Cancer of Unknown Primary FRCR Flashcards
What is cancer of unknown primary origin?
Cancer of unknown primary origin is a condition in which a patient has metastatic tumour without an identified primary source.
(NICE, 2010)
What terms are used in NICE clinical guideline CG104 for cancer of unknown primary?
The terms used are: metastatic malignancy of uncertain origin (MUO), provisional carcinoma of unknown primary (provisional CUP), and confirmed carcinoma of unknown primary (confirmed CUP).
(NICE, 2010)
What should be done if a primary tumour is identified?
If a primary tumour is identified, treatment should continue as for that individual tumour site.
What is the treatment approach if a primary tumour is not identified?
If a primary tumour is not identified, treatment has to be empirical and based on research in patients whose primary tumour is known.
What percentage of all invasive malignancies does cancer of unknown primary account for?
Cancer of unknown primary accounts for 3–5% of all invasive malignancies in the western world.
How many new cases of cancer of unknown primary were diagnosed in the UK in 2011?
In 2011, there were 9762 new cases of cancer of unknown primary diagnosed in the United Kingdom.
What is the age-standardised rate of cancer of unknown primary in the UK?
The age-standardised rate in the UK is 10.2 per 100,000 population.
What is the median age at diagnosis for cancer of unknown primary?
The median age at diagnosis is 65–70 years.
What are the commonest primary sites identified in patients with cancer of unknown primary?
The commonest primary sites are pancreas (20–26%), lung (17–23%), liver (3–11%), large bowel (4–10%), stomach (3–8%), kidney (4–6%), ovary (3–4%), prostate (3–4%), and breast (2%).
What is the importance of investigating patients with cancer of unknown primary?
It is important to consider potentially curable malignancies, such as germ cell tumours or lymphoma.
What is the definition of malignancy of undefined primary origin (MUO)?
Metastatic malignancy identified on the basis of a limited number of tests, without an obvious primary site, before comprehensive investigation.
What is the definition of provisional carcinoma of unknown primary origin (provisional CUP)?
Metastatic epithelial or neuroendocrine malignancy identified on the basis of histology/cytology, with no primary site detected despite a selected initial screen of investigations.
What is the definition of confirmed carcinoma of unknown primary origin (confirmed CUP)?
Metastatic epithelial or neuroendocrine malignancy identified on the basis of final histology, with no primary site detected despite a selected initial screen of investigations.
What are some risk factors and aetiology associated with CUP?
Several genetic mutations have been implicated including MYC, RAS, EGFR, PDGFR, MET, KIT, and p53.
What percentage of cancer patients present with symptoms from metastases from an unknown primary site?
15% of cancer patients present with symptoms from metastases from an unknown primary site.
What are the most frequent presenting features of malignancy of undefined primary origin (MUO)?
The most frequent presenting features are pain (60%), liver mass or abdominal symptoms (40%), palpable nodal disease (20%), bone pain or fracture (15%), chest symptoms (15%), and central nervous system abnormality (5%).
What is the recommended approach for the investigation of MUO?
There should be a CUP team in every cancer centre, and a two-phase diagnostic approach should be carried out guided by the patient’s clinical condition.
What initial assessments are required for patients with MUO?
Patients require a thorough comprehensive history and physical examination, along with laboratory tests including full blood count and serum LDH.
What imaging tests are recommended for patients with MUO?
CXR and CT scanning of the thorax, abdomen, and pelvis are recommended.
What are the common histopathological findings in patients with MUO?
The most common findings are adenocarcinoma, squamous carcinoma, and poorly differentiated types.
What immunohistochemical biomarkers help in tumour diagnosis?
Some biomarkers include CK for carcinoma, PSA for prostate, and ER for breast.
What is the significance of t(11;22) in cancer?
It is associated with Ewing sarcoma and peripheral neuroendocrine tumours (PNET).
What specialized tests may be indicated for patients with MUO?
Further investigations may be indicated, particularly if patients have symptoms or signs suggesting a primary site.
What blood tests are performed for suspected germ cell tumours or hepatocellular carcinoma?
Serum αFP and βhCG are carried out.
What blood test is done if prostate cancer is suspected?
PSA is done if the presentation is compatible with prostate cancer.
What blood test is done if ovarian cancer is suspected?
CA125 is done if the presentation is compatible with ovarian cancer.
What is the purpose of endoscopy in cancer diagnosis?
Endoscopy is performed if clinically indicated and not already carried out.
What procedure is used for patients with intrapulmonary nodules unsuitable for percutaneous biopsy?
Flexible bronchoscopy with biopsy, brushings, and washings is used.
What is VATs exploration used for?
It is used in patients with a negative bronchoscopy where percutaneous biopsy is not appropriate.
What should be combined with panendoscopy in patients with malignant neck lymph nodes?
Directed biopsies of the nasopharynx, tongue base, hypopharynx, and if negative, bilateral tonsillectomy.
When should 18F-FDG PET-CT be offered?
It should be offered to patients with cervical lymphadenopathy and no primary tumour identified.
What is the role of mammography in cancer diagnosis?
Mammography is undertaken if the presentation is compatible with breast cancer.
When is breast MRI carried out?
Breast MRI is carried out for patients with adenocarcinoma of the axillary nodes.
What is the TNM classification used for?
It is used to stage patients with cancer of unknown primary according to the most likely primary site.
What should be done when a probable primary site is found in patients with MUO?
Standard treatment for that site should be given.
What percentage of patients with CUP are in the unfavourable group?
80% of patients will be in the unfavourable group.
What role does surgery play in managing patients with CUP?
Surgery may be useful, particularly for those in one of the more favourable subgroups.
What was investigated in a phase II prospective trial regarding cancer classification?
The effectiveness of a 92-gene reverse transcriptase polymerase chain reaction cancer classification assay to predict tumour origin.
What was the median survival for patients receiving assay-directed site-specific treatment?
The median survival was 12.5 months compared to 8.9 months historically.
What is the management approach for isolated brain metastases?
Management includes palliation of specific problems and referral to an MDT for local treatments
MDT refers to multidisciplinary team, which may consider other factors like unusual primary tumors.
In which scenarios may radiotherapy be indicated?
Radiotherapy may be indicated for:
* Standard treatment for probable primary cancer in CUP
* Treatment of squamous cell cancer of cervical lymph nodes
* Palliative treatment for bone pain, epidural spinal cord compression, SVCO
What is the overall response rate of chemotherapy regimens for CUP based on phase II studies?
The overall response rate is less than 20%
This indicates limited effectiveness of chemotherapy regimens in CUP.
Which chemotherapy regimens appear to have a higher response rate for CUP?
Platinum-based chemotherapy regimens appear to give a higher response rate
Examples include cisplatin and gemcitabine.
What percentage of patients with CUP are in the unfavourable subgroup?
80% of patients with CUP are in the unfavourable subgroup
What are commonly used chemotherapy regimens for patients with CUP?
Commonly used regimens include:
* Epirubicin
* Cisplatin
* 5-FU
* Capecitabine
* Gemcitabine
* Taxanes
What is the prognosis for one-year and five-year survival in CUP?
One-year survival is 16% and five-year survival is 8%
What are poor prognostic factors for CUP?
Poor prognostic factors include:
* More than three metastatic sites
* Performance Status (PS) 2 or more
* Male gender
* Non-lymph node metastases
* Raised LDH, lymphopaenia, low serum albumin
* Unfavourable CUP subgroup
What is the median survival for patients with CUP who undergo chemotherapy?
The median survival is 11.0 months with only 1.5% alive at 5 years
True or False: Patients treated with radiotherapy have a significantly better survival compared to those who had no radiotherapy.
False
What is the CUP-One trial focused on?
The CUP-One trial focuses on:
* New diagnostic tools like molecular profiling
* Effectiveness of ECX chemotherapy
Fill in the blank: The treatment for patients with cancer of unknown primary is likely to move towards treatment based on _______ predicting treatment response.
molecular profiling
What is the recommendation for patients with unfavourable CUP and poor performance status?
Best treated with supportive care alone, with palliative radiotherapy for local symptom control
What should be considered for patients with predominant liver metastases?
Consider a 5-FU-based regimen, e.g. epirubicin, oxaliplatin, and capecitabine
What is the recommended treatment for patients with predominant lung metastases if breast or thyroid cancer is thought unlikely?
Consider a platinum-based treatment regimen
What is the suggested approach for patients with bone metastases?
Consider radiotherapy for painful sites and a trial of hormone treatment or possible combination chemotherapy
What is the significance of obtaining a tissue diagnosis in CUP cases?
It is valuable if epithelial markers are positive with radiological evidence of malignancy, PS > 2, and more than three metastatic sites
