Intraventricular schwannomas: A case report and literature review

Abstract

Intraventricular schwannomas are a very rare pathological entity with few reported cases in the literature. A medline search up to July 2021 has identified 40 articles for a total of 42 cases. Our personal case has been also added. The patients were divided in two groups in regards of histological features and were separately analyzed and compared: benign intraventricular schwannomas (group A) and malignant intraventricular schwannomas (group B). Intraventricular schwannoma affected patients from the pediatric to the elderly age (range 7–78 years) and mainly the sex male (M,67% vs F,33%). The malignant forms were extremely rare (5%) with only two cases described in literature, but they were associated whit aggressive behavior with tendence to recur (100%). The clinical symptoms were mainly due to obstructive hydrocephalus or mass effect. The lateral ventricles (58%) were the most common involved site, followed by fourth (35%) and finally by the third (7%) ventricle. The hydrocephalus was present in 24% in the preoperative and in 5% of cases in the postoperative period. The surgical procedure performed is microscopic in all except in one of the cases, in which the endoscopic technique was used. There was a need for a ventricular shunting in six (15%) cases in the preoperative and in 7 (17%) in the postoperative course. A complete resection was achieved in 34 out of 43 cases, subtotal in 8 and in one case the tumor was discovered during the autopsy.Intraventricular schwannomas are mainly benign tumors, without tendence to regrowth or recurrence or malignant transformation after resection. A gross total removal is possible in the most of cases and adjuvant therapies are not necessary. Nevertheless, due to its rarity of occurrence, there are not defined guidelines of treatment. Intraventricular schwannomas should be considered in the differential diagnosis among intraventricular tumors.