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.
A definitive tissue diagnosis is always required before initiating lymphoma-specific treatment. Biopsy may have been performed before referral. Both fresh and fixed tissue samples should be collected from the tissue biopsy for anatomical pathology and potentially flow cytometry, cytogenetics and gene mutation testing (e.g. TP53 mutation testing for MCL). Additional immunohistochemical stains may in the future provide information that affects therapy (e.g. Ki67 for FL).
Lymph node architecture is a key part of the pathological examination and therefore excisional biopsy is the preferred biopsy method where possible. Where excision biopsy or large incisional biopsy is not possible, a core needle biopsy that samples the maximum number of cores with the largest calibre possible should be considered (NICE 2016).
FNA is not suitable for diagnosing lymphomas (Armitage et al. 2017). However, FNA may be an appropriate investigation in circumstances where excision or needle core biopsy is overly invasive or unfeasible (e.g. mediastinal disease). Construction of tissue blocks from cytological samples may aid diagnosis.
For low-grade lymphomas, surgery is a diagnostic procedure and rarely therapeutic. Therefore, the least invasive surgical method is recommended for diagnosis. If a highly invasive or extensive surgical procedure is being considered, consultation with the lymphoma multidisciplinary team is required.
Pathology specimens should be reviewed by a pathologist with expertise in diagnosing low-grade lymphomas. This should be done at the treatment centre conducting the MDM, before a treatment plan has been instituted.
Additional blood tests – B2M, immunoglobulins, serum protein electrophoresis – are part of the diagnostic work-up.
Evaluate relevant organ function based on history, clinical examination and, where appropriate, laboratory or imaging investigations (cardiac, respiratory, renal, hepatic). Autoimmune screens are sometimes warranted in MZL, specifically autoimmune haemolytic anaemia.
Test for occult or latent infections (e.g. HIV, hepatitis B and C, tuberculosis) that may be affected by therapy or pose a risk of reactivation due to the immunosuppressive effects of treatment. For MZL, site-specific infectious disease testing should be performed.
After receiving a referral for suspected lymphoma, the referral should be triaged accordingly, on the basis of presence of indicators of concern such as B symptoms or organ dysfunction. The timing of diagnostic investigations should be guided by the initial severity of symptoms. Staging (see 3.2 Staging and prognostic assessment) should be completed within four weeks.
Currently there are no genetic tests applicable to predict family risk of low-grade lymphomas.
Staging is a critical element in treatment planning and should be clearly documented in the patient’s medical record.
A number of lymphoma prognostic indices have been developed for the different low-grade lymphoma entities and are documented in the relevant guidelines (BSH 2020; Cheah et al., 2019; Dreyling et al. 2017; 2020). It is appropriate to document the relevant low-grade lymphoma prognostic indices at diagnosis, which may assist in the therapeutic approach.
The disease stage based on the 2014 Lugano classification (Cheson 2014) should be determined in all patients according to evidence-based guidelines.
Staging and prognostic assessment for low-grade lymphomas involves the following:
- Clinical examination and history for assessment of B It is particularly important to document the sites and extent of FL involvement that are not easily visible on imaging (e.g. skin, conjunctiva)
- Imaging with PET-CT should be performed for the staging of indolent lymphomas, in particular FL and PET-CT has greater sensitivity than CT in detecting nodal and extranodal involvement and is valuable in identifying true stage I disease amenable to radiation therapy.
Some cases of MZL exhibit low positivity on PET-CT scanning and in these instances subsequent imaging with CT may be preferable
- The decision to do a bone marrow biopsy should be according to evidence-based guidelines for the specific A bone marrow aspirate and trephine is an element of complete staging of most low-grade lymphomas. However, it may not be necessary at diagnosis where the planned initial approach is watch and wait, nor before starting treatment if the results before and/or after treatment will not impact on prognosis or therapeutic approach. The purpose of the bone marrow biopsy should be clearly explained to the patient, especially when performed in a clinical trial where it may not otherwise have been indicated
- For MCL, symptoms that suggest gastrointestinal involvement should be investigated with gastroscopy and colonoscopy
- Endoscopic ultrasound for gastric MZL can be used to characterise gastric wall infiltration and peri-gastric lymph node involvement
- MRI imaging is of value in orbital/ocular adnexa MZL
- Additional tests are recommended to calculate the relevant prognostic scores for each low-grade
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.
Performance status should be recorded for both presentation and pre-morbid assessment. Consider using a validated geriatric assessment tool.
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
- additional tests to calculate the relevant prognostic scores for each low-grade lymphoma
- evaluation of relevant organ function based on history, clinical examination and, where appropriate, laboratory or imaging investigations (cardiac, respiratory, renal, hepatic)
- autoimmune screens (these are sometimes warranted in MZL, specifically autoimmune haemolytic anaemia)
- tests for occult infections (e.g. HIV, hepatitis B and C, tuberculosis) that may be affected by therapy or pose a risk of reactivation due to the immunosuppressive effects of For MZL, site-specific infectious disease testing should be performed.
The multidisciplinary team should meet to discuss newly diagnosed patients before definitive treatment so that a treatment plan can be recommended and there can be early preparation for the post-treatment phase. The level of discussion may vary, depending on the patient’s clinical and supportive care factors. The proposed treatment must be recorded in the patient’s medical record and should be recorded in an MDM database where one exists.
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.
Specifically in low-grade lymphomas, ‘watch and wait’, the dynamic observation of asymptomatic disease, can lead to significant anxiety. The patient’s anxiety can be alleviated with education from the treating specialist and counselling from a psychologist with experience in lymphoma.
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])
- 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
For more information on supportive care and needs that may arise for different population groups, see Appendices A, B and C.
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 and radiology expertise are essential.
See Appendix E for a list of team members who may be included in the multidisciplinary team for low-grade lymphomas.
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.
Patients should be encouraged to participate in research or clinical trials where available and appropriate.
Participation in clinical trials, patient registries and tissue banking, where available, is encouraged for patients with low-grade lymphomas. 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 systemic therapy and radiation therapy. Team members may include anaesthetists, oncologists, surgeons, haematologists, nurses, clinical psychologists, exercise physiologists, physiotherapists and dietitians, among others.
For patients with low-grade lymphomas who may undergo multiple treatments over many decades, prehabilitation may be an ongoing intervention that becomes increasingly relevant as the patient ages and experiences additional medical, physical and psychological limitations.
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 should 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 or during treatment. This can include an offer of referral to Quitline in addition to smoking cessation pharmacotherapy if clinically appropriate.
Evidence in solid cancers 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 low-grade lymphomas, including those who will initially undergo close observation in a ‘watch and wait’ approach, the multidisciplinary team should consider these specific prehabilitation assessments and interventions:
- conducting a physical and psychological assessment to establish a baseline function level, including assessing coping strategies/abilities
- identifying impairments and providing targeted interventions to improve the patient’s function level (Silver & Baima 2013)
- an integrated and structured approach to self-care enquiry – identifying Social Health, Exercise, Education, Diet and Sleep Hygiene (SEEDS) is one such practical tool (Arden 2015)
- referral to a psycho-oncology service to improve their knowledge and motivation towards healthy self-care care behaviours and to emotionally regulate throughout the course of the disease and treatments
- 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 and 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.
More information
See the Cancer Council website for more information.
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; stigma; 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
- physical distress caused by breathlessness and coughing, which may be alleviated through a referral to allied health professionals (physiotherapy, occupational therapy, exercise physiology or pulmonary rehabilitation); non-pharmacological strategies may be beneficial in breathlessness management (CareSearch 2019a) (note: if oxygen is medically indicated, this can be arranged through the relevant state aids and equipment program)
- hoarseness may require referral to a speech therapist or ENT specialist for palliative procedures
- management of physical symptoms such as pain, fatigue, cough and breathlessness (Australian Adult Cancer Pain Management Guideline Working Party 2019; Johnson & Currow 2016)
- delays in help-seeking for symptoms and psychological distress due to stigmatisation of Low-Grade Lymphomas associated with smoking (Cancer Australia 2020b)
- having issues with family and friends because of the association of tobacco use and Low-Grade Lymphomas (Carmack Taylor et al. 2008)
- 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])
- 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, particularly following an advanced stage Low-Grade Lymphomas diagnosis (stages III–IV) or to assist with pain management.
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 low-grade lymphomas as well as to relevant websites and support groups such as Cancer Council, the Leukaemia Foundation.
- 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
- explain the principles of ‘watch and wait’ if active surveillance is planned rather than commencing treatment
- discuss the benefits of multidisciplinary care and gain the patient’s consent before presenting their case at an MDM
- discuss progress in the cycle of quitting and refer to Quitline (13 7848) for behavioural intervention if the patient currently smokes (or has recently quit), and prescribe smoking cessation pharmacotherapy, as clinically appropriate
- recommend an ‘integrated approach’ throughout treatment regarding nutrition, exercise, sleep hygiene, cognitive function (commonly affected by steroids) 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 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.
More information
Refer to Principle 6 ‘Communication’ for communication skills training programs and resources.
