Limited dorsal myeloschisis involving one hemicord of a split cord malformation - a "hemi-LDM".

This is a case report of an exceedingly rare case of a limited dorsal myeloschisis (LDM) with its stalk inserted on the midline dorsal surface of one of a pair of hemicords in a type II split cord malformation. This entity, literally a "hemi-LDM," has been seen only once by the senior author in his catalogue of over 200 cases of LDM (Pang et al., 2020), nor has it been reported elsewhere before. We postulate that here the mechanism of focal nondisjunction of the hemi-neural plate during primary neurulation, which produces LDMs, occurs at the cusp of the consecutive developmental stages of gastrulation and primary neurulation, right after the appearance of the hemi-neural plates and hemi-notochords caused by the endomesenchymal tract. This child also had a terminal lipoma attached to the end of the conus, indicating that disruption of all three tandem stages of neural tube formation, namely, gastrulation, primary neurulation, and secondary neurulation, can occur in the same individual.

An integrated genomic analysis of anaplastic meningioma identifies prognostic molecular signatures.

Anaplastic meningioma is a rare and aggressive brain tumor characterised by intractable recurrences and dismal outcomes. Here, we present an integrated analysis of the whole genome, transcriptome and methylation profiles of primary and recurrent anaplastic meningioma. A key finding was the delineation of distinct molecular subgroups that were associated with diametrically opposed survival outcomes. Relative to lower grade meningiomas, anaplastic tumors harbored frequent driver mutations in SWI/SNF complex genes, which were confined to the poor prognosis subgroup. Aggressive disease was further characterised by transcriptional evidence of increased PRC2 activity, stemness and epithelial-to-mesenchymal transition. Our analyses discern biologically distinct variants of anaplastic meningioma with prognostic and therapeutic significance.

Predictors of early progression of surgically treated atypical meningiomas.

BACKGROUND: Clinical behaviour of atypical meningiomas is not uniform. While, as a group, they exhibit a high recurrence rate, some pursue a more benign course, whereas others progress early. We aim to investigate the imaging and pathological factors that predict risk of early tumour progression and to determine whether early progression is related to outcome. METHODS: Adult patients with WHO grade II meningioma treated in three regional referral centres between 2007 and 2014 were included. MRI and pathology characteristics were assessed. Gross total resection (GTR) was defined as Simpson 1-3. Recurrence was classified into early and late (≤ 24 vs. > 24 months). RESULTS: Among the 220 cases, 37 (16.8%) patients progressed within 24 months of operation. Independent predictors of early progression were subtotal resection (STR) (p = 0.005), parafalcine/parasagittal location (p = 0.015), peritumoural oedema (p = 0.027) and mitotic index (MI) > 7 (p = 0.007). Adjuvant radiotherapy was negatively associated with early recurrence (p = 0.046). Thirty-two per cent of patients with residual tumour and 26% after GTR received adjuvant radiotherapy. There was a significantly lower proportion of favourable outcomes at last follow-up (mRS 0-1) in patients with early recurrence (p = 0.001). CONCLUSIONS: Atypical meningiomas are a heterogeneous group of tumours with 16.8% patients having recurrence within 24 months of surgery. Residual tumour, parafalcine/parasagittal location, peritumoural oedema and a MI > 7 were all independently associated with early recurrence. As administration of adjuvant radiotherapy was not protocolised in this cohort, any conclusions about benefits of irradiation of WHO grade II meningiomas should be viewed with caution. Patients with early recurrence had worse neurological outcome. While histological and imaging characteristics provide some prognostic value, further molecular characterisation of atypical meningiomas is warranted to aid clinical decision making.

Dual lumbar bronchogenic and arachnoid cyst presenting with sciatica and left foot drop.

INTRODUCTION: Spinal bronchogenic cysts are rare findings, with only four cases of lumbar bronchogenic cysts reported in the literature. All of these bronchogenic cysts involved the conus medullaris. We present the first case of a lumbar bronchogenic cyst and arachnoid cyst arising from the cauda equina in a 68-year-old male. Uniquely, this bronchogenic cyst also contained components of an arachnoid cyst. METHODS: Magnetic resonance imaging (MRI) demonstrated a compressive cystic lesion at the level of the L3 vertebra splaying the cauda equina. An L3/L4 laminectomy was performed with marsupialisation of the cyst. RESULTS: Histological examination revealed pseudostratified ciliated columnar epithelium confirming the diagnosis of a bronchogenic cyst, as well as a pleated fibrovascular tissue lined by sparsely spaced small monomorphic arachnoidal cells, indicating an arachnoid cyst. CONCLUSION: We demonstrate that bronchogenic cysts can be successfully treated with marsupialisation.