Sporadic schwannomas and neurofibromas

Herbert B Newton MD (Dr. Newton, Director of the Neuro-Oncology Center at the Florida Hospital Cancer Institute, has no relevant financial relationships to disclose.)
Rimas V Lukas MD, editor. (Dr. Lukas has received honorariums from AstraZeneca as an advisory board member and AbbVie as a guest speaker.)
Originally released October 15, 1996; last updated March 1, 2017; expires March 1, 2020

This article includes discussion of sporadic schwannomas and neurofibromas, chitoneuroma, neurilemmoma, perineural fibroblastoma, abducens schwannoma, facial schwannoma, hypoglossal schwannoma, jugular foramen schwannoma, malignant schwannoma, neurofibroma, neurofibrosarcoma, peripheral nerve schwannoma, schwannoma, spinal schwannoma, sporadic neurofibromas, sporadic schwannomas, and trigeminal schwannoma. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

In this article, the author provides an in-depth review of the pathology, biology, clinical presentation, and treatment options for sporadic schwannomas and neurofibromas. These tumors arise from the nerve sheaths of cranial nerves, nerve roots, spinal nerves, and peripheral nerves. The most common location for schwannomas is the eighth cranial nerve, whereas neurofibromas more commonly arise along the spinal nerve roots. Maximal surgical resection is the treatment of choice for most tumors. Radiotherapy is only used in selected cases. Chemotherapy is still under investigation for therapeutic potential. In this update, the author reviews advances in the molecular biology and therapeutic approaches to these tumors.

Key points

 

• Schwannomas are slow-growing extra-axial tumors that can arise from any cranial nerve or spinal nerve root. They most commonly arise from the eighth cranial nerve.

 

• Surgical resection is the treatment option of choice for most tumors. Complete resection can result in a surgical cure.

 

• In selected cases, radiosurgery may be as effective as surgery at local control of tumor growth.

 

• Chemotherapy is not applicable to most schwannomas. Data suggest that anti-angiogenic treatment approaches may be helpful in selected cases.

Historical note and terminology

By most accounts, the earliest reported description of a vestibular schwannoma was by Sandifort, in 1777 (Sandifort 1777). The first clinicopathologic correlation was by Leveque-Lasource in 1810 (Leveque-Lasource 1810). The earliest complete clinical report of a patient with a vestibular schwannoma was by Bell in 1830, as described by Cushing (Cushing 1917). In 1835, Cruveilhier is credited with the most complete description of both the clinical and pathologic features of the tumor (Cruveilhier 1835). The first successful operative removal of a vestibular schwannoma was by Sir Charles Balance in 1894. Based on the clinical description and autopsy findings of a patient with a vestibular schwannoma that was localized and diagnosed antemortem, Von Monakow was of the opinion that patients with these tumors were excellent surgical candidates (Von Monakow 1900). This report generated much interest within the neurosurgical community in surgical therapy for vestibular schwannomas. Over the next 30 years, surgical techniques and approaches to intracranial schwannomas were refined to such a degree that operative mortality was reduced from approximately 50% to 60% in the early 1900s to 4% by 1931 (Macfarlane and King 1995). New techniques introduced by Cushing (eg, subtotal tumor removal, decompressive suboccipital craniectomy, uncapping of the cerebellum) were responsible for much of this improvement. Further refinement in surgical techniques, the use of intraoperative monitoring, and the advent of CT and MRI have further improved surgical morbidity and mortality over the last 30 years.

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