"T2/FLAIR: slightly hyperintense to grey matter"
"T1 C+ (Gd): mild heterogenous enhancement"
"DWI/ADC: reduced diffusivity, with similar ADC values to that of cortex."
"CNS tumour with BCOR internal tandem duplication (BCOR-ITD) is a rare brain tumour type that has been added to the WHO classification of CNS tumours 5th edition as part of the embryonal family of tumours. Originally identified through molecular analysis of tumours classified as primitive neuroectodermal tumours of the CNS 1, they share similar clinical and imaging characteristics and are primarily an intra-axial tumour of young children occurring in the cerebral and cerebellar hemispheres 2. As yet only a small numbers of cases that include imaging findings have been reported in the literature 3."
"Tumours showing BCOR-ITD are more common in males (male to female ratio of 1.5) with an average age at diagnosis of 4.5 years 4. While the majority of cases occur in children, some cases in young adults have been reported 5."
"Genetically related tumours with BCOR fusion (rather than the tandem duplication) appear to occur with a more even gender distribution and with an older age at presentation 4."
"The causative recurrent internal tandem duplication occurs within exon 15 of the BCOR transcriptional co‐repressor gene 1. Somatic alterations in BCOR are associated with a variety of tumour types, with internal tandem duplications thought to be the driving mutation behind clear cell sarcomas of the kidney and the sarcoma with BCOR genetic alteration 2."
"CNS tumours showing BCOR-ITD are typically densely cellular and well-circumscribed from the normal brain parenchyma 2,3,5, though occasionally showing infiltration at their interface with the surrounding brain 2. Tumour cells have round to oval nuclei. Ependymoma-like perivascular pseudorosettes, palisading necrosis and a dense capillary network without microvascular proliferation have been described as features 2,5."
"GFAP: majority of tumour cells negative 2,5"
"atypical teratoid / rhabdoid tumour (AT/RT)"
"atypical teratoid / rhabdoid tumour (AT/RT)"
"SSH subtype are typically peripherally located but show a more diffuse nodular pattern of growth"
"NeuN: positive in most tumours with variable labelling of cells 2"
"A case series of 10 patients published by Cardoen et al 3 described peripheral well-defined intra-axial masses occurring in both the cerebral and cerebellar hemispheres. The tumours were large at presentation and commonly abutted the dura without invasion. Areas of central necrosis were common, with some of the masses showing calcifications or areas of haemorrhagic change. The masses demonstrated minimal to no surrounding vasogenic oedema and only mild heterogenous enhancement. In 9 of 10 cases, large vessels draining to overlying cortical veins were seen within the tumour. Similar imaging findings were described in a case series of 10 additional patients published by Ferris et al 2."
"T1 C+ (Gd): mild heterogenous enhancement"
"similar imaging features, including intra-tumoural veins, restricted diffusion, minimal or absent oedema and frequent calcifications"
"Clinical symptoms are those of an intracranial mass lesion and include headaches alongside other symptoms of raised intracranial pressure, seizures and focal neurologic deficits 2,3."
"similar imaging features, including intra-tumoural veins, restricted diffusion, minimal or absent oedema and frequent calcifications"