A total of 40 patients with epidermoid cysts of the cerebellopontine angle (CPA) underwent surgery between 1980 and 1993. Total resection was achieved in 30 cases (75%); in 10 cases (25%) parts of the cyst capsule were left because they adhered to the brainstem and vascular structures of the CPA. One patient with very large bilateral epidermoid cysts, who underwent complete bilateral resection in one stage, died of pulmonary aspiration and infection. As of their latest clinical and radiological follow-up examinations (mean 5.7 years), 93% of the patients are able to lead useful lives. Three cases of cyst regrowth have been observed thus far. Modern radiological tools and microsurgery techniques have considerably improved the completeness of cyst resection and reduced postoperative mortality and morbidity rates; however, there still are some cases in which complete resection is impossible without producing severe neurological deficits.

A total of 27 patients with trigeminal schwannoma were treated between 1982 and 1992 at the Neurosurgery Department of Nordstadt Hospital. Twelve cases of solitary schwannoma without any family history or physical stigmata of neurofibromatosis were included and form the basis of this study. There were four women and eight men (mean age 44 years) in this series. Duration of symptoms ranged from 2 months to 6 years. The most frequent symptoms were either pain or numbness of the ipsilateral hemiface. The surgical approach was chosen depending on the tumor type. Tumors that belonged to Type A (five cases), which were predominantly in the middle fossa, were approached using a transsylvian method; Type B (one case), which presented predominantly in the cerebellopontine angle, was operated on via a retrosigmoid suboccipital craniectomy; Type C (five cases), which were dumbbell-shaped extending into both the middle and posterior fossa, were removed via a combined temporal craniotomy-presigmoidal method; and in Type D (one case), in which tumor was primarily extracranial with intracranial extension, an infratemporal extradural approach was undertaken. There was no operative mortality or long-term disability in this series. The follow-up period ranged from 12 to 60 months; during that time magnetic resonance imaging revealed tumor recurrence in two cases after 12 and 48 months, respectively, and these were excised again. An additional 178 cases collected from the world literature are also reviewed and analyzed.

Sixteen patients with schwannomas of the jugular foramen were operated on in the Department of Neurosurgery of the Nordstadt Hospital in Hannover, Germany, between 1986 and 1992. Patients with neurofibromatosis were excluded. The records of the 16 patients were retrospectively reviewed. There were five women and 11 men (mean age 43 years) with a symptom duration ranging from 6 months to 20 years (mean 5 years). The predominant symptoms were hearing loss, hoarseness, and cerebellar symptoms. Computerized tomography (CT), magnetic resonance (MR) imaging, and angiography were performed in all 16 patients. Depending on the radiological and surgical features, the tumors were classified into four types: Type A, a tumor primarily at the cerebellopontine angle with minimal enlargement of the jugular foramen (eight cases); Type B, a tumor primarily at the jugular foramen with intracranial extension (two cases); Type C, a primarily extracranial tumor with extension into the jugular foramen (one case); and Type D, a dumbbell-shaped tumor with both intra- and extracranial components (five cases). A retromastoid suboccipital craniectomy was performed for Type A tumors, and a combined cervical-mastoidectomy for Types B, C, and D. Total tumor removal was achieved in all cases. There was no operative mortality. Postoperative complications were cerebrospinal fluid leakage in one patient and mastoiditis in two patients. The follow-up period ranged from 12 to 42 months (mean 22 months). All patients were alive at the last follow-up review, and CT and/or MR imaging showed no tumor recurrence.

Background Variability in standard-of-care classifications precludes accurate predictions of early tumor recurrence for individual patients with meningioma, limiting the appropriate selection of patients who would benefit from adjuvant radiotherapy to delay recurrence. We aimed to develop an individualized prediction model of early recurrence risk combining clinical and molecular factors in meningioma. Methods DNA methylation profiles of clinically annotated tumor samples across multiple institutions were used to develop a methylome model of 5-year recurrence-free survival (RFS). Subsequently, a 5-year meningioma recurrence score was generated using a nomogram that integrated the methylome model with established prognostic clinical factors. Performance of both models was evaluated and compared with standard-of-care models using multiple independent cohorts. Results The methylome-based predictor of 5-year RFS performed favorably compared with a grade-based predictor when tested using the 3 validation cohorts (ΔAUC = 0.10, 95% CI: 0.03–0.018) and was independently associated with RFS after adjusting for histopathologic grade, extent of resection, and burden of copy number alterations (hazard ratio 3.6, 95% CI: 1.8–7.2, P < 0.001). A nomogram combining the methylome predictor with clinical factors demonstrated greater discrimination than a nomogram using clinical factors alone in 2 independent validation cohorts (ΔAUC = 0.25, 95% CI: 0.22–0.27) and resulted in 2 groups with distinct recurrence patterns (hazard ratio 7.7, 95% CI: 5.3–11.1, P < 0.001) with clinical implications. Conclusions The models developed and validated in this study provide important prognostic information not captured by previously established clinical and molecular factors which could be used to individualize decisions regarding postoperative therapeutic interventions, in particular whether to treat patients with adjuvant radiotherapy versus observation alone.

Neurofibromatosis 2 (NF2) is an autosomal dominant disease predisposing to multiple tumors of the central and peripheral nervous system. Bilateral vestibular schwannomas are the hallmark of the disease. To define the clinical spectrum of the disease, we performed gadolinium-enhanced magnetic resonance imaging of the brain and spine as well as neurological, dermatological, and ocular examinations in 48 patients with NF2 diagnosed with the National Institutes of Health diagnostic criteria. Patients were ascertained from patient workshops and publications and from referral as a result of vestibular schwannoma surgery. Vestibular schwannomas were found in 46 patients (96%, 43 bilateral and 3 unilateral), spinal tumors were found in 43 (90%), posterior subcapsular cataracts were found in 30 (63%), meningiomas were found in 28 (58%), and trigeminal schwannomas were found in 14 (29%). The presenting symptoms included hearing loss or tinnitus in 15 patients (31%), multiple or nonspecific symptoms in 15 (31%), skin tumors in 12 (25%), and ocular symptoms in 6 (13%). When the complete spine was imaged, spinal tumors were more common in patients with NF2 than has previously been reported. This is a noteworthy finding, because spinal tumors are a major cause of NF2 morbidity and mortality.