STEP 3: Diagnosis, staging and treatment planning
Step 3 outlines the process for confirming the diagnosis and stage of cancer and for planning subsequent treatment. The guiding principle is that interaction between appropriate multidisciplinary team members should determine the treatment plan.
The treatment team, after taking a thorough medical history and making a thorough medical examination of the patient, should undertake the following investigations under the guidance of a specialist.
Diagnostic evaluation is required for two complementary purposes – first, to establish a precise diagnosis according to the most recent classification system, and second, to assess the presence and management of comorbidities and the patient’s fitness because these affect both response to treatment and toxicity from treatment.
To achieve this, the treatment team should:
- perform a thorough physical examination, including assessing for the presence of extramedullary disease (e.g. leukaemia cutis, gum infiltration and/or central nervous system symptoms)
- undertake the following investigations under the guidance of a specialist:
- peripheral blood tests
- bone marrow aspirate (BMA)
- trephine biopsy +/- lumbar puncture, imaging or tissue biopsy when extramedullary disease is suspected.
Note: Details of specific tests appear below in section 3.2.
Pathology specimens should be collected and reviewed by a pathologist with expertise in diagnosing AML, before a treatment plan is instituted.
Where safe and timely to do so, it is preferable that the diagnostic blood tests and bone marrow biopsy are performed at the specialist treatment centre. This will facilitate review of blood and bone marrow results by the specialist management team and ensure all necessary tests are conducted.
Morphological assessment to identify APL should be conducted immediately and the result conveyed to the treating physician as soon as possible.
For all patients with AML, other results necessary for immediate management decisions should be available within 72 hours of the patient presenting.
Specialised testing is performed to:
- ensure accurate diagnoses
- accurately sub-classify AML
- inform prognosis
- inform treatment decisions that are evidence-based.
Classification is the principle process by which key information is collated to inform prognosis and management of patients with AML.
Classification and risk stratification for AML involves these tests:
- morphological assessment
- cytogenetics
- flow cytometry
- molecular pathology (genetic testing).
AML is classified according to the World Health Organization’s classification of AML tumours (Arber et al. 2016). The European LeukemiaNet stratification system classifies patients as having favourable, intermediate and adverse risk based on karyotype, selected molecular abnormalities (currently FLT3, NPM1, TP53, ASXL1, RUNX1 and CEBPA mutation status) (Döhner et al. 2017).
The other most important prognostic features are age at diagnosis, performance status, presence of extramedullary disease, hyperleukocytosis, therapy-related AML (previous exposure to cytotoxics), presence of an antecedent bone marrow failure syndrome and response to induction chemotherapy.
Newer molecular markers with prognostic and therapeutic relevance in AML are likely to become clinically routine in the near future (Grimwade et al. 2016).
Most genetic abnormalities in AML only occur in abnormal blood cells and are not related to genetic abnormalities that affect the whole body and/or are inherited. However, heritable genetic abnormalities may be identifiable in a very small number of patients. AML with a genetic predisposition is an entity in the World Health Organization classification, and most diagnostic centres have access to identification of heritable genetic abnormalities related to leukaemia. This becomes significantly relevant if a family member is being considered as a stem cell donor.
It is important to evaluate and document relevant organ functions (e.g. respiratory, cardiac, hepatic, renal) and physiological robustness using validated assessment tools for all patients, especially older patients (Sorror et al. 2017).
Careful clinical and haematological assessment is required to identify patients in whom the start of chemotherapy could or should be delayed. The presence of an active infection at diagnosis is important to identify.
In addition to a clinical examination, the following investigations/procedures are recommended:
- coagulation status to detect leukemia-related coagulopathy (Döhner et al. 2017)
- MRI brain +/– lumbar puncture if central nervous system involvement is suspected
- CT/PET scan to help assess for extramedullary disease where this is clinically suspected
- cardiac investigation including an ECHO or a gated heart pool scan in patients being considered for induction therapy
- human leukocyte antigen (HLA) typing and HLA antibody screening at diagnosis in patients being considered for induction therapy.
Each unit should have a policy about if, and when, HLA typing of available first- and second-degree family members should occur. This policy should be agreed with the allo-SCT unit to which referrals are usually directed.
In patients with adverse or intermediate risk disease, early allo-SCT should be considered (Döhner et al. 2010) and, therefore, a donor search should be carried out as early as possible in accordance with agreed policies of the allo-SCT unit to which referrals are usually directed.
Patient performance status is a central factor in cancer care and should be clearly documented in the patient’s medical record.
Performance status should be measured and recorded using an established scale such as the Karnofsky scale or the Eastern Cooperative Oncology Group (ECOG) scale.
Because of the urgency and complexity of treatment, every clinical haematology unit should have predefined, peer-reviewed treatment models of care that have been endorsed by the multidisciplinary team. Assessment of the premorbid state is an essential component of the treatment planning process.
Prevention and management of infections in AML include the following:
- All patients should undergo screening for infections at high risk of reactivation or transmission before beginning treatment.
- Some infections are determined by epidemiological risk of exposure and history of recent travel and/or extended habitation in high-risk countries.
- Minimum requirements would include cytomegalovirus, hepatitis B, hepatitis C and HIV screening. Other tests such as for tuberculosis, strongyloides serology, and screening for multidrug-resistant pathogens as per institutional policy, should be considered.
- Patients with antibiotic allergy labels should have suspected allergies reassessed where possible.
- All institutions should have empiric sepsis guidelines/pathways that include appropriate recommendations for the initial management of neutropenic fever. Specialists in infectious diseases may be required for advice about duration and appropriate antibiotics based on pathogens isolated and patient factors (allergy, renal impairment).
- Vaccination status should be assessed for all patients. Vaccination with influenza and Streptococcus pneumoniae can recommence after three months if in complete remission.
- Prophylaxis guidelines for fungal and viral infections should accord with published national guidelines.
A number of factors should be considered at this stage:
- the patient’s overall condition, life expectancy, personal preferences and decision- making capacity
- discussing the multidisciplinary team approach to care with the patient
- appropriate and timely referral to an MDM
- pregnancy and fertility
- support with travel and accommodation
- teleconferencing or videoconferencing as required.
Induction treatment is often required before a full MDM ratifies details of the ongoing management plan (which should include full details of the response assessment). Most patients will receive their initial treatment as inpatients, allowing their initial multidisciplinary treatment planning to be established on the ward. For patients undergoing induction chemotherapy, presentation to, and consideration within, an MDM is most important once the outcome of the induction therapy is known. At this point, a review of the patient is required to inform further management and supportive care needs.
For patients not eligible for induction chemotherapy, or where uncertainty of the approach exists, a review at an MDM should occur as soon as practicable (before definitive treatment), to establish the recommended treatment plan and all aspects of supportive care, including early preparation for the post-treatment phase.
The level of discussion may vary, depending on the patient’s clinical and supportive care factors. Some patients with non-complex cancers may not be discussed by a multidisciplinary team; instead the team may have treatment plan protocols that will be applied if the patient’s case (cancer) meets the criteria. If patients are not discussed at an MDM, they should at least be named on the agenda for noting. The proposed treatment must be recorded in the patient’s medical record and should be recorded in an MDM database where one exists.
Teams may agree on standard treatment protocols for non-complex care, facilitating patient review (by exception) and associated data capture.
Results of all relevant tests and access to images should be available for the MDM. Information about the patient’s concerns, preferences and social and cultural circumstances should also be available.
The multidisciplinary team requires administrative support in developing the agenda for the meeting, for collating patient information and to ensure appropriate expertise around the table to create an effective treatment plan for the patient. The MDM has a chair and multiple lead clinicians. Each patient case will be presented by a lead clinician (usually someone who has seen the patient before the MDM). In public hospital settings, the registrar or clinical fellow may take this role. A member of the team records the outcomes of the discussion and treatment plan in the patient history and ensures these details are communicated to the patient’s general practitioner. The team should consider the patient’s values, beliefs and cultural needs as appropriate to ensure the treatment plan is in line with these. There may be early consideration of post-treatment pathways at this point – for example, shared follow-up care.
The multidisciplinary team should be composed of the core disciplines that are integral to providing good care. Team membership should reflect both clinical and supportive care aspects of care. Pathology expertise is essential.
See ‘About this OCP’ for a list of team members who may be included in the multidisciplinary team for AML.
Core members of the multidisciplinary team are expected to attend most MDMs either in person or remotely via virtual mechanisms. Additional expertise or specialist services may be required for some patients. An Aboriginal and Torres Strait Islander cultural expert should be considered for all patients who identify as Aboriginal or Torres Strait Islander.
The general practitioner who made the referral is responsible for the patient until care is passed to another practitioner who is directly involved in planning the patient’s care.
The general practitioner may play a number of roles in all stages of the cancer pathway including diagnosis, referral, treatment, shared follow-up care, post-treatment surveillance, coordination and continuity of care, as well as managing existing health issues and providing information and support to the patient, their family and carer.
A nominated contact person from the multidisciplinary team may be assigned responsibility for coordinating care in this phase. Care coordinators are responsible for ensuring there is continuity throughout the care process and coordination of all necessary care for a particular phase (COSA 2015). The care coordinator may change over the course of the pathway.
The lead clinician is responsible for overseeing the activity of the team and for implementing treatment within the multidisciplinary setting.
Participation in clinical trials, registries and tissue banking, where available, is considered a standard of care for patients with AML. Cross-referral between clinical trials centres should be encouraged to facilitate participation.
For more information visit:
Cancer prehabilitation uses a multidisciplinary approach combining exercise, nutrition and psychological strategies to prepare patients for the challenges of cancer treatment such as surgery, systemic therapy and radiation therapy. Team members may include anaesthetists, oncologists, surgeons, haematologists, clinical psychologists, exercise physiologists, physiotherapists and dietitians, among others.
Patient performance status is a central factor in cancer care and should be frequently assessed. All patients should be screened for malnutrition using a validated tool, such as the Malnutrition Screening Tool (MST). The lead clinician may refer obese or malnourished patients to a dietitian preoperatively or before other treatments begin.
Patients who currently smoke should be encouraged to stop smoking before receiving treatment. This should include an offer of referral to Quitline in addition to smoking cessation pharmacotherapy if clinically appropriate.
Evidence indicates that patients who respond well to prehabilitation may have fewer complications after treatment. For example, those who were exercising before diagnosis and patients who use prehabilitation before starting treatment may improve their physical or psychological outcomes, or both, and this helps patients to function at a higher level throughout their cancer treatment (Cormie et al. 2017; Silver 2015).
For patients with AML, the multidisciplinary team should consider these specific prehabilitation assessments and interventions for treatment-related complications or major side effects:
- conducting a physical and psychological assessment to establish a baseline function level
- identifying impairments and providing targeted interventions to improve the patient’s function level (Silver & Baima 2013)
- reviewing the patient’s medication to ensure optimisation and to improve adherence to medicine used for comorbid conditions.
Following completion of primary cancer treatment, rehabilitation programs have considerable potential to enhance physical function.
Cancer and cancer treatment may cause fertility problems. This will depend on the age of the patient, the type of cancer and the treatment received. Infertility can range from difficulty having a child to the inability to have a child. Infertility after treatment may be temporary, lasting months to years, or permanent (AYA Cancer Fertility Preservation Guidance Working Group 2014).
Patients need to be advised about and potentially referred for discussion about fertility preservation before starting treatment and need advice about contraception before, during and after treatment. Patients and their family should be aware of the ongoing costs involved in optimising fertility. Fertility management may apply in both men and women. Fertility preservation options are different for men and women. Fertility preservation procedures may not always be feasible in patients with newly diagnosed AML, which can require immediate chemotherapy. The need for ongoing contraception applies to both men and women.
The potential for impaired fertility should be discussed and reinforced at different time points as appropriate throughout the diagnosis, treatment, surveillance and survivorship phases of care. These ongoing discussions will enable the patient and, if applicable, the family to make informed decisions. All discussions should be documented in the patient’s medical record.
See the Cancer Council website for more information.
Rehabilitation may be required at any point of the care pathway. If it is required before treatment, it is referred to as prehabilitation (see section 3.6.1).
All members of the multidisciplinary team have an important role in promoting rehabilitation. Team members may include occupational therapists, speech pathologists, dietitians, social workers, psychologists, physiotherapists, exercise physiologists and rehabilitation specialists.
To maximise the safety and therapeutic effect of exercise for people with cancer, all team members should recommend that people with cancer work towards achieving, and then maintaining, recommended levels of exercise and physical activity as per relevant guidelines. Exercise should be prescribed and delivered under the direction of an accredited exercise physiologist or physiotherapist with experience in cancer care (Vardy et al. 2019). The focus of intervention from these health professionals is tailoring evidence-based exercise recommendations to the individual patient’s needs and abilities, with a focus on the patient transitioning to ongoing self-managed exercise.
Other issues that may need to be dealt with include managing cancer-related fatigue, improving physical endurance, achieving independence in daily tasks, optimising nutritional intake, returning to work and ongoing adjustment to cancer and its sequels. Referrals to dietitians, psychosocial support, return-to-work programs and community support organisations can help in managing these issues.
The lead or nominated clinician should take responsibility for these tasks:
- discussing treatment options with patients and carers, including the treatment intent and expected outcomes, and providing a written version of the plan and any referrals
- providing patients and carers with information about the possible side effects of treatment, managing symptoms between active treatments, how to access care, self-management strategies and emergency contacts
- encouraging patients to use question prompt lists and audio recordings, and to have a support person present to aid informed decision making
- initiating a discussion about advance care planning and involving carers or family if the patient wishes.
See validated screening tools mentioned in Principle 4 ‘Supportive care’.
A number of specific challenges and needs may arise for patients at this time:
- assistance for dealing with psychological and emotional distress while adjusting to the diagnosis; treatment phobias; existential concerns; stress; difficulties making treatment decisions; anxiety or depression or both; psychosexual issues such as potential loss of fertility and premature menopause; history of sexual abuse; and interpersonal problems
- management of physical symptoms such as pain and fatigue (Australian Adult Cancer Pain Management Guideline Working Party 2019)
- malnutrition or undernutrition, identified using a validated nutrition screening tool such as the MST (note that many patients with a high BMI [obese patients] may also be malnourished [WHO 2018])
- patients with a high or very high body mass index should have chemotherapy dosed as per agreed upon guidelines (Griggs et al. 2012) – specialty pharmacy advice may also be required concerning dosing of other pharmaceuticals in this patient group
- access to peer support
- support for families or carers who are distressed with the patient’s cancer diagnosis
- support for families/relatives who may be distressed after learning of a genetically linked cancer diagnosis
- specific spiritual needs that may benefit from the involvement of pastoral/spiritual care.
Additionally, palliative care may be required at this stage.
For more information on supportive care and needs that may arise for different population groups, see Appendices A and B, and special population groups.
In discussion with the patient, the lead clinician should undertake the following:
- establish if the patient has a regular or preferred general practitioner and if the patient does not have one, then encourage them to find one
- provide written information appropriate to the health literacy of the patient about the diagnosis and treatment to the patient and carer and refer the patient to the Guide to best cancer care (consumer optimal care pathway) for AML, as well as to relevant websites and support groups as appropriate
- offer all patients of childbearing years undergoing allo-SCT the opportunity of preserving their fertility prior to treatment (referral to fertility counselling may be appropriate as discussed in section 3.6.2)
- provide a treatment care plan including contact details for the treating team and information on when to call the hospital
- discuss a timeframe for diagnosis and treatment with the patient and carer
- discuss the benefits of multidisciplinary care and gain the patient’s consent before presenting their case at an MDM
- provide brief advice and refer to Quitline (13 7848) for behavioural intervention if the patient currently smokes (or has recently quit), and prescribe smoking cessation pharmacotherapy, if clinically appropriate
- recommend an ‘integrated approach’ throughout treatment regarding nutrition, exercise and minimal or no alcohol consumption among other considerations
- communicate the benefits of continued engagement with primary care during treatment for managing comorbid disease, health promotion, care coordination and holistic care
- where appropriate, review fertility needs with the patient and refer for specialist fertility management (including fertility preservation, contraception, management during pregnancy and of future pregnancies)
- be open to and encourage discussion about the diagnosis, prognosis (if the patient wishes to know) and survivorship and palliative care while clarifying the patient’s preferences and needs, personal and cultural beliefs and expectations, and their ability to comprehend the communication
- encourage the patient to participate in advance care planning including considering appointing one or more substitute decision-makers and completing an advance care directive to clearly document their treatment preferences. Each state and territory has different terminology and legislation surrounding advance care directives and substitute decision-makers.
The lead clinician has these communication responsibilities:
- involving the general practitioner from the point of diagnosis
- ensuring regular and timely communication with the general practitioner about the diagnosis, treatment plan and recommendations from MDMs and inviting them to participate in MDMs (consider using virtual mechanisms)
- supporting the role of general practice both during and after treatment
- discussing shared or team care arrangements with general practitioners or regional cancer specialists, or both, together with the patient.
Refer to Principle 6 ‘Communication’ for communication skills training programs and resources.