Molecular description of meningeal solitary fibrous tumors/hemangiopericytomas compared to meningiomas: two completely separate entities

Introduction: Meningeal solitary fibrous tumors (SFT), like all SFT, are defined by NAB2-STAT6 fusion and share clinicopathologic similarities with meningiomas, the most frequent meningeal tumors. Our aim is to establish the molecular identity of meningeal SFT and seek molecular prognostic factors. Methods: RNA sequencing and whole exome sequencing (WES) were performed in STAT6-positive SFT and grade 2–3 meningiomas, and data concerning other soft tissues tumors was obtained from the local database. Uniform manifold approximation and projection (UMAP), individual gene expression and Gene Set Enrichment Analysis (GSEA) were performed. Results: RNA clustering shows that SFT share a common molecular signature, different from any other type of tumoral tissue. Meningeal SFT aggregate with other SFT, with no clinical or histological subgroup. Comparison of genes expressions suggests significant over-expressions of ZIC2, ZIC3, ZIC5, GABBR2, TP53 in CNS-SFT. The pathogenic TP53 c.743G > T variant, previously undescribed in SFT, was found in one sample of meningeal SFT during malignant progression. Conclusions: Meningeal SFT are molecular counterparts of extra-meningeal SFT, completely separate from meningiomas. They might develop from the same tissues and benefit from the same treatments as SFT.

Epidemiologic and histologic characteristics of CNS lesions: a 20-year experience of a tertiary center in Lebanon

Aim: Report the epidemiologic and histologic characteristics of CNS lesions in the Lebanese population. Methods: We conducted a retrospective study evaluating 2025 CNS lesions diagnosed between 1998 and 2017 in the pathology laboratory of a Lebanese tertiary center. Results: 52.2% of patients were men with a median age of 50 years. The most frequent symptoms were epilepsy (22.5%), headache (20.6%) and motor impairment (19.9%). 90.7% of tumors were primary. Lung (35.6%) and breast (16.5%) were the most frequent primaries of metastases. 46.2% of primary CNS tumors were glial, predominantly astrocytic (56.4%), and (42.5%) were nonglial, predominantly meningeal tumors (58%). Conclusion: Compared with Western literature, the Lebanese population is characterized by a younger age of onset of brain tumors, a lower rate of meningiomas and a higher rate of gliomas.

Microsurgical Anatomy of the Meningeal Branch of the Dorsolateral Medullary Plexus

BACKGROUND Meningeal branches originating from intradural arteries may be involved in several diseases such as meningeal tumors and arteriovenous lesions. These “pial-dural” arterial connections have been described for anterior cerebral, posterior cerebral, and cerebellar arteries. However, to the best of our knowledge, meningeal supply originating from the arterial plexus over the dorsolateral aspect of the medulla oblongata (dorsolateral medullary plexus [DLMP]) has not been described. OBJECTIVE To define the microsurgical anatomy of the meningeal branch of DLMP. METHODS A total of 20 cadaver heads (40 sides) underwent far-lateral craniotomy and the cerebellomedullary cisterns were explored to find the DLMP and any meningeal branches. Additionally, de-identified intraoperative images of 85 patients with vertebral artery (VA)/posterior inferior cerebellar artery aneurysms who had undergone far-lateral craniotomy were studied to find any meningeal branches of DLMP. RESULTS The meningeal branches of DLMP were identified in 4 cadavers/sides. These branches reached the region of jugular tubercle (JT) after crossing the accessory nerve. In 3 specimens, these branches were joined by a small twig from V4-VA before penetrating the dura. DLMP meningeal branches were found in 12 patients of the studied cohort (14%) with similar anatomical features as those found in the cadaveric study. CONCLUSION DLMP may give rise to meningeal branches to the adjacent dura of JT. The actual prevalence of this anatomic variation is difficult to estimate using our data. However, when present, these branches may have important clinical implications, ie, diseases such as dural arteriovenous fistulas, pial arteriovenous malformations (AVMs), and meningeal-based tumors.

Pediatric Meningeal Tumors of the Sylvian Fissure Region without Dural Attachment: A Series of Three Patients and Review of the Literature

Pediatric meningeal tumors are rare, but those in the region of the sylvian fissure without dural attachment are extremely rare, with only 24 previously reported cases in the world literature. In this series, we report two additional cases of sylvian fissure meningioma without dural attachment and one case of perisylvian meningioangiomatosis in the medial temporal lobe. All three patients presented with complex partial seizures, but the diagnosis was delayed in each case because the symptoms were misinterpreted to be behavioral rather than epileptic. The seizures were eventually confirmed with electroencephalogram, and subsequent imaging showed enhancing masses within the sylvian fissure region that were at least partially calcified in all three cases. Each patient underwent craniotomy. In the first case, gross total resection was achieved, and in the second case, a small residual portion of tumor was densely calcified and adherent to the middle cerebral artery branches. Both of these were World Health Organization (WHO) grade I meningiomas. The third patient underwent biopsy and limited resection of meningioangiomatosis. No dural attachments were noted in any of the tumors, but one of the meningiomas was intraparenchymal in location, surrounding the sylvian fissure in both the frontal and temporal lobes, which has been described in only a small number of these cases previously. The patients underwent pre- and postsurgical neuropsychiatric testing and did not experience any significant cognitive deficits. At 10-year follow-up, the patient who had gross total resection of the tumor has had no recurrence and is seizure-free without anticonvulsant medications. The incompletely resected intraparenchymal meningioma in the second patient recurred after 5 years, however, and at repeat surgery was found to have transformed to a WHO grade II tumor. Radiation therapy was delivered and the tumor has been stable for 2 years, but the patient continues to have occasional seizures despite medication. The patient with meningioangiomatosis has had no further growth and has excellent control of seizures but remains on medication. We review the clinical presentation of these rare tumors and discuss the treatment, outcomes, and possible relationship of meningiomas to meningioangiomatosis.