Sinonasal malignancies make up less than 5% of all head and neck cancers, a small number compared with the hundreds of thousands of patients diagnosed with more common cancers, such as lung or breast malignancies. However, the unique challenges of sinonasal tumors are outsized — making it pivotal for patients with these cancers to be seen at large tertiary care centers, says Johns Hopkins sinus and skull base surgeon Nyall London, who also directs the Sinonasal and Skull Base Tumor Section at the National Cancer Institute, part of the National Institutes of Health. Johns Hopkins is one of the highest volume centers in the nation for these conditions.

            “Our multidisciplinary team cares for many of these patients every week,” London says, “and every member of this multidisciplinary team is crucial to achieve successful outcomes.”

            The importance of the team’s expertise and experience comes into play right at the start of patients’ cancer journeys, he explains, with getting a correct diagnosis. Unlike most other cancers, sinonasal tumors have extraordinary histological diversity — many different cancer types can arise in this space, with the majority requiring different treatment protocols for successful outcomes. To distinguish between these variants, London and his colleagues rely on head and neck pathologist Lisa Rooper, a national leader in diagnosing sinonasal tumors.

            Once patients have an accurate diagnosis, London says, he and other surgeons, medical oncologists and radiation oncologists work together to develop a personalized treatment plan. Surgery may not be possible in every case, but the oncology team can offer treatment options tailored to each patient, which may include radiation, chemotherapy and immunotherapy.

If surgery is part of a patient’s treatment plan, resecting tumors safely can present another challenge. The sinonasal space is relatively small and walled in by numerous critical structures, including the orbit, carotid arteries, optic and other cranial nerves, and the brain — anatomy that can become distorted by tumors.

For some patients, resection in this space can be performed endoscopically through the nose, leaving no visible scars; for others, open surgery is the only option. But sometimes this distinction is unclear, with successful resection hinging on being able to convert from endoscopic to open based on intraoperative findings.

London and the other otolaryngology skull base surgeons, including Masaru Ishii, Murray Ramanathan, Nick Rowan and Wojtek Mydlarz, often partner with neurosurgeons to complete these surgeries.

London works with Gary Gallia, neurosurgeon and director of the Johns Hopkins Neurosurgery Skull Base Center, on complex sinonasal surgeries — which sometimes go late into the night in the operating room — as well as basic and translational research on sinonasal malignancies. London and Gallia is one of the few teams in the country trained in both endoscopic and open techniques, allowing them to switch midstream if the opposite technique ends up being the better choice.   They are also among a small number of physician-scientists in the world with research programs focused on these relatively rare tumors. Additionally, they team up with Ramanathan to direct an annual patient education meeting on olfactory neuroblastoma, a rare sinonasal cancer type that affects less than one in a million individuals worldwide (see sidebar).

More than a decade ago, Gallia worked with neuroradiology colleagues to develop high-resolution skull base imaging protocols, enhancing the visualization of tumors and their extensions to improve surgical planning and outcomes. Additionally, Gallia and Ishii implemented a protocol for intraoperative circumferential contiguous margin assessment, maximizing the likelihood of complete cancer removal.

Other pivotal members of the care team are reconstructive surgeons, including Kofi Boahene and Shaun Desai, who step in once the resection is complete. Because sinonasal tumors can be cosmetically disfiguring, London says, these experts can help repair damage from both the cancer and its necessary treatment, helping patients feel whole again after this life-changing experience.

“I really enjoy caring for these patients.” London says. “It’s incredibly rewarding to hear patients tell me that I saved their life, or that I gave them another Thanksgiving with their family,” he says.