Diffuse Leptomeningeal Glioneuronal Tumors: A Case Series of Five Patients with Parenchymal Forms and an Analysis of the Diagnostic Challenges, Treatment Options and Outcomes.

BACKGROUND: Diffuse leptomeningeal glioneuronal tumors (DL-GNT) are rare glioneuronal neoplasms with oligodendroglioma-like cells. These tumors can present as a dominant intracranial mass or as a solitary spinal cord mass without leptomeningeal involvement. In this study, we aimed to determine the magnetic resonance imaging and histopathological features, treatment modalities, and clinical outcomes of the parenchymal forms of DL-GNTs. METHODS: This is a retrospective three-center case series study of 5 patients with a confirmed parenchymal form of DLGTs, out of which 4 patients were adults. Brain and spinal cord MR imaging were performed in all patients at either 1.5 or 3T. The patients' age ranged from 5 years to 50 years with a mean age of 27.6 years at presentation. RESULTS: Four of the tumors were located in the frontal lobe, and one in the tectum. They were usually solid-cystic enhancing tumors as the other mixed neuronal-glial tumors. All of the tumors had an extension to the superficial surface of a cerebral hemisphere. One had systemic bone metastases. The clinical signs and symptoms of the parenchymal form varied based on the location of the mass, in contrast to the leptomeningeal form associated with hydrocephalus. In one case, the tumor's initial grade was defined as intermediate. The initial histopathology of the two cases was low-grade and no upgrade occurred in the follow-up period. In two cases, although the tumors were low grade initially, they progressed to an anaplastic form in the follow-up period. CONCLUSION: The parenchymal form of DL-GNTs is common in adults. Extension to the superficial surface of a cerebral hemisphere is a distinctive imaging feature. Systemic osseous metastasis may occur. Due to the presence of common histopathological features, including the biphasic composition of glial and neuronal cell elements and oligodendroglioma-like cells, a proposed classification approach might be more beneficial for the histopathological and imaging description, and management of the glioneuronal tumors with oligodendroglioma-like features.

Extensive widening of Virchow-Robin spaces in the frontal lobe: two case reports and systematic review of the literature.

AIM: To report our experience on giant tumefactive Virchow-Robin spaces (GTVRS) in the frontal lobe and perform a systematic review of previous reports on GTVRS. MATERIALS AND METHODS: This is a retrospective single-center study reporting the clinical manifestations, magnetic resonance (MR) imaging appearance, differential diagnosis, and management of two patients diagnosed with frontal lobe GTVRS at Bahcesehir University School of Medicine Goztepe Hospital in the past 5 years. A systematic literature search was performed in the PubMed and Google Scholar databases, with case selection criteria including Virchow-Robin spaces (VRS) size greater than 1.5 cm, frontal lobe localization, and the presence of MR imaging. The search strategy included only English language keywords. The systematic review was searched between database inception and May 6, 2022. RESULTS: A total of 18 cases were included in the study. Of the 15 cases with known sex, nine were female and six male. The median age was 29.8 with an age range of 4-57. Eleven of the 18 lesions were in the right frontal lobe. The lesions were multilocular in 15 cases and unilocular in three cases. All lesions had signal intensity as cerebrospinal fluid, showed no perifocal edema, and did not enhance. A hyperintensity was noted around the 14 lesions on the FLAIR sequence. Ten lesions showed cortical thinning adjacent to the lesion. No abnormality was detected on DWI, SWI, and MRS. Follow-up imaging was available in ten patients without any interval change. Unnecessary surgical interventions were noted in three cases. CONCLUSIONS: The results of reported cases and the literature review emphasize the role of MR imaging in the diagnosis of frontal lobe GTVRS. Beyond diagnostic consideration, GTVRS may have prognostic value and often indicate a "don't touch lesion" albeit requiring further consideration on a case-to-case basis. Familiarity with this entity improves diagnostic accuracy and, prevents accidental diagnosis of any neoplasm or other diseases.