Sinonasal Malignancies and Cancer of the Nasopharynx

Written by: Dr. London, MD

Sinonasal malignancies, tumors that arise inside the nose and nearby sinus area, are an uncommon subset of head and neck cancer. These are a diverse group of malignancies and include sinonasal squamous cell carcinoma (SNSCC), olfactory neuroblastoma, nasopharyngeal carcinoma (NPC) and others. Initial symptoms are not specific for sinus cancer and can include difficulty breathing through the nose and nosebleeds, which are commonly seen due to benign sources like sinus infection and septal deviation. Thus, sinonasal tumors may grow to be large before they are diagnosed.

A heightened awareness and concern for a sinus tumor should be raised when symptoms also include facial numbness, double vision, or protrusion of the eye. This is because these tumors grow close to the eye, important nerves of the face, and the brain. Sinonasal tumors may also be related to viruses such as the Epstein Barr Virus (EBV) for NPC or the human papillomavirus for SNSCC. Sinonasal tumors are rare, their treatment complex, and thus, should be treated by high-volume multi- disciplinary cancer treatment teams. In this article we will discuss common questions related to the diagnosis, treatment, and long-term care of patients with sinonasal malignancies.

Diagnosis: Getting the correct diagnosis can be challenging as many of these tumor types may look similar under the microscope. However, getting the correct diagnosis is essential because there are many different types of sinonasal tumors and the treatment varies significantly for each type.

How can I assist with obtaining the right diagnosis?
It is recommended that any biopsy sample be evaluated by an expert head and neck pathologist familiar with distinguishing sinonasal tumors. If one is not available at your institution, a second opinion pathology review by an outside head and neck pathology specialist is recommended.

What additional studies are important for diagnosis and treatment?
A computed tomography (CT) scan is ideal for evaluating bone invasion and a high-resolution magnetic resonance imaging (MRI) study is ideal for evaluating soft tissue infiltration. Together these imaging studies help to ascertain whether a tumor has broken through bone protecting the eye and brain and whether the tumor may be growing into these structures or along nerves. Although it is uncommon for sinonasal malignancies to spread to other locations in the body, it is important to perform additional imaging such as either a PET-CT scan or a CT scan of the neck, chest, abdomen, and pelvis to rule out distant tumor spread.

When is a biopsy performed?
Obtaining a biopsy sample is important to establish the proper diagnosis. A biopsy may be performed either during a clinic visit or in the operating room depending on a variety of circumstances. Prior to performing a biopsy, it is important to have performed imaging of the mass. This can help avoid biopsy of lesions that should not undergo a biopsy such as an encephalocele (portion of brain protruding into the nose) or a tumor that may be rich in blood vessels. Additionally, if body imaging identifies a potentially area of concern for spread in the neck or elsewhere in the body a needle biopsy by an interventional radiologist may be recommended. Either an ultrasound or CT scan may be used to guide placement of the biopsy needle into the appropriate location.

Treatment: After clinical evaluation by a multi-disciplinary cancer treatment team including an otolaryngologist – head and neck surgeon, radiation oncologist, and medical oncologist, sinonasal malignancy cases should be presented at a group at your institution called a multi-disciplinary tumor board for discussion. The tumor board is a collection of specialists at an institution including head and heck cancer surgery, radiation and medical oncology, radiology, pathology, and other specialists. Case presentation may include review of pertinent imaging studies, pathology, and other exam findings followed by a 4 Find SPOHNC at www.spohnc.org multi-disciplinary discussion and treatment recommendations.

How will my tumor be treated?
The answer to this question is complex and depends on a large number of factors including the tumor type and disease extent. Some tumor types such as primary NPC respond well to non-surgical approaches such as radiation and chemotherapy and therefore are seldom treated with surgery except if they recur. Other tumor types may respond more favorably to primary surgical approaches; however, the decision whether to perform surgery depends on the disease extent and whether the tumor can be completely removed by surgery. Some cancer centers advocate for induction chemotherapy approaches for tumors including sinonasal undifferentiated carcinoma (SNUC) and other locally advanced tumors. In this scenario several rounds of chemotherapy may be given prior to establishing whether definitive treatment will be non-surgical or by surgical methods.

Should my surgery be performed endoscopically?
Surgically removing sinonasal tumors through an endoscopic approach, with cameras and instruments through the nostrils without making incisions on the head or face, has gained increasing popularity in past years. However, whether your tumor should be removed endoscopically or through an open craniofacial approach depends on a variety of circumstances.

In most cases, the goal of surgery is to remove the entire tumor and obtain negative pathological margins. This goal should not be compromised when a surgical approach is chosen. Some sinonasal tumors may not be amenable to an endoscopic approach such as those that involve the skin, eye, and palate. In some cases an endoscope may be used to assist tumor removal during an open approach.

What are the side effects of treatment?
The side effects of treatment vary greatly depending upon the extent and type of surgery, as well as the type and amount of radiation therapy or chemotherapy utilized in the treatment plan. Common side effects of surgery may include pain, nosebleeds, nasal congestion, numbness, nasal crusting, loss or change in sense of smell and others. The severity of nasal crusting can be more pronounced when surgery is combined with radiation therapy and may take longer to resolve. Additional side effects of radiation and chemotherapy may include dry mouth, change in taste, numbness in the fingers or toes, fatigue, decreased immune cell function, nausea, loss of appetite, and others.

Long-term: Completion of treatment is only the beginning and there are significant long-term impact and considerations for patients with sinonasal tumors.

What is cancer survivorship?
Cancer survivorship has many definitions but starts at cancer diagnosis and entails a long-term care management plan to optimize patient well-being. This plan may involve a cancer survivorship nurse or internal medicine physician. Patients may find it useful to join support groups to allow for interaction with other patients who are undergoing similar experiences. a patient may ask about local cancer support groups. There may be national groups available such as SPOHNC. Certain types of sinonasal tumors are so rare they only affect a couple hundred patients in the United States per year thus it may be challenging to find a specialized support group. One example is olfactory neuroblastoma (ONB) for which specific support groups may be found on social media and ONB-specific patient centered events are conducted on an annual basis (https://www.cancer. gov/pediatric-adult-rare-tumor/rare-tumors/other-rare-tumors/ olfactory-neuroblastoma).

What is included in and what is the duration of cancer surveillance?
After completion of treatment, it is important for the patient to follow closely with the cancer treatment team for cancer surveillance. This includes regular clinical visits and physical exam and nasal endoscopy for close evaluation of the sinonasal cavity. This may also include high-resolution MRI imaging of the sinonasal cavity as well as surveillance imaging of the neck and chest according to standard head and neck cancer practices. The duration of surveillance varies for sinonasal malignancies. Some sinonasal tumors such as ONB can recur 20 years later, thus cancer surveillance may be life-long for some sinonasal tumor types. For virally associated tumors such as EBV+ NPC, surveillance may also include blood tests to assess for the presence of EBV in the blood as a potential marker of the tumor coming back.

What if my cancer comes back?
Close surveillance with your cancer treatment team is important to try and help detect cancer recurrence early. Should a cancer 5 recurrence unfortunately occur, a similar process with a multi-disciplinary cancer team evaluation, biopsy, and thorough imaging studies are important to assess the extent of disease. Presentation of your case at a multi-disciplinary tumor board is important to determine which manner of treatment may be best for your particular case. Clinical trial opportunities may be available.or search for clinical trial opportunities that may be available at other institutions (www.clinicaltrials.gov).

Disclaimer: This article contains generalized advice and is not a substitute for personalized medical care and recommendations. It is important to work closely with a multi- disciplinary cancer treatment team during diagnosis, treatment, and continued long-term surveillance. Editor’s Note: Dr. London was born and raised in Southern California. He received his bachelor’s degree in Microbiology from Brigham Young University followed by his M.D. Ph.D. from The University of Utah. Dr. London then completed his residency in Otolaryngology-Head and Neck Surgery at Johns Hopkins. He then completed a fellowship at The Ohio State University in open and endoscopic skull base surgery under the direction of Dr. Ricardo Carrau.

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