Khaled Elsayad1, Jan Kriz2, Laith Samhouri2, Uwe Haverkamp2, Ronald Straeter3, Walter Stummer4, Hans Theodor Eich2. 1. Radiation oncology department, University Hospital Münster, Albert-Schweitzer-Campus 1, Building A1, 48149, Münster, Germany. Khaled.Elsayad@uni-muenster.de. 2. Radiation oncology department, University Hospital Münster, Albert-Schweitzer-Campus 1, Building A1, 48149, Münster, Germany. 3. Pediatric oncology department, University Hospital Münster, Münster, Germany. 4. Neurosurgery department, University Hospital Münster, Münster, Germany.
Abstract
BACKGROUND: Atypical teratoid rhabdoid tumor (ATRT) is a highly aggressive disease of embryonic origin accounting for <5% of all pediatric central nervous system (CNS) tumors. PATIENTS AND METHODS: We describe a series of five cases of CNS ATRT. The first three patients underwent subtotal tumor resection. Gross total resection of the tumor was achieved in the fourth and fifth patients. Only 4 patients received chemotherapy, whereas all 5 patients received additive radiotherapy (RT). The latter included three dimensional (3D) conformal RT or intensity modulated RT (IMRT) with a median dose of 54 Gy (range 50.4-59.0 Gy) applied in daily fractions of 1.8 Gy. The median interval between surgery and RT was 5 months (range 2-11 months). RESULTS: Two months after completion of RT, 4 patients had achieved complete radiologic remission. The median event-free survival period was 46 months (range 10-90 months). However, the first patient died 17 months after developing an out-of-field recurrence. The third patient developed a recurrence 11 months after salvage RT. The other 3 patients (cases 2, 4, and 5) remain alive with no evidence of disease 59, 46 and 90 months after therapy, respectively. CONCLUSION: Overall, the 5 patients survived for a median of 48 months (range 25-90 months) from the time of initial diagnosis and they tolerated the RT well, without severe acute or late onset toxicities. The results imply a potential survival gain after irradiation at acceptable toxicity level.
BACKGROUND: Atypical teratoid rhabdoid tumor (ATRT) is a highly aggressive disease of embryonic origin accounting for <5% of all pediatric central nervous system (CNS) tumors. PATIENTS AND METHODS: We describe a series of five cases of CNS ATRT. The first three patients underwent subtotal tumor resection. Gross total resection of the tumor was achieved in the fourth and fifth patients. Only 4 patients received chemotherapy, whereas all 5 patients received additive radiotherapy (RT). The latter included three dimensional (3D) conformal RT or intensity modulated RT (IMRT) with a median dose of 54 Gy (range 50.4-59.0 Gy) applied in daily fractions of 1.8 Gy. The median interval between surgery and RT was 5 months (range 2-11 months). RESULTS: Two months after completion of RT, 4 patients had achieved complete radiologic remission. The median event-free survival period was 46 months (range 10-90 months). However, the first patient died 17 months after developing an out-of-field recurrence. The third patient developed a recurrence 11 months after salvage RT. The other 3 patients (cases 2, 4, and 5) remain alive with no evidence of disease 59, 46 and 90 months after therapy, respectively. CONCLUSION: Overall, the 5 patients survived for a median of 48 months (range 25-90 months) from the time of initial diagnosis and they tolerated the RT well, without severe acute or late onset toxicities. The results imply a potential survival gain after irradiation at acceptable toxicity level.
Entities:
Keywords:
ATRT; Central nervous system; Children; Intensity-modulated radiotherapy; Tomotherapy; Toxicity
Authors: T M Schneiderhan; K Beseoglu; M Bergmann; U Neubauer; S Macht; D Hänggi; G Reifenberger; M J Riemenschneider Journal: Neuropathol Appl Neurobiol Date: 2011-04 Impact factor: 8.090
Authors: Andrew J Bishop; Mark W McDonald; Andrew L Chang; Natia Esiashvili Journal: Int J Radiat Oncol Biol Phys Date: 2010-10-29 Impact factor: 7.038
Authors: Lucie Lafay-Cousin; Taryn Fay-McClymont; Donna Johnston; Chris Fryer; Katrin Scheinemann; Adam Fleming; Juliette Hukin; Laura Janzen; Sharon Guger; Douglas Strother; Donald Mabbott; Annie Huang; Eric Bouffet Journal: Pediatr Blood Cancer Date: 2015-03-18 Impact factor: 3.167
Authors: M Benesch; K Bartelheim; G Fleischhack; B Gruhn; P G Schlegel; O Witt; K D Stachel; H Hauch; C Urban; F Quehenberger; M Massimino; T Pietsch; M Hasselblatt; F Giangaspero; U Kordes; R Schneppenheim; P Hauser; T Klingebiel; M C Frühwald Journal: Bone Marrow Transplant Date: 2014-01-13 Impact factor: 5.483
Authors: Martin Kocher; Andrea Wittig; Marc Dieter Piroth; Harald Treuer; Heinrich Seegenschmiedt; Maximilian Ruge; Anca-Ligia Grosu; Matthias Guckenberger Journal: Strahlenther Onkol Date: 2014-04-09 Impact factor: 3.621
Authors: D Schrey; F Carceller Lechón; G Malietzis; L Moreno; C Dufour; S Chi; L Lafay-Cousin; K von Hoff; T Athanasiou; L V Marshall; S Zacharoulis Journal: J Neurooncol Date: 2015-11-25 Impact factor: 4.130
Authors: Susan M Blaney; Mehmet Kocak; Amar Gajjar; Murali Chintagumpala; Thomas Merchant; Mark Kieran; Ian F Pollack; Sri Gururangan; Russ Geyer; Peter Phillips; Roger E McLendon; Roger Packer; Stewart Goldman; Anu Banerjee; Richard Heideman; James M Boyett; Larry Kun Journal: J Neurooncol Date: 2012-07-13 Impact factor: 4.130