Literature DB >> 26090549

Embryonal tumor with abundant neuropil and true rosettes in the brainstem: case report.

Hidetoshi Sato1, Yuzo Terakawa1, Naohiro Tsuyuguchi1, Yuko Kuwae2, Masahiko Ohsawa2, Kenji Ohata1.   

Abstract

Embryonal tumor with abundant neuropil and true rosettes (ETANTR) is rarely seen in the brainstem, and there are few case reports of brainstem ETANTR in the literature. Accordingly, the characteristics and the role of surgical treatment of this rare entity remain unclear. The authors present a case of brainstem ETANTR involving a 33-month-old boy along with a review of the literature and discuss the role of surgical removal in the treatment of this entity. In the authors' case, the tumor was surgically treated with subtotal resection, which resulted in improvement of the patient's preoperative symptoms. Chemotherapy was initiated but did not appear to be effective, radiotherapy was declined, and the boy died 6 months after the operation. Based on their analysis of 10 previously reported cases and their own case, the authors conclude that, with respect to survival, surgery may be beneficial even in cases of ETANTR in the brainstem. They note, however, that further studies with a large number of cases are needed to validate the role of surgical treatment in brainstem ETANTR.

Entities:  

Keywords:  EBL = ependymoblastoma; EMA = epithelial membrane antigen; ETANTR; ETANTR = embryonal tumor with abundant neuropil and true rosettes; ETMR; ETMR = embryonal tumor with multilayered rosette; FISH = fluorescence in situ hybridization; GFAP = glial fibrillary acidic protein; ICE = a combination of ifosfamide, carboplatin, and etoposide; INI-1 = integrase interactor 1; LIN28A = Lin-28 homolog A; NeuN = neuronal nuclei; PET = positron emission tomography; VDCy = a combination of vincristine, doxorubicin, and cyclophosphamide; brainstem; embryonal tumor; oncology; surgical treatment

Mesh:

Substances:

Year:  2015        PMID: 26090549     DOI: 10.3171/2015.3.PEDS14727

Source DB:  PubMed          Journal:  J Neurosurg Pediatr        ISSN: 1933-0707            Impact factor:   2.375


  4 in total

1.  CT and Multimodal MR Imaging Features of Embryonal Tumors with Multilayered Rosettes in Children.

Authors:  V Dangouloff-Ros; A Tauziède-Espariat; C-J Roux; R Levy; D Grévent; F Brunelle; A Gareton; S Puget; K Beccaria; T Blauwblomme; J Grill; C Dufour; P Varlet; N Boddaert
Journal:  AJNR Am J Neuroradiol       Date:  2019-03-07       Impact factor: 3.825

2.  Embryonal tumor with multilayered rosettes, C19MC-altered (ETMR): a newly defined pediatric brain tumor.

Authors:  Yu-Chun Pei; Guo-Hao Huang; Xiao-Hong Yao; Xiu-Wu Bian; Fei Li; Yan Xiang; Lin Yang; Sheng-Qing Lv; Jun Liu
Journal:  Int J Clin Exp Pathol       Date:  2019-08-01

3.  A mouse model for embryonal tumors with multilayered rosettes uncovers the therapeutic potential of Sonic-hedgehog inhibitors.

Authors:  Julia E Neumann; Annika K Wefers; Sander Lambo; Edoardo Bianchi; Marie Bockstaller; Mario M Dorostkar; Valerie Meister; Pia Schindler; Andrey Korshunov; Katja von Hoff; Johannes Nowak; Monika Warmuth-Metz; Marlon R Schneider; Ingrid Renner-Müller; Daniel J Merk; Mehdi Shakarami; Tanvi Sharma; Lukas Chavez; Rainer Glass; Jennifer A Chan; M Mark Taketo; Philipp Neumann; Marcel Kool; Ulrich Schüller
Journal:  Nat Med       Date:  2017-09-11       Impact factor: 53.440

4.  Radiation for ETMR: Literature review and case series of patients treated with proton therapy.

Authors:  Sergio Jaramillo; David R Grosshans; Nancy Philip; Ali Varan; Canan Akyüz; Mary Frances McAleer; Anita Mahajan; Susan L McGovern
Journal:  Clin Transl Radiat Oncol       Date:  2018-11-07
  4 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.