Metastatic squamous neck cancer with occult primary is a very rare type of carcinoma (cancer arising from tissues that line organs) that develops in the neck. It is a type of secondary cancer (cancer that has spread from another part of the body) that forms from the squamous cells lining the neck that have spread from an unknown (or occult) point of origin in the body. Cancer/s that have spread from an unknown point of origin in the body are known as cancers of unknown primary (CUP).
CUPs are often considered advanced cancers as they have already metastasised (spread). Your doctor will try to find the primary cancer to decide treatment options, however if it can’t be found the cancer cells can be tested to give some indication on what the primary cancer is likely to be.
Treatment
If metastatic squamous neck cancer with occult primary is detected, it will be staged and graded based on size and how the cancer cells look under the microscope. Staging and grading helps your doctors determine the best treatment for you.
Cancers may be staged using the TNM staging system:
- T (tumour) indicates the size and depth of the tumour.
- N (nodes) indicates whether the cancer has spread to nearby lymph nodes.
- M (metastasis) indicates whether the cancer has spread to other parts of the body.
This system can also be used in combination with a numerical value, from stage 0-IV:
- Stage 0: this stage describes cancer cells in the place of origin (or ‘in situ’) that have not spread to nearby tissue.
- Stage I: cancer cells have begun to spread to nearby tissue. It is not deeply embedded into nearby tissue and had not spread to lymph nodes. This stage is also known as early-stage cancer.
- Stage II: cancer cells have grown deeper into nearby tissue. Lymph nodes may or may not be affected. This is also known as localised cancer.
- Stage III: the cancer has become larger and has grown deeper into nearby tissue. Lymph nodes are generally affected at this stage. This is also known as localised cancer.
- Stage IV: the cancer has spread to other tissues and organs in the body. This is also known as advanced or metastatic cancer.
Cancers can also be graded based on the rate of growth and how likely they are to spread:
- Grade I: cancer cells present as slightly abnormal and are usually slow growing. This is also known as a low-grade tumour.
- Grade II: cancer cells present as abnormal and grow faster than grade-I tumours. This is also known as an intermediate-grade tumour.
- Grade III: cancer cells present as very abnormal and grow quickly. This is also known as a high-grade tumour.
Once your tumour has been staged and graded, your doctor may recommend genetic testing, which analyses your tumour DNA and can help determine which treatment has the greatest chance of success. They will then discuss the most appropriate treatment option for you.
Treatment is dependent on several factors, including location, stage of disease and overall health.
Treatment options for this disease may include:
- Surgery, potentially including:
- Radical neck dissection (removal of all tissues in the neck from the jawbone to the collarbone, including muscles, nerves, salivary glands and major blood vessels).
- Modified radical neck dissection (removal of lymph nodes and some tissues in the neck).
- Partial neck dissection (removal of some lymph nodes in the neck, and possibly some tissue).
- Radiation therapy.
- Chemotherapy.
- Clinical trials.
- Palliative care.
For more information on the treatment options, please refer to the Rare Cancers Australia Treatment Options page.
Risk factors
Because of how rare metastatic squamous neck cancer with occult primary is, there has been limited research done into the risk factors of this disease. However, a potential link of being infected with the human papilloma virus (HPV) has been observed.
Early symptoms
Due to the rarity of this disease, there have been few symptoms reported. The only symptom that has been linked to this disease is a persistent lump or pain in the neck and/or throat.
Not everyone with the symptoms above will have cancer but see your general practitioner (GP) if you are concerned.
Diagnosis/diagnosing
If your doctor suspects you have a metastatic squamous neck cancer with occult primary, they will order a range of diagnostic tests to confirm the diagnosis, and refer you to a specialist for treatment.
Physical examination
Your doctor will collect your overall medical history, as well as your current symptoms. Following this, they may examine your body to check for any abnormalities. More specifically, they check your head and neck to check for signs of lumps or any other abnormalities.
Imaging & blood tests
The doctor will take images of your body using magnetic resonance imaging (MRI), a computed tomography scan (CT scan), positron emission tomography scan (PET scan) and/or ultrasound, depending on where it is suspected the cancer is. The doctor may also look at other parts of the body and looks for signs of metastasis. Additionally, a blood test may be taken to assess your overall health and help guide treatment decisions.
Biopsy
Once the location(s) of the cancer has been identified, the doctor will perform a biopsy to remove a section of tissue using a needle. This can be done during an endoscopy (surgical procedure that examines internal organs and tissues) or a tonsillectomy (removal of the tonsils).
The tissue sample will then be analysed for cancer cells. This can be done by a fine needle aspiration (FNA), excisional biopsy or a core needle biopsy (CNB).
Prognosis (Certain factors affect the prognosis and treatment options)
While it is not possible to predict the exact course of the disease, your doctor may be able to give you a general idea based on the rate and depth of tumour growth, susceptibility to treatment, age, overall fitness, and medical history. Generally, early-stage metastatic squamous neck cancers with occult primary have a better prognosis and survival rates. However, if the cancer is advanced and has spread, the prognosis may not be as good and there may be a higher risk of cancer recurrence. It is very important to discuss your individual circumstances with your doctor to better understand your prognosis.
References
Some references are to overseas websites. There may be references to drugs and clinical trials that are not available here in Australia.