Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress
Clinical Collections
Advances in Alzheimer’s

At a glance: The STEP trials

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.
ADVANCED SEARCH
Log in
Top
Strahlentherapie und Onkologie
Published in: Strahlentherapie und Onkologie 8/2016

01-08-2016 | Original Article

Long-term survival following additive radiotherapy in patients with atypical teratoid rhabdoid tumors

Authors: Khaled Elsayad, MD, Jan Kriz, MD, Laith Samhouri, MD, Uwe Haverkamp, MD PhD, Ronald Straeter, MD PhD, Walter Stummer, MD PhD, Hans Theodor Eich, MD PhD

Published in: Strahlentherapie und Onkologie | Issue 8/2016

Login to get access

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.
Appendix
Available only for authorised users
Literature
1.
Bishop AJ, McDonald MW, Chang AL et al (2012) Infant brain tumors. Incidence, survival, and the role of radiation based on surveillance, epidemiology, and end results (SEER) data. Int J Radiat Oncol Biol Phys 82(1):341–347. doi:10.1016/j.ijrobp.2010.08.020CrossRefPubMed
2.
Buscariollo DL, Park HS, Roberts KB et al (2012) Survival outcomes in atypical teratoid rhabdoid tumor for patients undergoing radiotherapy in a surveillance, epidemiology, and end results analysis. Cancer 118(17):4212–4219. doi:10.1002/cncr.27373CrossRefPubMed
3.
Lau CS, Mahendraraj K, Chamberlain RS (2015) Atypical teratoid rhabdoid tumors: a population-based clinical outcomes study involving 174 patients from the surveillance, epidemiology, and end results database (1973–2010). Cancer Manag Res 7:301–309. doi:10.2147/CMAR.S88561PubMedPubMedCentral
4.
Sinha P, Ahmad M, Varghese A et al (2015) Atypical teratoid rhabdoid tumour of the spine: report of a case and literature review. Eur Spine J 24:S472–S484. doi:10.1007/s00586-014-3445-1CrossRefPubMed
5.
Schneiderhan TM, Beseoglu K, Bergmann M et al (2011) Sellar atypical teratoid/rhabdoid tumours in adults. Neuropathol Appl Neurobiol 37(3):326–329. doi:10.1111/j.1365-2990.2010.01111.xCrossRefPubMed
6.
Packer RJ, Biegel JA, Blaney S et al (2002) Atypical teratoid/rhabdoid tumor of the central nervous system: report on workshop. J Pediatr Hematol Oncol 24(5):337–342. doi:10.1097/00043426-200206000-00004CrossRefPubMed
7.
Takahashi K, Nishihara H, Katoh M et al (2011) A case of atypical teratoid/rhabdoid tumor in an adult, with long survival. Brain Tumor Pathol 28(1):71–76. doi:10.1007/s10014-010-0008-yCrossRefPubMed
8.
Lee Y, Yang Y, Huang M et al (2014) MicroRNA142-3p promotes tumor-initiating and radioresistant properties in malignant pediatric brain tumors. Cell Transplant 23(4):669–690. doi:10.3727/096368914X678364CrossRefPubMed
9.
Venkataraman S, Alimova I, Tello T et al (2012) Targeting Aurora Kinase A enhances radiation sensitivity of atypical teratoid rhabdoid tumor cells. J Neurooncol 107(3):517–526. doi:10.1007/s11060-011-0795-yCrossRefPubMedPubMedCentral
10.
Alimova I, Birks DK, Harris PS et al (2013) Inhibition of EZH2 suppresses self-renewal and induces radiation sensitivity in atypical rhabdoid teratoid tumor cells. Neuro-oncology 15(2):149–160. doi:10.1093/neuonc/nos285CrossRefPubMed
11.
Knipstein JA, Birks DK, Donson AM et al (2012) Histone deacetylase inhibition decreases proliferation and potentiates the effect of ionizing radiation in atypical teratoid/rhabdoid tumor cells. Neuro-oncology 14(2):175–183. doi:10.1093/neuonc/nor208CrossRefPubMed
12.
Morgenstern DA, Gibson S, Brown T et al (2010) Clinical and pathological features of paediatric malignant rhabdoid tumours. Pediatr Blood Cancer 54(1):29–34. doi:10.1002/pbc.22231CrossRefPubMed
13.
Chi SN, Zimmerman MA, Yao X et al (2008) Intensive multimodality treatment for children with newly diagnosed CNS atypical teratoid rhabdoid tumor. J Clin Oncol 27(3):385–389. doi:10.1200/JCO.2008.18.7724CrossRefPubMed
14.
Park HG, Yoon JH, Kim SH et al (2014) Adult-onset sellar and suprasellar atypical teratoid Rhabdoid tumor treated with a multimodal approach. A case report. Brain Tumor Res Treat 2(2):108 doi:10.14791/btrt.2014.2.2.108CrossRefPubMedPubMedCentral
15.
Lafay-Cousin L, Fay-McClymont T, Johnston D et al (2015) Neurocognitive evaluation of long term survivors of atypical teratoid rhabdoid tumors (ATRT). The Canadian registry experience. Pediatr Blood Cancer 62(7):1265–1269. doi:10.1002/pbc.25441CrossRefPubMed
16.
Slavc I, Chocholous M, Leiss U et al (2014) Atypical teratoid rhabdoid tumor. Improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna experience 1992–2012. Cancer Med 3(1):91–100. doi:10.1002/cam4.161CrossRefPubMed
17.
Verma V, Johnson CP, Bennion NR et al (2015) Atypical teratoid rhabdoid tumor: long-term survival after chemoradiotherapy. Childs Nerv Syst. doi:10.1007/s00381-015-2723-5PubMed
18.
Bouvier C, Paula AM de, Fernandez C et al (2008) Atypical teratoid/rhabdoid tumour. 7‑year event-free survival with gross total resection and radiotherapy in a 7‑year-old boy. Childs Nerv Syst 24(1):143–147. doi:10.1007/s00381-007-0438-yCrossRefPubMed
19.
Tekautz TM, Fuller CE, Blaney S et al (2005) Atypical teratoid/rhabdoid tumors (ATRT): improved survival in children 3 years of age and older with radiation therapy and high-dose alkylator-based chemotherapy. J Clin Oncol 23(7):1491–1499. doi:10.1200/JCO.2005.05.187CrossRefPubMed
20.
Squire SE, Chan MD, Marcus KJ (2007) Atypical teratoid/rhabdoid tumor: the controversy behind radiation therapy. J Neurooncol 81(1):97–111. doi:10.1007/s11060-006-9196-zCrossRefPubMed
21.
Patel S, Bhatnagar A, Wear C et al (2014) Are pediatric brain tumors on the rise in the USA? Significant incidence and survival findings from the SEER database analysis. Childs Nerv Syst 30(1):147–154. doi:10.1007/s00381-013-2307-1CrossRefPubMed
22.
Combs SE, Nüsslin F, Wilkens JJ (2016) Individualized radiotherapy by combining high-end irradiation and magnetic resonance imaging. Strahlenther Onkol 192(4):209–215. doi:10.1007/s00066016-0944-5CrossRefPubMed
23.
Takahashi-Fujigasaki J, Matumoto M, Kan I et al (2012) Atypical teratoid/rhabdoid tumor with 26-year overall survival: case report. J Neurosurg Pediatr 9(4):400–405. doi:10.3171/2012.1.PEDS11350CrossRefPubMed
24.
Benesch M, Bartelheim K, Fleischhack G et al (2014) High-dose chemotherapy (HDCT) with auto-SCT in children with atypical teratoid/rhabdoid tumors (AT/RT): a report from the European Rhabdoid Registry (EU-RHAB). Bone Marrow Transplant 49(3):370–375. doi:10.1038/bmt.2013.208CrossRefPubMed
25.
Hoff K von, Hinkes B, Dannenmann-Stern E et al (2011) Frequency, risk-factors and survival of children with atypical teratoid rhabdoid tumors (AT/RT) of the CNS diagnosed between 1988 and 2004, and registered to the German HIT database. Pediatr Blood Cancer 57(6):978–985. doi:10.1002/pbc.23236CrossRef
26.
Peters O, Marienhagen J, Stadler P et al (2007) Combined multimodality therapy for pedaitric central nervous system atypical teratoid/rhabdoid tumor: an interim analysis of the German ATRT/CNS pilot study. Abstract 148. Neuro-oncology 9(2):169–221. doi:10.1215/15228517-2006-039CrossRef
27.
Park ES, Sung KW, Baek HJ et al (2012) Tandem high-dose chemotherapy and autologous stem cell transplantation in young children with atypical teratoid/rhabdoid tumor of the central nervous system. J Korean Med Sci 27(2):135 doi:10.3346/jkms.2012.27.2.135CrossRefPubMedPubMedCentral
28.
Schrey D, Carceller Lechón F, Malietzis G et al (2015) Multimodal therapy in children and adolescents with newly diagnosed atypical teratoid rhabdoid tumor: individual pooled data analysis and review of the literature. J Neurooncol. doi:10.1007/s11060-015-1904-0PubMed
29.
Athale UH, Duckworth J, Odame I et al (2009) Childhood atypical teratoid rhabdoid tumor of the central nervous system a meta-analysis of observational studies. J Pediatr Hematol Oncol 31(9):651–663CrossRefPubMed
30.
Blaney SM, Kocak M, Gajjar A et al (2012) Pilot study of systemic and intrathecal mafosfamide followed by conformal radiation for infants with intracranial central nervous system tumors. A Pediatric Brain Tumor Consortium Study (PBTC-001). J Neurooncol 109(3):565–571. doi:10.1007/s11060-012-0929-xCrossRefPubMedPubMedCentral
31.
Kocher M, Wittig A, Piroth MD et al (2014) Stereotactic radiosurgery for treatment of brain metastases. A report of the DEGRO Working Group on stereotactic radiotherapy. Strahlenther Onkol 190(6):521–532. doi:10.1007/s00066-014-0648-7CrossRefPubMed
32.
Hirth A, Pedersen PH, Wester K et al (2003) Cerebral atypical teratoid/rhabdoid tumor of infancy: long-term survival after multimodal treatment, also including triple intrathecal chemotherapy and gamma knife radiosurgery – case report. Pediatr Hematol Oncol 20(4):327–332. doi:10.1080/08880010390203116CrossRefPubMed
33.
Timmermann B (2015) Proton therapy of brain tumors: chance or risk? Strahlenther Onkol 191(8):689–690
34.
Perreault S, Lober RM, Carret A et al (2013) Relapse patterns in pediatric embryonal central nervous system tumors. J Neurooncol 115(2):209–215. doi:10.1007/s11060-013-1213-4CrossRefPubMed
35.
Pixberg C, Koch R, Eich HT et al (2016) Acute toxicity grade 3 and 4 after irradiation in children and adolescents. Results from the IPPARCA collaboration. Int J Radiat Oncol Biol Phys 94(4):792–799. doi:10.1016/j.ijrobp.2015.12.353CrossRefPubMed
36.
Padovani L, André N, Constine LS et al (2012) Neurocognitive function after radiotherapy for paediatric brain tumours. Nat Rev Neurol 8(10):578–588. doi:10.1038/nrneurol.2012.182CrossRefPubMed
37.
Weiler LJ, Pletschko T, Schwarzinger A et al (2015) Attention functioning following radiotherapy in children with brain tumors. Strahlenther Onkol 191(8):689
38.
Shitara S, Akiyama Y (2014) Atypical teratoid/rhabdoid tumor in sellar turcica in an adult: a case report and review of the literature. Surg Neurol Int 5:75 doi:10.4103/2152-7806.133105CrossRefPubMedPubMedCentral
39.
Gorayski P, Boros S, Ong B et al (2013) Radiation-induced primary cerebral atypical teratoid/rhabdoid tumour in an adult. J Clin Neurosci 20(10):1466–1468. doi:10.1016/j.jocn.2013.03.041CrossRefPubMed
40.
Leon-Bojorge B de, Rueda-Franco F, Anaya-Jara M (2008) Central nervous system atypical teratoid rhabdoid tumor: experience at the National Institute of Pediatrics, Mexico City. Childs Nerv Syst 24(3):307–312. doi:10.1007/s00381-007-0464-9CrossRefPubMed
41.
Arita K, Sugiyama K, Sano T et al (2008) Atypical teratoid/rhabdoid tumour in sella turcica in an adult. Acta Neurochir (Wien) 150(5):491–496. doi:10.1007/s00701-008-1500-yCrossRef
42.
Makuria AT, Rushing EJ, McGrail KM et al (2008) Atypical teratoid rhabdoid tumor (AT/RT) in adults: review of four cases. J Neurooncol 88(3):321–330. doi:10.1007/s11060-008-9571-zCrossRefPubMed
43.
Chen YW, Wong TT, Ho DM et al (2006) Impact of radiotherapy for pediatric CNS atypical teratoid/rhabdoid tumor (single institute experience). Int J Radiat Oncol Biol Phys 64(4):1038–1043. doi:10.1016/j.ijrobp.2005.10.001CrossRefPubMed
44.
Raisanen J, Biegel JA, Judkins A et al (2005) Chromosome 22q deletions in atypical teratoid/rhabdoid tumors in adults. Brain Pathol 15(1):23–28CrossRefPubMed
45.
Zimmerman MA, Goumnerova LC, Proctor M et al (2005) Continuous remission of newly diagnosed and relapsed central nervous system atypical teratoid/rhabdoid tumor. J Neurooncol 72(1):77–84. doi:10.1007/s11060-004-3115-yCrossRefPubMed
46.
Arrazola J, Pedrosa I, Mendez R et al (2000) Primary malignant rhabdoid tumour of the brain in an adult. Neuroradiology 42(5):363–367CrossRefPubMed
47.
Byram D (1999) Regarding Weiss et al., IJROBP 41:103–109; 1998. Int J Radiat Oncol Biol Phys 45(1):247CrossRefPubMed
48.
Weinblatt M, Kochen J (1992) Rhabdoid tumor of the central nervous system. Med Pediatr Oncol 20(3):258CrossRefPubMed
49.
Horn M, Schlote W, Lerch KD et al (1992) Malignant rhabdoid tumor: primary intracranial manifestation in an adult. Acta Neuropathol 83(4):445–448CrossRefPubMed
Metadata
Title
Long-term survival following additive radiotherapy in patients with atypical teratoid rhabdoid tumors
Authors
Khaled Elsayad, MD
Jan Kriz, MD
Laith Samhouri, MD
Uwe Haverkamp, MD PhD
Ronald Straeter, MD PhD
Walter Stummer, MD PhD
Hans Theodor Eich, MD PhD
Publication date
01-08-2016
Publisher
Springer Berlin Heidelberg
Published in
Strahlentherapie und Onkologie / Issue 8/2016
Print ISSN: 0179-7158
Electronic ISSN: 1439-099X
DOI
https://doi.org/10.1007/s00066-016-0978-8

Other articles of this Issue 8/2016

Strahlentherapie und Onkologie 8/2016 Go to the issue

Nachruf

Zur Erinnerung an Prof. Dr. med. Wolfgang Hinkelbein

Literatur kommentiert

Restaging-PET-CT nach Radiochemotherapie kann Patienten mit fortgeschrittenen HNO-Karzinomen die elektive Neck-Dissektion ersparen, sofern sie eine komplette Remission zeigen

Original Article

The field size matters: low dose external beam radiotherapy for thumb carpometacarpal osteoarthritis

Literatur kommentiert

Keine generelle Empfehlung zum Verzicht auf eine konsolidierende Radiotherapie bei Morbus Hodgkin und PET-negativem Befund nach abgeschlossener Chemotherapie

Original Article

IMRT/VMAT for malignancies in the head-and-neck region

Original Article

Evaluation of radiotherapy techniques for radical treatment of lateralised oropharyngeal cancers