| Title: |
Newly Diagnosed and Growing Subependymal Giant Cell Astrocytoma in Adults With Tuberous Sclerosis Complex: Results From the International TOSCA Study |
| Authors: |
Jansen, AC; Belousova, E; Benedik, MP; Carter, T; Cottin, V; Curatolo, P; D'Amato, L; d'Augeres, GB; de Vries, PJ; Ferreira, JC; Feucht, M; Fladrowski, C; Hertzberg, C; Jozwiak, S; Lawson, JA; Macaya, A; Marques, R; Nabbout, R; O'Callaghan, F; Qin, J; Sander, V; Sauter, M; Shah, S; Takahashi, Y; Touraine, R; Youroukos, S; Zonnenberg, B; Kingswood, JC; Shinohara, N; Horie, S; Kubota, M; Tohyama, J; Imai, K; Kaneda, M; Kaneko, H; Uchida, Y; Kirino, T; Endo, S; Inoue, Y; Uruno, K; Serdaroglu, A; Yapici, Z; Anlar, B; Altunbasak, S; Lvova, O; Belyaev, OV; Agranovich, O; Levitina, EV; Maksimova, YV; Karas, A; Jiang, Y; Zou, L; Xu, K; Zhang, Y; Luan, G; Wang, Y; Jin, M; Ye, D; Liao, W; Zhou, L; Liu, J; Liao, J; Yan, B; Deng, Y; Jiang, L; Liu, Z; Huang, S; Li, H; Kim, K; Chen, P-L; Lee, H-F; Tsai, J-D; Chi, C-S; Huang, C-C; Riney, K; Yates, D; Kwan, P; Likasitwattanakul, S; Nabangchang, C; Chomtho, LTK; Katanyuwong, K; Sriudomkajorn, S; Wilmshurst, J; Segel, R; Gilboa, T; Tzadok, M; Fattal-Valevski, A; Papathanasopoulos, P; Papavasiliou, AS; Giannakodimos, S; Gatzonis, S; Pavlou, E; Tzoufi, M; Vergeer, AMH; Dhooghe, M; Verhelst, H; Roelens, F; Nassogne, MC; Defresne, P; De Waele, L; Leroy, P; Demonceau, N; Legros, B; Van Bogaert, P; Ceulemans, B; Dom, L; Castelnau, P; Martin, ADS; Riquet, A; Milh, M; Cances, C; Pedespan, J-M; Ville, D; Roubertie, A; Auvin, S; Berquin, P; Richelme, C; Allaire, C; Gueden, S; Sylvie, NTT; Godet, B; Falco Rojas, MLR; Campistol Planas, J; Martinez Bermejo, A; Smeyers Dura, P; Roldan Aparicio, S; Martinez Gonzalez, MJ; Lopez Pison, J; Blanco Barca, MO; Lopez Laso, E; Alonso Luengo, O; Aguirre Rodriguez, FJ; Malaga Dieguez, I; Camacho Salas, A; Marti Carrera, I; Martinez Salcedo, E; Yoldi Petri, ME; Cancho Candela, R; Carrilho, IDC; Vieira, JP; da Silva Oliveira Monteiro, JP; de Oliveira Ferreira Leao, MJS; Marceano Ribeiro Luis, CS; Mendonca, CP; Endziniene, M; Strautmanis, J; Talvik, I; Canevini, MP; Gambardella, A; Pruna, D; Buono, S; Fontana, E; Dalla Bernardina, B; Burloiu, C; Cosma, ISB; Vintan, MA; Popescu, L; Zitterbart, K; Payerova, J; Bratsky, L; Zilinska, Z; Gruber-Sedlmayr, U; Baumann, M; Haberlandt, E; Rostasy, K; Pataraia, E; Elmslie, F; Johnston, CA; Crawford, P; Uldall, P; Dahlin, M; Uvebrant, P; Rask, O; Bjoernvold, M; Brodtkorb, E; Sloerdahl, A; Solhoff, R; Jaatun, MSG; Mandera, M; Radzikowska, EJ; Wysocki, M; Fischereder, M; Kurlemann, G; Wilken, B; Wiemer-Kruel, A; Budde, K; Marquard, K; Knuf, M; Hahn, A; Hartmann, H; Merkenschlager, A; Trollmann, R |
| Source: |
Frontiers in Neurology , 10 , Article 821. (2019) |
| Publisher Information: |
FRONTIERS MEDIA SA |
| Publication Year: |
2019 |
| Collection: |
University College London: UCL Discovery |
| Subject Terms: |
mTOR; registry; SEGA; TOSCA; tuberous sclerosis complex |
| Description: |
The onset and growth of subependymal giant cell astrocytoma (SEGA) in tuberous sclerosis complex (TSC) typically occurs in childhood. There is minimal information on SEGA evolution in adults with TSC. Of 2,211 patients enrolled in TOSCA, 220 of the 803 adults (27.4%) ever had a SEGA. Of 186 patients with SEGA still ongoing in adulthood, 153 (82.3%) remained asymptomatic, and 33 (17.7%) were reported to ever have developed symptoms related to SEGA growth. SEGA growth since the previous scan was reported in 39 of the 186 adults (21%) with ongoing SEGA. All but one patient with growing SEGA had mutations in TSC2. Fourteen adults (2.4%) were newly diagnosed with SEGA during follow-up, and majority had mutations in TSC2. Our findings suggest that surveillance for new or growing SEGA is warranted also in adulthood, particularly in patients with mutations in TSC2. |
| Document Type: |
article in journal/newspaper |
| File Description: |
text |
| Language: |
English |
| Relation: |
https://discovery.ucl.ac.uk/id/eprint/10080432/ |
| Availability: |
https://discovery.ucl.ac.uk/id/eprint/10080432/1/fneur-10-00821.pdf; https://discovery.ucl.ac.uk/id/eprint/10080432/ |
| Rights: |
open |
| Accession Number: |
edsbas.AC876659 |
| Database: |
BASE |