Multidisciplinary Neurofibromatosis Type 2 Clinic: The Mayo Clinic Experience

Background: Neurofibromatosis Type 2 (NF2) is a debilitating neurocutaneous disorder with a prevalence of 1 in 80,000 worldwide with ~5,000 patients affected in the United States. Patients present with multiple schwannomas, meningiomas, and ependymomas affecting the entirety of the neuro-axis. The care of these patients, by necessity, is complex and requires multiple specialties to work together in a coordinated fashion. As a result, it can be challenging for patients to obtain comprehensive care in a single center, which can lead to delayed treatment and increased neurologic morbidity. There are 83 centers in 34 states recognized by the NF network.

Objective: To propose the necessary medical and surgical specialties to create an efficient, patient oriented NF2 clinic experience. We present our center's experience with an integrated, multidisciplinary NF2 clinic.

Results: We have ~200 patients followed in the multidisciplinary NF2 clinic. The specialties involved in the patient care include otology, head and neck surgery, skull base neurosurgery, spine neurosurgery, pediatric neurosurgery, neuro-oncology, neuroradiology, radiation oncology, audiology, and medical genetics. Every quarter, there is a dedicated clinic day during which 8 to 12 patients with NF2 are seen. Each patient undergoes the necessary neuro-axis imaging within 1 to 2 days prior to the clinic appointment. They are evaluated by neuro-oncology and their functional status is determined prior to the main clinic day. For each patient, a dossier is then created detailing their clinical history and prior treatments. On the morning of the clinic day, a meeting is held with all specialties involved in which the dossiers are reviewed alongside the most recent imaging with volumetric measurements presented by neuroradiology with comparisons to previous imaging. During this meeting, a tentative consensus treatment plan is recommended. Then, the patients are evaluated and counseled in clinic in tandem by physicians from each specialty involved in the care of the patient. Prior to leaving the clinic, the patients are scheduled for any necessary procedures and future imaging. Most patients can have all their care addressed within 2 to 3 days.

Conclusion: NF2 is a highly morbid disease requiring integration of multiple medical and surgical specialties. Patients can understandably become confused with their care plan between each physician, which can lead to them being lost to follow-up and can suffer unmonitored disease progression. To mitigate unnecessary neurologic morbidity, these patients require efficient multidisciplinary care in a single setting. This can only be accomplished through a dedicated clinic specifically for NF2 patients integrating all physician and non-physician specialties necessary for their care.

Publication History

Article published online:
01 February 2023