Prevention

The two most effective prevention strategies are avoiding tobacco smoking and maintaining a normal body weight.

Risk factors

• Tobacco smoking (most established risk factor; risk increases significantly with greater intensity and duration)
• Cystic lesions of the pancreas
• Obesity
• Increased consumption of red meat and processed meat
• Family history of pancreatic cancer
• Older age
• Chronic pancreatitis
• Longstanding type 2 diabetes mellitus
• Male gender
• Asian or Pacific Islander ethnicity
• Chronic alcohol consumption
• Liver cirrhosis
• Stomach infections
• Heavy occupational exposure to certain pesticides, dyes and chemicals used in metal refining
• Certain hereditary conditions

Early detection

People with a strong family history of pancreatic cancer and related hereditary conditions should be referred to a familial cancer service, geneticist or oncologist for possible genetic testing.

Potential monitoring for pancreatic cancer in high-risk populations includes endoscopic ultrasound for small pancreatic head tumours and blood tests (CA 19-9, carcinoembryonic antigen and liver biochemistry).

Screening

Population screening is not recommended for pancreatic cancer in Australia.

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Many cases present with non-specific symptoms or are asymptomatic until advanced stages of the disease.

The following signs and symptoms should be investigated:

• acute pancreatitis where the cause is not alcohol ingestion and gall stones are not evident
• new-onset diabetes
• jaundice that is progressive, together with unexplained weight loss and abdominal pain that may radiate to the back (the jaundice may also be accompanied with dark urine, light-coloured stools and itchy skin)
• pain, which is often severe, unrelenting and of a short duration
• unexplained weight loss
• pale and greasy stools
• nausea and vomiting
• constipation
• fatigue
• gall bladder enlargement
• a blood clot in the leg without a clear risk factor
• incidental lesions found on radiology.

Initial investigations

Where there is suspicion of pancreatic cancer, consider an abdominal CT scan with pancreatic protocol, conduct serum CA 19-9 and liver function tests and early referral is strongly indicated, usually prior to a definitive diagnosis. Where jaundice is present, the following should be performed urgently: liver function tests, abdominal ultrasound and CT where appropriate.

Referral options

At the referral stage, the patient’s GP or other referring doctor should advise the patient about their options for referral, waiting periods, expertise, if there are likely to be out-of-pocket costs and the range of services available. This will enable patients to make an informed choice of specialist and health service.

Communication

The GP’s responsibilities include:

• explaining to the patient and/or carer who they are being referred to and why
• supporting the patient and/or carer while waiting for specialist appointments
• informing the patient and/or carer that they can contact Cancer Council on 13 11 20.

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Timeframe

Patients who present with jaundice should be referred for tests within 48 hours and followed up rapidly.

Other symptoms require review within 2 weeks.

Patients with suspected or proven pancreatic cancer should be seen by a gastroenterologist, oncologist or hepatopancreaticobiliary surgeon with expertise in pancreatic cancer management and linked to a multidisciplinary team within 1 week of referral to the specialist.

Diagnosis and staging

Most diagnostic procedures should be completed before the MDM. Biopsy is only required where there is diagnostic uncertainty, or tissue is required for further management or clinical trials.

A contrast-enhanced CT scan should be completed first if not already performed. Additional tests may also be requested by the multidisciplinary team. If diagnostic uncertainty still remains, conduct:

• endoscopic ultrasound with or without biopsy
• contrast-enhanced MRI of the pancreas or magnetic resonance cholangiopancreatography (MRCP) in patients who cannot tolerate contrast or where diagnostic uncertainty remains
• diagnostic laparoscopy with or without laparoscopic ultrasound when resection is planned.

Staging pancreatic cancer may include the following investigations:

• CT scan of the chest/abdomen/pelvis and PET scan
• MRI pancreatic and/or liver
• laparoscopy plus or minus laparoscopic ultrasound for high-risk patients.

Genetic testing

Five to 10 per cent of pancreatic cancers arise due to a genetic predisposition.

A referral to a familial cancer service should be considered for all patients newly diagnosed with pancreatic cancer, particularly if any of the following features are noted:

• a family history of pancreatic cancer
• young age of diagnosis (< 60 years)
• a personal and/or family history of melanoma, breast, ovarian, stomach or colorectal cancer
• a family history of chronic pancreatitis
• Ashkenazi Jewish ancestry.

Treatment planning

The patient’s case must be discussed within 1 week of completing the diagnostic and staging investigations and a management plan should be finalised.

Research and clinical trials

Consider enrolment where available and appropriate. Search for a trial.

Communication

The lead clinician’s (1) responsibilities include:

• discussing a timeframe for diagnosis and treatment options with the patient and/or carer
• explaining the role of the multidisciplinary team in treatment planning and ongoing care
• encouraging discussion about the diagnosis, prognosis, advance care planning and palliative care while clarifying the patient’s wishes, needs, beliefs and expectations, and their ability to comprehend the communication
• providing appropriate information and referral to support services as required
• communicating with the patient’s GP about the diagnosis, treatment plan and recommendations from multidisciplinary meetings (MDMs).

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Timeframe

Diagnostic and staging investigations should be completed within 2 weeks of referral.

Establish intent of treatment

• Curative
• Anti-cancer therapy to improve quality of life and/or longevity without expectation of cure
• Symptom palliation

Treatment for resectable pancreatic cancer: The potential for curative surgery depends on the staging of the tumour, and only 10–20 per cent of patients have clearly resectable disease after careful pretherapeutic staging. This potential is assessed by the multidisciplinary team.

Curative surgery includes Whipple procedure, distal or total pancreatectomy, with or without chemotherapy (adjuvant or neoadjuvant chemotherapy or neoadjuvant chemoradiation).

Treatment for unresectable pancreatic cancer: If unresectable, any other treatment is almost certainly palliative because pancreatic cancer is unlikely to be cured by chemotherapy or radiation therapy. The most commonly used therapies include:

• endoscopic or radiological intervention
• surgical interventions
• chemotherapy with or without chemoradiation
• coeliac plexus or intrapleural block.

Palliative care: In general, all patients with pancreatic cancer, given the poor prognosis, should be offered a referral for a palliative care assessment as an integrated aspect of their overall oncology care. For more, visit the Palliative Care Australia website.

Communication

The lead clinician and team’s responsibilities include:

• discussing treatment options with the patient and/or carer including the intent of treatment as well as risks and benefits
• discussing advance care planning with the patient and/or carer where appropriate
• communicating the treatment plan to the patient’s GP
• helping patients to find appropriate support for exercise programs where appropriate to improve treatment outcomes.

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Timeframe

Treatment should begin within 4 weeks of the initial diagnosis, depending on urgency and modality.

Postoperative adjuvant chemotherapy should begin within 12 weeks of surgery.

Most patients with pancreatic cancer are palliated. If a patient is thought to have been cured after their treatment, then care in the post-treatment phase is driven by predicted risks, as well as individual clinical and supportive care needs.

Provide a treatment and follow-up summary to the patient, carer and GP outlining:

• the diagnosis, including tests performed and results
• tumour characteristics
• treatment received (types and date)
• current toxicities (severity, management and expected outcomes)
• interventions and treatment plans from other health professionals
• potential long-term and late effects of treatment and care of these
• supportive care services provided
• a follow-up schedule, including tests required and timing
• contact information for key healthcare providers who can offer support for lifestyle modification
• a process for rapid re-entry to medical services for suspected recurrence.

Communication

The lead clinician’s responsibilities include:

• explaining the treatment summary and follow-up care plan to the patient and/or carer
• informing the patient and/or carer about secondary prevention and healthy living
• discussing the follow-up care plan with the patient’s GP.

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Detection

It is likely that the patient’s current symptoms will progressively worsen. This should be managed following discussion by a multidisciplinary team in consultation with palliative care specialists.

Treatment

Evaluate each patient for whether referral to the original multidisciplinary team is appropriate. Treatment will depend on the location and extent of disease, previous management and the patient’s preferences.

Advance care planning

Advance care planning is important for all patients but especially those with advanced disease. It allows them to plan for their future health and personal care by thinking about their values and preferences. This can guide future treatment if the patient is unable to speak for themselves.

Survivorship and palliative care

Specialist palliative care is recommended for all patients with pancreatic cancer. Survivorship and palliative care should be addressed and offered early. Early referral to palliative care can improve quality of life.

Communication

The lead clinician and team’s responsibilities include:

• explaining the treatment intent, likely outcomes and side effects to the patient and/or carer and the patient’s GP.

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Palliative care

Consider a referral to palliative care.

Ensure an advance care directive is in place.

Communication

The lead clinician’s responsibilities include:

• being open about the prognosis and discussing palliative care options with the patient
• establishing transition plans to ensure the patient’s needs and goals are considered in the appropriate environment.

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