Neurocutaneous disorders, a host of congenital tumor syndromes resulting from mutations in tumor suppressor genes, result in lesions of the brain, spine, and peripheral nerves as well as other organ systems. The lifelong progression of systemic tumor-associated symptoms creates a unique intersection with multiple subspecialties, including neuro-oncology, neurology, neurosurgery, radiology, dermatology, ophthalmology, and genetics. This intersection has seen historical shifts, with successful treatment requiring a multidisciplinary approach. The development of robust cross-specialty collaborations has led to significant advancements over the past decade with respect to quality and quantity of life for these patients. In some cases, these advancements have centered around decreasing surgical burden, as with the development of everolimus for the treatment of subependymal giant cell astrocytomas. In other cases, there has been the further development of surgical tools, as in the treatment of multiple seizure foci in tuberous sclerosis complex through philosophical and technical innovation, defining a larger cohort of surgical candidates in this population and conferring improved cognition and decreased burden of antiepileptic drugs.
While pediatric neurosurgeons play an essential role in the treatment of patients with neurocutaneous disorders, some of the most important challenges and advancements stem from broadening and deepening our understanding of the underpinnings and natural history of these disorders to more thoughtfully and deliberately use our surgical tools.
This issue of Neurosurgical Focus therefore seeks to highlight current work that better quantifies the neurosurgical role in the most common neurocutaneous disorders as well as the role and impact of multidisciplinary approaches in these disease processes. We hope you enjoy this look into avenues for future study in this subdiscipline and look forward to the further conversation, innovation, and exploration these concepts spur.