This article was last reviewed and updated on October 17, 2018.
by Jason Wasserman, MD PhD FRCPC
When we breathe, air enters our body through our nose and mouth and travels down a long tube called the trachea into our lungs. The inside of the nose is called the nasal cavity and it helps to warm and clean the air before it reaches our lungs. The nasal cavity is surrounded by small paired (there is on each side of the face) air-filled spaces called paranasal sinuses that connect to the nasal cavity by small openings. Because the nasal cavity and sinuses are connected to each other, pathologists describe this area of the body as the sinonasal tract.
The inside of the nasal cavity and the sinuses are lined by cells that form a barrier called the epithelium. The tissue underneath the epithelium is called stroma and it is made up of blood vessels and small round structures called glands that make a substance called mucin. Pathologists use the word mucosa to describe tissue that includes both the epithelium and the stroma.
Sinonasal undifferentiated carcinoma (SNUC) is a cancer that starts from the cells in the epithelium of the sinonasal tract. When examined under the microscope, the cells in sinonasal undifferentiated carcinoma do not look like any of the cells normally found in the epithelium. Pathologists use the word undifferentiated to describe cancer cells that do not look anything like normal, healthy cells.
Sinonasal undifferentiated carcinoma can start in the nasal cavity or any of the surrounding paranasal sinuses. The tumour is often large at the time of diagnosis and symptoms include nose bleeds, pain, congestion, or visual changes.
Compared to other cancers of the head and neck, very little is known about what causes sinonasal undifferentiated carcinoma although some patients may have received radiation therapy in the past for a different cancer in the same area of the body.
Sinonasal undifferentiated carcinoma is an aggressive disease with poor prognosis. The cancer cells in sinonasal undifferentiated carcinoma commonly spread outside of the sinonasal tract into the bones of the jaw, the orbit, and the base of the skull. The cancer cells also commonly travel (metastasize) to lymph nodes and other more distant parts of the body.
Most patients with sinonasal undifferentiated carcinoma are diagnosed after a biopsy is performed and sent to a pathologist for examination. In some cases, the entire tumour can be removed, however, many patients will receive radiation therapy and/or chemotherapy after the biopsy.
If you had surgery to remove the tumour from your body, your pathologist will attempt to measure the tumour and this measurement will be included in your report. For example, if the tumour measures 5 cm x 3 cm x 1 cm, the report may describe the tumour size as 5 cm in greatest dimension.
Tumours from the sinonasal tract are often removed in multiple pieces. As a result, your pathologist may not be able to accurately measure the tumour size. In this case, an approximate tumour size may be described.
Tumour size will only be described in your report after the entire tumour has been removed.
Nerves are like long wires made up of groups of cells called neurons. Nerves transmit information (such as temperature, pressure, and pain) between your brain and your body. Perineural invasion is a term pathologists use to describe cancer cells attached to a nerve.
Perineural invasion is important because cancer cells that have attached to a nerve can use the nerve to travel into tissue outside of the original tumour. For this reason, perineural invasion is associated with a higher risk that the tumour will come back in the same area of the body (local recurrence) after treatment.
Lymphatics and blood vessels are long tubes that allow fluid (lymph and blood, respectively) and cells to travel around the body. When cancer cells enter a lymphatic or blood vessel it is called lymphovascular invasion and is associated with a higher risk that cancer cells will travel (metastasize) to a lymph node or a distant site such as the lungs.
A margin is any tissue that was cut by the surgeon in order to remove the tumour from your body. Whenever possible, surgeons will try to cut tissue outside of the tumour to reduce the risk that any cancer cells will be left behind after the tumour is removed.
Your pathologist will carefully examine all the margins in your tissue sample to see how close the cancer cells are to the edge of the cut tissue.
A margin is considered positive when there are cancer cells at the very edge of the cut tissue. A positive margin is associated with a higher risk that the tumour will grow back (recur) in the same site after treatment.
A negative margin means there were no cancer cells at the very edge of the cut tissue. If all the margins are negative, most pathology reports will say how far the closest cancer cells were to a margin. The distance is usually described in millimeters.
Margins will only be described in your report after the entire tumour has been removed.
Metastatic disease describes the process where cancer cells escape the main tumour and travel to another part of the body. Lymph nodes are small immune organs located throughout the body. They are a common target for metastatic disease.
The presence of cancer cells in a lymph node (also called lymph node metastases) is associated with a higher risk that the cancer cells will be found in other lymph nodes or in a distant organ such as the lungs. For this reason, lymph nodes in the area of the tumour are often removed and submitted for pathological examination.
Lymph nodes from the neck are typically removed at the same time the main tumour in a procedure called a neck dissection. The lymph nodes removed usually come from different areas of the neck and each area is called a level. The levels in the neck include 1, 2, 3, 4, and 5. Your pathology report will often describe how many lymph nodes were seen in each level submitted for examination.
Most reports include the total number of lymph nodes examined and the number that contain cancer cells. A group of tumour cells inside a lymph node is called a tumour deposit. Your pathologist will measure each tumour deposit and the largest deposit at each neck level will be included in your report.
Laterality refers to whether the lymph node is on the same side as the tumour (ipsilateral) or the opposite side of the tumour (contralateral). Cancer cells found in lymph nodes on both sides of the neck are described as bilateral.
All lymph nodes are surrounded by a capsule. Extranodal extension(or extracapsular extension) means that cancer cells have broken through the capsule and into the tissue that surrounds the lymph node. Extranodal extension is also associated with a higher risk of new tumours developing in the neck and is often used by your doctors to guide your treatment.
Pathologic stage (pTNM)
The pathologic stage for sinonasal undifferentiated carcinoma is based on the TNM staging system, an internationally recognized system originally created by the American Joint Committee on Cancer.
This system uses information about the primary tumour (pT), lymph nodes (pN), and distant metastatic disease (pM) to determine the complete pathologic stage (pTNM). Your pathologist will examine the tissue submitted and give each part a number. In general, a higher number means more advanced disease and worse prognosis.
Tumour stage (pT) for tumours that start in the nasal cavity or ethmoid sinus
These tumours are given a tumour stage between 1 and 4. The tumour stage is based on how far the tumour has spread outside of the nasal cavity or ethmoid sinus.
Tumour stage (pT) for tumours that start in the maxillary sinus
These tumours are given a tumour stage between 1 and 4. The tumour stage is based on how far the tumour has spread outside of the maxillary sinus.
Nodal stage (pN) for tumours that start in the nasal cavity or paranasal sinuses
These tumours are given a nodal stage between 0 and 3 based on the following three features:
The nodal stage will be higher if any of the tumour deposits are larger than 3 cm, more than one lymph node contains cancer cells, cancer cells are found in lymph nodes on both sides of the neck, and if any of the lymph nodes show extranodal extension.
If no cancer cells are found in any of the lymph nodes examined, the nodal stage is N0.
If no lymph nodes are submitted for pathological examination, the nodal cannot be determined and the stage is listed as NX.
Metastatic stage (pM) for sinonasal undifferentiated carcinoma
Sinonasal undifferentiated carcinoma is given an metastasis stage (pM) of 0 or 1 based on the presence of cancer cells at a distant site in the body (for example the lungs). The metastatic stage can only be determined if tissue from a distant site is submitted for pathological examination. Because this tissue is rarely present, the metastasis stage cannot be determined and is listed as MX.
Your pathologist may perform a test called immunohistochemistry on tissue from the tumour before they make the diagnosis of sinonasal undifferentiated carcinoma. If this test was performed on your tissue, you will probably see the results described in the microscopic description section of your pathology report.
The cancer cells in sinonasal undifferentiated carcinoma are usually positive (reactive) for proteins called keratins including pan-cytokeratin, low molecular weight keratin, and cytokeratin 8/18 (CK8/18).
The cancer cells are usually negative (non-reactive) for cytokeratin 5 (CK5), p40, synaptophysin, and chromogranin although any of these may be described as ‘focally’ positive. Your report may also describe the cancer cells as being negative (non-reactive) for p16, EBER, S100, and CD45.