Clinical Characteristics of Subependymal Giant Cell Astrocytoma in Tuberous Sclerosis Complex

BACKGROUND: This study evaluated the characteristics of subependymal giant cell astrocytoma (SEGA) in patients with tuberous sclerosis complex (TSC) entered into the TuberOus SClerosis registry to increase disease Awareness (TOSCA). METHODS: The study was conducted at 170 sites across 31 countries. Data from patients of any age with a documented clinical visit for TSC in the 12 months preceding enrollment or those newly diagnosed with TSC were entered. RESULTS: SEGA were reported in 554 of 2,216 patients (25%). Median age at diagnosis of SEGA was 8 years (range, 18 years. SEGA were symptomatic in 42.1% of patients. Symptoms included increased seizure frequency (15.8%), behavioural disturbance (11.9%), and regression/loss of cognitive skills (9.9%), in addition to those typically associated with increased intracranial pressure. SEGA were significantly more frequent in patients with TSC2 compared to TSC1 variants (33.7 vs. 13.2 %, p < 0.0001). Main treatment modalities included surgery (59.6%) and mammalian target of rapamycin (mTOR) inhibitors (49%). CONCLUSIONS: Although SEGA diagnosis and growth typically occurs during childhood, SEGA can occur and grow in both infants and adults

Zirconolite, zircon and monazite-(Ce) U-Th-Pb age constraints on the emplacement, deformation and alteration history of the Cummins Range Carbonatite Complex, Halls Creek Orogen, Kimberley region, Western Australia

In situ SHRIMP U-Pb dating of zirconolite in clinopyroxenite from the Cummins Range Carbonatite Complex, situated in the southern Halls Creek Orogen, Kimberley region, Western Australia, has provided a reliable 207Pb/206Pb age of emplacement of 1009 ± 16 Ma. Variably metamict and recrystallised zircons from co-magmatic carbonatites, including a megacryst ~1.5 cm long, gave a range of ages from ~1043–998 Ma, reflecting partial isotopic resetting during post-emplacement deformation and alteration. Monazite-(Ce) in a strongly foliated dolomite carbonatite produced U-Th-Pb dates ranging from ~900–590 Ma. Although the monazite-(Ce) data cannot give any definitive ages, they clearly reflect a long history of hydrothermal alteration/recrystallisation, over at least 300 million years. This is consistent with the apparent resetting of the Rb-Sr and K-Ar isotopic systems by a post-emplacement thermal event at ~900 Ma during the intracratonic Yampi Orogeny. The emplacement of the Cummins Range Carbonatite Complex probably resulted from the reactivation of a deep crustal structure within the Halls Creek Orogen during the amalgamation of Proterozoic Australia with Rodinia over the period ~1000–950 Ma. This may have allowed an alkaline carbonated silicate magma that was parental to the Cummins Range carbonatites, and generated by redox and/or decompression partial melting of the asthenospheric mantle, to ascend from the base of the continental lithosphere along the lithospheric discontinuity constituted by the southern edge of the Halls Creek Orogen. There is no evidence of a link between the emplacement of the Cummins Range Carbonatite Complex and mafic large igneous province magmatism indicative of mantle plume activity. Rather, patterns of Proterozoic alkaline magmatism in the Kimberley Craton may have been controlled by changing plate motions during the Nuna–Rodinia supercontinent cycles (~1200–800 Ma)

Stable H–C–O isotope and trace element geochemistry of the Cummins Range Carbonatite Complex, Kimberley region, Western Australia: implications for hydrothermal REE mineralization, carbonatite evolution and mantle source regions

The Neoproterozoic Cummins Range Carbonatite Complex (CRCC) is situated in the southern Halls Creek Orogen adjacent to the Kimberley Craton in northern Western Australia. The CRCC is a composite, subvertical to vertical stock ∼2 km across with a rim of phlogopite–diopside clinopyroxenite surrounding a plug of calcite carbonatite and dolomite carbonatite dykes and veins that contain variable proportions of apatite–phlogopite–magnetite ± pyrochlore ± metasomatic Na–Ca amphiboles ± zircon. Early high-Sr calcite carbonatites (4,800–6,060 ppm Sr; La/YbCN = 31.6–41.5; δ13C = −4.2 to −4.0 ‰) possibly were derived from a carbonated silicate parental magma by fractional crystallization. Associated high-Sr dolomite carbonatites (4,090–6,310 ppm Sr; La/YbCN = 96.5–352) and a late-stage, narrow, high rare earth element (REE) dolomite carbonatite dyke (La/YbCN = 2756) define a shift in the C–O stable isotope data (δ18O = 7.5 to 12.6 ‰; δ13C = −4.2 to −2.2 ‰) from the primary carbonatite field that may have been produced by Rayleigh fractionation with magma crystallization and cooling or through crustal contamination via fluid infiltration. Past exploration has focussed primarily on the secondary monazite-(Ce)-rich REE and U mineralization in the oxidized zone overlying the carbonatite. However, high-grade primary hydrothermal REE mineralization also occurs in narrow (<1 m wide) shear-zone hosted lenses of apatite–monazite-(Ce) and foliated monazite-(Ce)–talc rocks (≤∼25.8 wt% total rare earth oxide (TREO); La/YbCN = 30,085), as well as in high-REE dolomite carbonatite dykes (3.43 wt% TREO), where calcite, parisite-(Ce) and synchysite-(Ce) replace monazite-(Ce) after apatite. Primary magmatic carbonatites were widely hydrothermally dolomitized to produce low-Sr dolomite carbonatite (38.5–282 ppm Sr; La/YbCN = 38.4–158.4; δ18O = 20.8 to 21.9 ‰; δ13C = −4.3 to −3.6 ‰) that contains weak REE mineralization in replacement textures, veins and coating vugs. The relatively high δD values (−54 to −34 ‰) of H2O derived from carbonatites from the CRCC indicate that the fluids associated with carbonate formation contained a significant amount of crustal component in accordance with the elevated δ13C values (∼−4 ‰). The high δD and δ13C signature of the carbonatites may have been produced by CO2–H2O metasomatism of the mantle source during Paleoproterozoic subduction beneath the eastern margin of the Kimberley Craton