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Metastatic malignant disease of unknown primary origin

About

What is covered

This interactive flowchart covers the diagnosis and management of metastatic malignant disease of unknown primary origin.
Most patients with newly diagnosed cancer are found to have a clearly defined primary tumour after initial investigation and staging. In England and Wales about 4% of these patients are found to have cancer without an identifiable primary site, despite exhaustive tests.
Most patients who have malignancy without an identifiable primary site have tumours derived from epithelial cells, that is, carcinoma of unknown primary origin. The care of patients with tumours derived from non-epithelial cells (such as melanoma, sarcoma, lymphoma and germ-cell tumours) is not covered because it is well defined in existing guidelines for the specific tumour types.
Because of the lack of dedicated clinical services, patients who have malignancy without an identifiable primary site can be denied the care offered to patients with site-specific cancers. This guidance aims to address the needs of such patients and covers investigations, clinical and organisational arrangements plus optimal treatment and supportive care.

Updates

Updates to this interactive flowchart

15 December 2015 Link added to NICE guideline NG31 on care of dying adults in the last days of life.

Person-centred care

People have the right to be involved in discussions and make informed decisions about their care, as described in your care.
Making decisions using NICE guidelines explains how we use words to show the strength (or certainty) of our recommendations, and has information about prescribing medicines (including off label use), professional guidelines, standards and laws (including on consent and mental capacity), and safeguarding.

Your responsibility

Guidelines

The recommendations in this guideline represent the view of NICE, arrived at after careful consideration of the evidence available. When exercising their judgement, professionals and practitioners are expected to take this guideline fully into account, alongside the individual needs, preferences and values of their patients or the people using their service. It is not mandatory to apply the recommendations, and the guideline does not override the responsibility to make decisions appropriate to the circumstances of the individual, in consultation with them and their families and carers or guardian.
Local commissioners and providers of healthcare have a responsibility to enable the guideline to be applied when individual professionals and people using services wish to use it. They should do so in the context of local and national priorities for funding and developing services, and in light of their duties to have due regard to the need to eliminate unlawful discrimination, to advance equality of opportunity and to reduce health inequalities. Nothing in this guideline should be interpreted in a way that would be inconsistent with complying with those duties.
Commissioners and providers have a responsibility to promote an environmentally sustainable health and care system and should assess and reduce the environmental impact of implementing NICE recommendations wherever possible.

Technology appraisals

The recommendations in this interactive flowchart represent the view of NICE, arrived at after careful consideration of the evidence available. When exercising their judgement, health professionals are expected to take these recommendations fully into account, alongside the individual needs, preferences and values of their patients. The application of the recommendations in this interactive flowchart is at the discretion of health professionals and their individual patients and do not override the responsibility of healthcare professionals to make decisions appropriate to the circumstances of the individual patient, in consultation with the patient and/or their carer or guardian.
Commissioners and/or providers have a responsibility to provide the funding required to enable the recommendations to be applied when individual health professionals and their patients wish to use it, in accordance with the NHS Constitution. They should do so in light of their duties to have due regard to the need to eliminate unlawful discrimination, to advance equality of opportunity and to reduce health inequalities.
Commissioners and providers have a responsibility to promote an environmentally sustainable health and care system and should assess and reduce the environmental impact of implementing NICE recommendations wherever possible.

Medical technologies guidance, diagnostics guidance and interventional procedures guidance

The recommendations in this interactive flowchart represent the view of NICE, arrived at after careful consideration of the evidence available. When exercising their judgement, healthcare professionals are expected to take these recommendations fully into account. However, the interactive flowchart does not override the individual responsibility of healthcare professionals to make decisions appropriate to the circumstances of the individual patient, in consultation with the patient and/or guardian or carer.
Commissioners and/or providers have a responsibility to implement the recommendations, in their local context, in light of their duties to have due regard to the need to eliminate unlawful discrimination, advance equality of opportunity, and foster good relations. Nothing in this interactive flowchart should be interpreted in a way that would be inconsistent with compliance with those duties.
Commissioners and providers have a responsibility to promote an environmentally sustainable health and care system and should assess and reduce the environmental impact of implementing NICE recommendations wherever possible.

Short Text

Everything NICE has said on diagnosing and managing metastatic malignant disease of unknown primary origin in an interactive flowchart

What is covered

This interactive flowchart covers the diagnosis and management of metastatic malignant disease of unknown primary origin.
Most patients with newly diagnosed cancer are found to have a clearly defined primary tumour after initial investigation and staging. In England and Wales about 4% of these patients are found to have cancer without an identifiable primary site, despite exhaustive tests.
Most patients who have malignancy without an identifiable primary site have tumours derived from epithelial cells, that is, carcinoma of unknown primary origin. The care of patients with tumours derived from non-epithelial cells (such as melanoma, sarcoma, lymphoma and germ-cell tumours) is not covered because it is well defined in existing guidelines for the specific tumour types.
Because of the lack of dedicated clinical services, patients who have malignancy without an identifiable primary site can be denied the care offered to patients with site-specific cancers. This guidance aims to address the needs of such patients and covers investigations, clinical and organisational arrangements plus optimal treatment and supportive care.

Updates

Updates to this interactive flowchart

15 December 2015 Link added to NICE guideline NG31 on care of dying adults in the last days of life.

Sources

NICE guidance and other sources used to create this interactive flowchart.
SimpliCT laser-guided needle placement in interventional radiology (2017) NICE medtech innovation briefing 98
ERBE flexible cryoprobes for bronchoscopic diagnosis and treatment (2015) NICE medtech innovation briefing 39

Quality standards

Quality statements

Effective interventions library

Effective interventions library

Successful effective interventions library details

Implementation

NICE has produced resources to help implement its guidance on:

Information for the public

NICE has written information for the public on each of the following topics.

Pathway information

Person-centred care

People have the right to be involved in discussions and make informed decisions about their care, as described in your care.
Making decisions using NICE guidelines explains how we use words to show the strength (or certainty) of our recommendations, and has information about prescribing medicines (including off label use), professional guidelines, standards and laws (including on consent and mental capacity), and safeguarding.

Your responsibility

Guidelines

The recommendations in this guideline represent the view of NICE, arrived at after careful consideration of the evidence available. When exercising their judgement, professionals and practitioners are expected to take this guideline fully into account, alongside the individual needs, preferences and values of their patients or the people using their service. It is not mandatory to apply the recommendations, and the guideline does not override the responsibility to make decisions appropriate to the circumstances of the individual, in consultation with them and their families and carers or guardian.
Local commissioners and providers of healthcare have a responsibility to enable the guideline to be applied when individual professionals and people using services wish to use it. They should do so in the context of local and national priorities for funding and developing services, and in light of their duties to have due regard to the need to eliminate unlawful discrimination, to advance equality of opportunity and to reduce health inequalities. Nothing in this guideline should be interpreted in a way that would be inconsistent with complying with those duties.
Commissioners and providers have a responsibility to promote an environmentally sustainable health and care system and should assess and reduce the environmental impact of implementing NICE recommendations wherever possible.

Technology appraisals

The recommendations in this interactive flowchart represent the view of NICE, arrived at after careful consideration of the evidence available. When exercising their judgement, health professionals are expected to take these recommendations fully into account, alongside the individual needs, preferences and values of their patients. The application of the recommendations in this interactive flowchart is at the discretion of health professionals and their individual patients and do not override the responsibility of healthcare professionals to make decisions appropriate to the circumstances of the individual patient, in consultation with the patient and/or their carer or guardian.
Commissioners and/or providers have a responsibility to provide the funding required to enable the recommendations to be applied when individual health professionals and their patients wish to use it, in accordance with the NHS Constitution. They should do so in light of their duties to have due regard to the need to eliminate unlawful discrimination, to advance equality of opportunity and to reduce health inequalities.
Commissioners and providers have a responsibility to promote an environmentally sustainable health and care system and should assess and reduce the environmental impact of implementing NICE recommendations wherever possible.

Medical technologies guidance, diagnostics guidance and interventional procedures guidance

The recommendations in this interactive flowchart represent the view of NICE, arrived at after careful consideration of the evidence available. When exercising their judgement, healthcare professionals are expected to take these recommendations fully into account. However, the interactive flowchart does not override the individual responsibility of healthcare professionals to make decisions appropriate to the circumstances of the individual patient, in consultation with the patient and/or guardian or carer.
Commissioners and/or providers have a responsibility to implement the recommendations, in their local context, in light of their duties to have due regard to the need to eliminate unlawful discrimination, advance equality of opportunity, and foster good relations. Nothing in this interactive flowchart should be interpreted in a way that would be inconsistent with compliance with those duties.
Commissioners and providers have a responsibility to promote an environmentally sustainable health and care system and should assess and reduce the environmental impact of implementing NICE recommendations wherever possible.

Supporting information

Information about diagnosis of provisional and confirmed CUP

The aim of the initial diagnostic phase is to perform the most appropriate investigations efficiently, to identify:
  • a primary site, which will guide treatment decisions or
  • non-epithelial malignancy, which can be treated regardless of primary site (for example, lymphoma, other haematological malignancies, melanoma, sarcoma, and germ-cell tumours) or
  • metastatic epithelial or neuro-endocrine malignancy without an identifiable primary site (a diagnosis of provisional CUP).
If further investigation is appropriate, a second phase of special investigations may be offered to patients with provisional CUP. When these are complete and if a primary site has still not been identified, a diagnosis of confirmed CUP can be made.

Glossary

alpha-fetoprotein
cancer antigen 125
cytokeratin 7
cytokeratin 20
confirmed carcinoma of unknown primary origin is metastatic epithelial or neuro-endocrine malignancy identified on the basis of final histology, with no primary site detected despite a selected initial screen of investigations, specialist review and further specialised investigations as appropriate
carcinoma of unknown primary origin
computed tomography
oestrogen receptor
human chorionic gonadotrophin
multidisciplinary team
malignancy of undefined primary origin is metastatic malignancy identified on the basis of a limited number of tests, without an obvious primary site, before comprehensive investigation
placental alkaline phosphatase
prostate-specific antigen
provisional carcinoma of unknown primary origin is metastatic epithelial or neuro-endocrine malignancy identified on the basis of histology or cytology, with no primary site detected despite a selected initial screen of investigations, before specialist review and possible further specialised investigations
thyroid transcription factor-1

Paths in this pathway

Pathway created: January 2013 Last updated: March 2017

© NICE 2017

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