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National Collaborating Centre for Cancer (UK). Diagnosis and Management of Metastatic Malignant Disease of Unknown Primary Origin. Cardiff (UK): National Collaborating Centre for Cancer (UK); 2010 Jul. (NICE Clinical Guidelines, No. 104.)

Cover of Diagnosis and Management of Metastatic Malignant Disease of Unknown Primary Origin

Diagnosis and Management of Metastatic Malignant Disease of Unknown Primary Origin.

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Key priorities

  1. 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.

  2. 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.

  3. 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.

  4. 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.
  5. 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 on page 15)

    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 health-care 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.

  6. 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 on page 24)

    cancer antigen 125 (CA125) in women with peritoneal malignancy or ascites (see recommendation on page 24)

    alpha-fetoprotein (AFP) and human chorionic gonadotrophin (hCG) (particularly in the presence of midline nodal disease) (see recommendation on page 24)

    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.

  7. Do not use gene-expression-based profiling to identify primary tumours in patients with provisional CUP.
  8. 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.

  9. Include the patient’s prognostic factors in decision aids and other information for patients and their relatives or carers about treatment options.
  10. 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.
Copyright © 2010, National Collaborating Centre for Cancer.

No part of this publication may be reproduced, stored or transmitted in any form or by any means, without the prior written permission of the publisher or, in the case of reprographic reproduction, in accordance with the terms of licenses issued by the Copyright Licensing Agency in the UK. Enquiries concerning reproduction outside the terms stated here should be sent to the publisher at the UK address printed on this page.

The use of registered names, trademarks etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant laws and regulations and therefore for general use.

Bookshelf ID: NBK82162
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