Key priorities for implementation

Key priorities for implementation

The following recommendations have been identified as priorities for implementation.

The CUP team and its functions

  • Every hospital with a cancer centre or unit should establish a carcinoma of unknown primary (CUP) team, and ensure that patients have access to the team when malignancy of undefined primary origin (MUO) is diagnosed. The team should:

    • consist of an oncologist, a palliative care physician and a CUP specialist nurse or key worker as a minimum

    • have administrative support and sufficient designated time in their job plans for this specialist role and

    • have a named lead clinician.

  • Every hospital with a cancer centre or unit should assign a CUP specialist nurse or key worker to patients diagnosed with MUO or CUP. The CUP specialist nurse or key worker should:

    • take a major role in coordinating the patient's care in line with this guideline

    • liaise with the patient's GP and other community support services

    • ensure that the patient and their carers can get information, advice and support about diagnosis, treatment, palliative care, spiritual and psychosocial concerns

    • meet with the patient in the early stages of the pathway and keep in close contact with the patient regularly by mutual agreement and

    • be an advocate for the patient at CUP team meetings.

  • Refer outpatients with MUO to the CUP team immediately using the rapid referral pathway for cancer, so that all patients are assessed within 2 weeks of referral. A member of the CUP team should assess inpatients with MUO by the end of the next working day after referral. The CUP team should take responsibility for ensuring that a management plan exists which includes:

    • appropriate investigations

    • symptom control

    • access to psychological support and

    • providing information.

  • A CUP network multidisciplinary team (MDT) should be set up to review the treatment and care of patients with confirmed CUP, or with MUO or provisional CUP and complex diagnostic or treatment issues. This team should carry out established specialist MDT responsibilities.

Organisation of CUP services at network and national level

  • Every cancer network should establish a network site-specific group to define and oversee policies for managing CUP. The group should:

    • ensure that every CUP team in the network is properly set up (see recommendation 1.1.1.1)

    • ensure that the local care pathway for diagnosing and managing CUP is in line with this guideline

    • be aware of the variety of routes by which newly diagnosed patients present

    • advise the cancer network on all matters related to CUP, recognising that many healthcare professionals have limited experience of CUP

    • maintain a network-wide audit of the incidence of CUP, its timely management and patient outcomes

    • arrange and hold regular meetings for the group to report patient outcomes and review the local care pathway.

Initial diagnostic phase

  • Offer the following investigations to patients with MUO, as clinically appropriate, guided by the patient's symptoms:

    • comprehensive history and physical examination including breast, nodal areas, skin, genital, rectal and pelvic examination

    • full blood count; urea, electrolytes and creatinine; liver function tests; calcium; urinalysis; lactate dehydrogenase

    • chest X-ray

    • myeloma screen (when there are isolated or multiple lytic bone lesions)

    • symptom-directed endoscopy

    • computed tomography (CT) scan of the chest, abdomen and pelvis

    • prostate-specific antigen (PSA) in men (see recommendation 1.2.2.1)

    • cancer antigen 125 (CA125) in women with peritoneal malignancy or ascites (see recommendation 1.2.2.1)

    • alpha-fetoprotein (AFP) and human chorionic gonadotrophin (hCG) (particularly in the presence of midline nodal disease) (see recommendation 1.2.2.1)

    • testicular ultrasound in men with presentations compatible with germ-cell tumours

    • biopsy and standard histological examination, with immunohistochemistry where necessary, to distinguish carcinoma from other malignant diagnoses.

Second diagnostic phase – special investigations

  • Do not use gene-expression-based profiling to identify primary tumours in patients with provisional CUP.

When to stop investigations

  • Perform investigations only if:

    • the results are likely to affect a treatment decision

    • the patient understands why the investigations are being carried out

    • the patient understands the potential benefits and risks of investigation and treatment and

    • the patient is prepared to accept treatment.

Selecting optimal treatment

  • Include the patient's prognostic factors in decision aids and other information for patients and their relatives or carers about treatment options.

Chemotherapy in patients with confirmed CUP

  • If chemotherapy is being considered for patients with confirmed CUP, with no clinical features suggesting a specific treatable syndrome, inform patients about the potential benefits and risks of treatment.

  • National Institute for Health and Care Excellence (NICE)