Section Editor: Jason Wasserman MD PhD FRCPC
October 27, 2026
SWI/SNF complex-deficient sinonasal carcinoma is a rare, fast-growing type of cancer that starts in the nasal cavity or the sinuses. The tumor cells have lost one of the proteins that make up a group called the SWI/SNF complex. Pathologists confirm the diagnosis by showing that the tumor cells lack this protein.
Two types of SWI/SNF complex-deficient sinonasal carcinoma exist. The more common type has lost a protein called SMARCB1, also known as INI1. The less common type has lost a protein called SMARCA4, also known as BRG1. Both types were once diagnosed as other cancers, such as sinonasal undifferentiated carcinoma or neuroendocrine carcinoma, before testing for these proteins became routine.
This article explains what SWI/SNF complex-deficient sinonasal carcinoma is, how the two types differ, and what the findings in your pathology report mean for your care.
SWI/SNF complex-deficient sinonasal carcinoma is most commonly found in adults. People with the SMARCB1-deficient type are typically diagnosed in their 50s. People with the SMARCA4-deficient type tend to be younger, typically in their early 40s. About 2 in 3 people with either type are men.
The tumor most often starts in the nasal cavity or the ethmoid sinuses, the small air spaces between the eyes. By the time it is found, it often involves several sinuses.
SWI/SNF complex-deficient sinonasal carcinoma is caused by a change in one of the genes that build the SWI/SNF complex. This complex is a group of proteins that controls which genes in a cell are switched on or off. It does this by loosening or tightening the way DNA is packed inside the cell.
SMARCB1 and SMARCA4 normally act as tumor suppressors, meaning they help stop cells from growing out of control. When a gene change removes one of these proteins, the cell loses that control and can grow into a tumor. These changes develop in tumor cells during a person’s life.
Unlike some other cancers of the nose, SWI/SNF complex-deficient sinonasal carcinoma has not been linked to infection with human papillomavirus or Epstein-Barr virus. No other clear risk factors have been identified.
The symptoms of SWI/SNF complex-deficient sinonasal carcinoma are caused by a mass growing inside the nose and sinuses. Because the tumor grows quickly, symptoms can appear and worsen quickly. Common symptoms include:
The diagnosis of SWI/SNF complex-deficient sinonasal carcinoma is made after tissue from the tumor is examined under the microscope by a pathologist. An ear, nose, and throat surgeon usually takes a biopsy during nasal endoscopy, using a thin camera passed through the nostril.
Under the microscope, the tumor consists of sheets and nests of cancer cells that do not resemble normal nasal tissue. In the SMARCB1-deficient type, the cells are often small and dark, a pattern called basaloid. Some tumors instead have larger cells with a pink cytoplasm pushed to one side, described as rhabdoid or plasmacytoid. The SMARCA4-deficient type is usually made of large cells with areas of dead tumor, called necrosis.
Because these features overlap with other cancers of the nose, immunohistochemistry confirms the diagnosis. The key result is the loss of either SMARCB1 or SMARCA4 in tumor cells, as described in the next section.
Once the diagnosis is confirmed, CT and MRI scans of the head and neck show how far the tumor has spread. Chest scans or a PET scan may also be used to look for spread to other parts of the body.
Immunohistochemistry is the main test used to diagnose SWI/SNF complex-deficient sinonasal carcinoma. Immunohistochemistry uses antibodies to show which proteins are present in the tumor cells. The pathologist looks for a missing SWI/SNF protein and uses other stains to rule out similar cancers. Results that may appear in your report include:
Laboratories use different panels, and not every report will include every stain.
The 5th edition of the World Health Organization (WHO) classification of head and neck tumors, published in 2022, divides SWI/SNF complex-deficient sinonasal carcinoma into two types. The type is named for the protein that is missing from the tumor cells:
Older reports, written before the 2022 WHO classification, may use names such as SMARCB1-deficient carcinoma, INI1-deficient carcinoma, or sinonasal undifferentiated carcinoma for these tumors.
SWI/SNF complex-deficient sinonasal carcinoma is not given a histologic grade. Many carcinomas are graded as well, moderately, or poorly differentiated, depending on how much they resemble normal tissue. This tumor is defined by its missing protein rather than its appearance, and it behaves as a high-grade cancer.
The College of American Pathologists (CAP) protocol for the nasal cavity and sinuses notes that some newer tumor types, defined by specific genetic changes, do not need a grade. Your report may therefore say that grade is not applicable, or it may not mention grade at all.
Perineural invasion means cancer cells are seen along or inside a nerve. In SWI/SNF complex-deficient sinonasal carcinoma, nerves near the skull base can give the tumor a path to spread toward the brain. Your report will say whether perineural invasion was identified, not identified, or could not be determined.
Lymphovascular invasion means cancer cells are seen inside a blood vessel or lymphatic channel. In SWI/SNF complex-deficient sinonasal carcinoma, this finding raises concern that tumor cells could travel to lymph nodes or other organs. Your report will say whether lymphovascular invasion was identified, not identified, or could not be determined.
A margin is the edge of tissue the surgeon cuts to remove the tumor. Margins are described only when SWI/SNF complex-deficient sinonasal carcinoma has been removed by surgery, not on a biopsy. Your report will describe them as follows:
Lymph nodes are small immune organs that filter fluid from the tissues. In SWI/SNF complex-deficient sinonasal carcinoma, spread to lymph nodes in the neck is found in fewer than 1 in 10 people at diagnosis. Lymph nodes are removed in an operation called a neck dissection when scans show they may contain cancer.
When lymph nodes are examined, your report will say how many were removed and how many contain cancer cells. It will also give the size of the largest group of cancer cells in a lymph node. The report will also state whether extranodal extension is present, meaning cancer cells have broken through the lymph node’s outer capsule into surrounding tissue. Extranodal extension increases the nodal stage and is one of the findings your care team considers when planning treatment after surgery.
For SWI/SNF complex-deficient sinonasal carcinoma, no established biomarker tests currently guide the choice of a specific targeted drug. The loss of SMARCB1 or SMARCA4 is used for diagnosis, as described in the immunohistochemistry section above. Some laboratories also confirm the gene change using next-generation sequencing (NGS).
Researchers have studied drugs called EZH2 inhibitors in tumors that have lost SMARCB1. One of these, tazemetostat, received accelerated approval from the US Food and Drug Administration (FDA) for other cancers but never for sinonasal carcinoma. Its manufacturer withdrew it from all markets in March 2026 because of a safety concern. You may still see it mentioned in older articles about this tumor.
Research into other treatments that target SWI/SNF loss is ongoing. Your oncologist can tell you whether a clinical trial or broader molecular testing is appropriate in your situation. More information is available in our Biomarkers and Genetic Testing section.
The pathologic stage for SWI/SNF complex-deficient sinonasal carcinoma uses the TNM system in the AJCC Cancer Staging Manual, 8th edition. The pTNM stage combines the tumor stage (pT) and the nodal stage (pN). Imaging rather than the pathology report usually determines the metastasis stage (M).
The tumor stage for SWI/SNF complex-deficient sinonasal carcinoma depends on where the tumor started. Because most of these tumors have grown into nearby structures by the time they are found, the majority are pT4.
Nasal cavity and ethmoid sinus:
Maxillary sinus:
If the tumor stage cannot be assessed, for example because the tumor was removed in many small pieces, your report may say that the pT stage was not assigned.
Your care team combines the pT stage, the pN stage, and your scans to assign an overall stage group from stage I to stage IV. A tumor that is pT4a is at least stage IVA, and a tumor that is pT4b is stage IVB. Cancer that has spread to distant organs is stage IVC.
SWI/SNF complex-deficient sinonasal carcinoma is a serious cancer, and the prognosis is guarded. Because it is rare, most of what we know comes from small patient groups. For the SMARCB1-deficient type, about 6 in 10 people are alive 2 years after diagnosis, and about half are alive at 3 years.
Less is known about the SMARCA4-deficient type. Some reports describe very short survival of only months, while larger combined analyses find outcomes similar to the SMARCB1-deficient type. About 1 in 3 people with either type develop metastasis (spread to distant organs) at some point. The lungs, bones, and brain are the most common sites.
The tumor stage in the pathology report is the finding most strongly linked to outcome. A pT4b tumor has grown into the brain, the dura, or the back of the eye socket. People with a pT4b tumor have a poorer outlook than people with smaller tumors. Your care team can explain what your report findings mean for your outlook.
Once SWI/SNF complex-deficient sinonasal carcinoma has been confirmed, the next steps depend on the type, the tumor stage, and how far the tumor has spread. Your care team usually includes a head and neck surgeon, often with a skull base surgeon or neurosurgeon. A radiation oncologist and a medical oncologist are also involved. Treatment usually combines more than one approach:
After treatment, regular examinations with a nasal endoscope and scans are used to look for any sign that the cancer has returned.
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