Rhabdoid meningiomas (RM) shows heterogeneous histological findings, and a wide variety of chromosomal copy number alterations (CNA) are associated with an unpredictable course of the disease. In this study, we analyzed a series of 305 RM samples from patients previously reported in the literature and 33 samples from 23 patients studied in our laboratory. Monosomy 22-involving the minimal but most common recurrent region loss of the 22q11.23 chromosomal region was the most observed chromosomal alteration, followed by losses of chromosomes 14, 1, 6, and 19, polysomies of chromosomes 17, 1q, and 20, and gains of 13q14.2, 10p13, and 21q21.2 chromosomal regions. Based on their CNA profile, RM could be classified into two genetic subgroups with distinct clinicopathologic features characterized by the presence of (1) chromosomal losses only and (2) combined losses and gains of several chromosomes. The latter displays a higher frequency of WHO grade 3 tumors and poorer clinical outcomes.

For routine clinical practice, it is vital prior to surgery to plan the steps of the same as possible complications that may occur and how to resolve them. This case is of special interest for being a large tumor that during its resection, to make it total and facilitate healing, leaves a large defect in the skull shell.

We report two rare and infrequent cases, one of a patient with a Cognard type V tentorial dural fistula diagnosed at the beginning as a trunk glioma in a patient with gait disturbance. And another with a Guillian Barré syndrome with tetraparesis secondary to a tentorial dural arteriovenous fistula.

Possible complications that may occur and how to solve them before surgery are essential. This case is of special interest for being a low-incidence pathology in a rare location. The case is of high interest because of the importance of evacuating the hemorrhage to prevent sequels and the complexity of its management and highlights the importance of considering the diagnosis of this event.