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

Abstract

<jats:p>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.</jats:p>

Clinical phenotypes and prognostic features of ETMRs (Embryonal Tumor with Multi-layered Rosettes) a new CNS tumor entity: A Rare Brain Tumor Registry study

“…Similar to smaller reports (14-16), HDC emerged as a positive prognostic factor in our study while treatment with CC correlated with rapid progression in all patients. We observed an OS of 41 ±16%, amongst patients with M0, GTR, non-brain stem tumors treated with HDC alone while EFS and OS of radiated counterparts were not significantly different and suggest HDC may spare radiation in a significant proportion of patients with these favorable clinical features.…”

“…Although ~200 cases of ETMRs are reported to date (12,(14)(15)(16)(17)(18), the incidence of this disease is likely underestimated due to lack of molecular confirmation in most published studies, and reporting of ETMRs as historical histologic diagnostic categories. Furthermore, our analyses based on a large cohort with matched molecular and clinical data indicate a much wider clinical spectrum of ETMRs than previously recognized.…”

“…In addition to providing a much-needed framework for future clinical trials, our comprehensive and unique study of a large cohort of primary ETMRs has immediate practical implications for diagnostic and therapeutic approaches to children with this orphan disease. Although ~200 cases of ETMRs are reported to date (12, 14-18), the incidence of this disease is likely underestimated due to lack of molecular confirmation in most published studies, and reporting of ETMRs as historical histologic diagnostic categories. Furthermore, our analyses based on a large cohort with matched molecular and clinical data indicate a much wider clinical spectrum of ETMRs than previously recognized.…”

“…Due to early studies indicating ETMRs are lethal diseases with <10% overall survival (4, 6) ETMR patients are frequently treated with various aggressive conventional multi-modal and experimental therapies. Although benefits of high dose chemotherapy and radiation has been reported in various small studies and literature reviews, the impact of these treatments (14-16), have been difficult to assess due to limited patient cohorts, inconsistent diagnostic methods for ETMRs and lack of treatment and outcome information for the majority of patients reported to date. In order to inform current clinical practice and future clinical trials, we examined molecular, clinical, treatment and survival features of a large cohort of primary ETMRs patients identified from the Rare Brain Tumor clinical registry (rarebraintumorconsortium.ca) using multiple diagnostic assays.…”

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

“…Other authors have reviewed cases of MEP and EPL described since the 1920s to 1990 [45] , [49] , [50] , [51] . The patient characteristics, treatment, and outcome of these 204 cases plus the seven MD Anderson cases are summarized in Table 3 [1] , [5] , [7] , [9] , [14] , [49] , [50] , [51] , [52] , [53] , [54] , [55] , [56] , [57] , [58] , [59] , [60] , [61] , [62] , [63] , [64] , [65] , [66] , [67] , [68] , [69] , [70] , [71] , [72] , [73] , [74] , [75] , [76] , [77] , [78] , [79] , [80] , [81] , [82] , [83] , [84] , [85] , [86] , [87] , [88] , [89] , [90] , [91] , [92] , [93] , [94] , [95] , [96] , [97] , [98] .…”