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.
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.