine programs
Cancer staging and risk stratification by tumour type in paediatrics
Complete history and examination
Establishing the diagnosis
Imaging and its relevance depending on cancer type:
- anatomical:
  - CT scan
  - MRI
  - plain film
  - ultrasound
- functional / nuclear medicine scans:
  - bone scans
  - metaiodobenzylguanidine
  - PET
Intent to cure and principles of management according to tumour risk, stage, and type, such as:
- local control measures, which may include surgery
- radiation
- systemic therapy
Other investigations:
- audiology assessment
- ECG
- electrocardiographic
- endoscopy
- fertility assessment and preservation
- formal renal function, such as:
  - diethylenetriaminepentaacetic acid
  - glomerular filtration rate
- lung function
Presentation of case at appropriate specialised multidisciplinary meetings
Relevant blood investigations
Special tests:
- alpha-fetoprotein
- beta human chorionic gonadotropin
- chromogranin A
- inflammatory markers, such as:
  - C-reactive protein (CRP)
  - erythrocyte sedimentation rate
- plasma metanephrines
- tumour burden markers, such as lactate dehydrogenase
- urine catecholamines

Procedures

Baseline height, nutritional assessment, physical activity level, and weight
Bone marrow aspiration and biopsy
Central line access
Fertility preservation
Lumber puncture and intrathecal chemotherapy administration
Omaya access

General management considerations

Advance care planning, such as referral to palliative care for patients with advanced stage disease, recurrent / relapsed disease, and known poorer outcomes
Consideration of stresses related to body changes and subsequent altered social and peer interactions due to cancer and treatment, such as amputation, hair loss, and weight changes
Goals of therapy
Multidisciplinary care in management and treatment
Shared care with local practitioners for patients not living close to main treatment centres
Special considerations and communication related to patients’ age, developmental stage, and psychosocial circumstances
Other considerations, such as:
- drug levels and toxicity monitoring
- drug sensitivity testing, such as thiopurine methyltransferase genotype for thiopurine
- enrolment in appropriate clinical trials if appropriate, such as precision medicine programs
- genetic testing, including for predisposition syndromes as appropriate
- multidisciplinary team discussions of all new diagnoses
- pharmacogenomics testing
- precision medicine assessment techniques, and multidisciplinary curation of results
- prophylactic treatment as indicated, such as immunoglobulin
- quality of life and patient-reported symptom measures
- rehabilitation
- tumour surveillance post-treatment and in at-risk populations, such as:
  - genetic susceptibility
  - immunodeficiency
  - post-infectious
  - syndromes
Unique requirements of adolescent and young adult population