Excerpt

The term “cancer of unknown primary” refers to a condition in which a patient has metastatic malignancy without an identified primary source. This is a very heterogeneous disease in which the type of tumour, the extent of spread, and the outcome of treatment all vary widely. When categorising patients with cancer of unknown primary, one important factor initially considered is the cell type of origin of the metastatic disease. The majority of patients have malignancy which appears to derive from epithelial cells, and hence are regarded as having carcinoma of unknown primary. Patients with tumours of non-epithelial lineage (melanoma, sarcoma, lymphoma, germ-cell) form a distinct and important minority, since subsequent management can often be satisfactorily undertaken even in the absence of an identifiable primary source. Such patients are not considered in this guideline, since their care is adequately defined in existing guidelines for their specific tumour type. The term “carcinoma of unknown primary” (CUP) is used henceforth to refer to those patients with metastatic malignancy of epithelial, neuroendocrine or undifferentiated lineage whose investigation and management is considered within the scope of this guideline.

Contents

• Key priorities
• Key research recommendations
• List of all recommendations
• Methodology
• Algorithms
• 1. Introduction and definitions
  • Definitions
• 2. Epidemiology
  • 2.1. Introduction
  • 2.2. Cancer registration
  • 2.3. Incidence
  • 2.4. Treatment data
  • 2.5. Treatment modalities
  • 2.6. Mortality data
  • 2.7. Survival
• 3. Organisation of services and support
  • 3.1. Introduction
  • 3.2. Current deficiencies in the management of patients with carcinoma of unknown primary (CUP)
  • 3.3. The CUP team and its functions
  • 3.4. Organisation of CUP services at network and national level
  • 3.5. Definitions and data collection for MUO and CUP
  • References
• 4. Diagnosis
  • 4.1. Introduction
  • 4.2. Initial diagnostic phase
  • 4.3. Second diagnostic phase – special investigations
  • 4.4. Investigation of specific clinical presentations
  • References
• 5. Factors influencing management decisions
  • 5.1. Introduction
  • 5.2. When to stop investigations
  • 5.3. Selecting optimal treatment
  • References
• 6. Managing specific presentations
  • 6.1. Introduction
  • 6.2. Presentations that may benefit from radical treatment
  • 6.3. Presentations with a poor prognosis
  • References
• 7. Systemic treatment
  • 7.1. Introduction
  • 7.2. Chemotherapy in patients with confirmed CUP
  • 7.3. Chemotherapy for recognised treatable syndromes
  • References
• Appendices
  • Appendix 1. What is the expected value of perfect information in reducing uncertainty surrounding the cost-effectiveness of systemic treatment in patients with confirmed carcinoma of unknown primary and no clinical features fitting a recognised syndrome?
  • Appendix 2. Immunohistochemistry
  • Appendix 3. Abbreviations
  • Appendix 4. Glossary
  • Appendix 5. Guideline scope
  • Appendix 6. List of topics covered by each chapter
  • Appendix 7. People and organisations involved in production of the guideline
• Evidence Review
  • Guideline chapter 1. Service Configuration
  • Guideline chapter 2. Diagnosis
  • Guideline chapter 3. Factors Influencing Management
  • Guideline chapter 4. Specific Presentations
  • Guideline chapter 5. Systemic Therapy
  • Appendix A. Search Strategies
  • Appendix B. Economic Plan
  • Appendix C. Health Economic Analyis
  • Appendix D. List of Excluded Studies

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