Treatments: Mucosal Melanoma of the Head and Neck

This page describes how mucosal melanoma of the nose, sinuses, mouth and throat is treated with surgery, radiation and systemic therapy.

This page describes how mucosal melanoma that starts in the lining of the nose, the sinuses, or the mouth and throat is treated, and what to expect. Because this cancer is uncommon, care is highly individualized, and the plan depends on where the tumor is, how far it has spread and a person's overall health. Treatment is guided by a team of specialists working together. The condition itself is described on the mucosal melanoma conditions page.

The Overall Goals of Treatment

Treatment usually has two aims: removing or controlling the tumor where it started (and in the neck, if involved), and lowering the chance that the cancer comes back or spreads elsewhere in the body. For most people with disease that can be removed, surgery is the main treatment, and radiation and medicines are added based on the individual situation.

Surgery to Remove the Tumor

Surgery is the cornerstone of treatment when the tumor can be removed completely. The goal is to take out all of the visible tumor along with a rim of normal-looking tissue around it.

• Nose and sinus tumors. These are often removed with an endoscopic approach, through the nostrils using a thin camera and specialized instruments, with no external incisions. Some tumors need an open operation through the face or mouth, and sometimes open and endoscopic techniques are combined to remove the tumor safely and rebuild the area. See treatment of nasal and sinus tumors and sinus surgery with mass excision recovery.

• Mouth and throat tumors. Tumors in the oral cavity (such as the gums, roof of the mouth or floor of the mouth) are removed through the mouth. Depending on the location, this may involve removing part of the upper jaw (maxilla) or lower jaw (mandible). See treatment of mouth tumors.

• Reconstruction. After the tumor is removed, the area may be rebuilt using nearby tissue, tissue transferred from another part of the body, grafts or a dental prosthesis. The goal is to restore appearance and important functions such as eating, swallowing and speaking.

• The least invasive effective approach. The approach is chosen to remove the cancer completely while causing the least harm to nearby healthy structures, such as the eye, the lining of the brain and nerves.

Understanding Surgical Margins

A "margin" is the border of normal tissue removed around the tumor. When no cancer cells are seen at the edge of the removed tissue, the margin is called clear or negative; this is the goal because it lowers the chance of the cancer returning in the same spot. During surgery, tissue may be sent to the laboratory right away to confirm whether more needs to be removed (see understanding pathology results).

In the nose and sinuses, important structures such as the eye, the base of the skull and major nerves sit very close to the tumor, so a wide margin of normal tissue is not always possible. When cancer cells are found at or near the edge, additional surgery and/or radiation afterward may be considered to improve local control.

Managing the Neck (Lymph Nodes)

Melanoma in the head and neck can sometimes spread to the lymph nodes of the neck. If imaging or examination shows the cancer has reached neck lymph nodes, those nodes are removed in an operation called a neck dissection, typically at the same time as, or as part of, the main tumor surgery. When the neck nodes appear normal, routine removal of neck nodes is generally not performed.

Radiation Therapy After Surgery

Radiation uses focused high-energy beams to destroy cancer cells that may remain after surgery (see radiation therapy). For mucosal melanoma of the head and neck, radiation after surgery is often strongly considered, especially when the tumor is more advanced or when margins are close or positive.

• What it helps with. Studies consistently show that adding radiation after surgery reduces the chance the cancer comes back in the area where it started. Radiation improves local control but has not been clearly shown to prevent spread to distant parts of the body or to reliably lengthen overall survival, which is why it is used as part of a larger plan.

• Timing and technique. Radiation is usually started within about 6 weeks of surgery. Modern techniques such as intensity-modulated radiation therapy (IMRT) shape the dose to the tumor area while protecting nearby healthy tissue. In some situations, proton therapy may be considered to further spare critical structures.

• When surgery is not possible. If a tumor cannot be removed, radiation (sometimes with medicine) may be used as the main treatment to control the disease.

Immunotherapy and Targeted Therapy

Medicines that travel through the body (systemic therapy) are an important and evolving part of care, particularly for advanced disease or when there is concern about spread beyond the original site. See immunotherapy for head and neck cancers and chemotherapy and immunotherapy.

• Immunotherapy (immune checkpoint inhibitors). These drugs help the immune system recognize and attack melanoma and are the main systemic treatment for advanced mucosal melanoma. Examples include PD-1 blocking drugs (such as nivolumab or pembrolizumab) used alone, and combinations such as nivolumab plus ipilimumab or nivolumab plus relatlimab. Combination immunotherapy tends to produce higher response rates than a single drug, usually with more side effects. Response rates in mucosal melanoma are generally lower than in melanoma of the skin, which is one reason clinical trials are so important.

• Immunotherapy after surgery. In some cases, immunotherapy may be offered after surgery to lower the risk of recurrence, though the evidence for mucosal melanoma is more limited than for melanoma of the skin.

• Immunotherapy before surgery (neoadjuvant). Giving immunotherapy before surgery is an active area of research. Early studies suggest it may shrink tumors and improve outcomes in some patients.

• Targeted therapy. Mucosal melanomas more often carry a change (mutation) in a gene called KIT than skin melanomas do. For tumors with certain KIT mutations, targeted pills that block KIT (such as imatinib) may be an option. Testing the tumor for specific mutations helps determine whether targeted therapy is appropriate.

The Role of Clinical Trials

Because mucosal melanoma of the head and neck is rare and standard treatments have limits, clinical trials are an especially important option. Trials study new medicines, new combinations and new ways of sequencing surgery, radiation and systemic therapy. In certain situations, enrolling in a clinical trial is considered a preferred choice, and asking about available trials is a reasonable part of any treatment discussion.

Team-Based Care

This cancer is best managed by a team of specialists who plan treatment together. The team commonly includes:

• Head and neck surgeons and, when needed, reconstructive surgeons

• Radiation oncologists

• Medical oncologists (who prescribe immunotherapy and targeted therapy)

• Pathologists (who examine tumor tissue and perform special staining)

• Radiologists (who interpret scans)

• Dental and prosthodontic specialists

• Nutrition, speech and swallowing therapists

• Support services for emotional well-being and help with quitting smoking

Before treatment begins, the workup usually includes a full head and neck examination, confirmation of the diagnosis under the microscope, and detailed imaging (such as CT and MRI) to map the tumor, along with scans such as PET-CT to check for spread. Dental, nutrition and swallowing evaluations may also be arranged.

Follow-Up and Surveillance

Regular follow-up is essential because recurrences are most common in the first couple of years. Visits allow the team to check for any return of the cancer, manage side effects, and support recovery of speech, swallowing, nutrition and dental health.

• Clinic visits. Examinations are most frequent in the first year and become less frequent over five years, then continue yearly. For nose and sinus tumors, visits include looking inside the nasal passages with a small scope.

• Imaging. Scans watch for recurrence in the treated area and for spread elsewhere; timing and type are tailored to the individual. Brain imaging may be included because melanoma can spread there.

• Other monitoring. People who received radiation to the neck may have thyroid blood tests over time. Those treated with immunotherapy are monitored for immune-related side effects even after treatment ends.

New symptoms or concerns between scheduled visits are best reported to the care team promptly.

Key Points

• Surgery to remove the tumor with clear margins is the main treatment when the disease can be removed; nose and sinus tumors are often removed endoscopically.

• Radiation after surgery improves control of the cancer in the treated area, as part of a broader plan.

• Immunotherapy is the main systemic treatment for advanced disease, and targeted therapy may be an option for tumors with specific gene changes.

• Clinical trials are an important and sometimes preferred option in this rare cancer.

• Care is delivered by a multidisciplinary team, with regular, long-term follow-up.