BACKGROUND: Atypical teratoid/rhabdoid tumor (AT/RT) is an aggressive malignant brain tumor that, since it was first identified, has been treated with aggressive treatment regimens, e.g. high-dose chemotherapy with stem cell rescue and early radiotherapy. We reviewed our experience because of concerns with respect to treatment-related toxicity in our patients. METHODS: Seven patients with a median age at presentation of 18 months were diagnosed with AT/RT between 1996 and 2006. Tumor location was supratentorial in 2 patients, in the posterior fossa in 4 and spinal in 1. Gross total resection was performed in 1 patient, subtotal resection in 5 and biopsy only in 1. Adjuvant treatment consisted of chemotherapy and radiotherapy in 5 patients. RESULTS: Median progression-free survival was 4 months, and median overall survival was 7 months. Two children are alive at 44 and 102 months. Significant surgical and chemotherapy-related morbidity was seen. Biopsy-proven multifocal necrotizing leukoencephalopathy (MNL) was seen in one patient who is alive 44 months after diagnosis. Another patient who was thought to have recurrent tumor in the brainstem 9 months after diagnosis had imaging findings compatible with MNL. CONCLUSION: Although improving results are reported for AT/RT using intensive treatment regimens, treatment-related morbidity is considerable in this young patient population.
BACKGROUND: Atypical teratoid/rhabdoid tumor (AT/RT) is an aggressive malignant brain tumor that, since it was first identified, has been treated with aggressive treatment regimens, e.g. high-dose chemotherapy with stem cell rescue and early radiotherapy. We reviewed our experience because of concerns with respect to treatment-related toxicity in our patients. METHODS: Seven patients with a median age at presentation of 18 months were diagnosed with AT/RT between 1996 and 2006. Tumor location was supratentorial in 2 patients, in the posterior fossa in 4 and spinal in 1. Gross total resection was performed in 1 patient, subtotal resection in 5 and biopsy only in 1. Adjuvant treatment consisted of chemotherapy and radiotherapy in 5 patients. RESULTS: Median progression-free survival was 4 months, and median overall survival was 7 months. Two children are alive at 44 and 102 months. Significant surgical and chemotherapy-related morbidity was seen. Biopsy-proven multifocal necrotizing leukoencephalopathy (MNL) was seen in one patient who is alive 44 months after diagnosis. Another patient who was thought to have recurrent tumor in the brainstem 9 months after diagnosis had imaging findings compatible with MNL. CONCLUSION: Although improving results are reported for AT/RT using intensive treatment regimens, treatment-related morbidity is considerable in this young patient population.
Authors: Irene Slavc; Monika Chocholous; Ulrike Leiss; Christine Haberler; Andreas Peyrl; Amedeo A Azizi; Karin Dieckmann; Adelheid Woehrer; Christina Peters; Georg Widhalm; Christian Dorfer; Thomas Czech Journal: Cancer Med Date: 2013-12-11 Impact factor: 4.452
Authors: Meerim Park; Jung Woo Han; Seung Min Hahn; Jun Ah Lee; Joo-Young Kim; Sang Hoon Shin; Dong-Seok Kim; Hong In Yoon; Kyung Taek Hong; Jung Yoon Choi; Hyoung Jin Kang; Hee Young Shin; Ji Hoon Phi; Seung-Ki Kim; Ji Won Lee; Keon Hee Yoo; Ki Woong Sung; Hong Hoe Koo; Do Hoon Lim; Hyung Jin Shin; Hyery Kim; Kyung-Nam Koh; Ho Joon Im; Seung Do Ahn; Young-Shin Ra; Hee-Jo Baek; Hoon Kook; Tae-Young Jung; Hyoung Soo Choi; Chae-Yong Kim; Hyeon Jin Park; Chuhl Joo Lyu Journal: Cancer Res Treat Date: 2020-10-28 Impact factor: 4.679