Embryonal tumors with multilayered rosettes in children: the SFCE experience

Abstract

Prognosis of ETMR remains dismal despite multimodal therapy. LIN28A immunostaining and 19q13.42 amplification should be systematically done to secure the diagnosis. Complete surgical resection, radiotherapy, and high-dose chemotherapy are associated with better outcome.

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] .…”

“…Only 18 patients have been reported who are long-term survivors (≥36 months) with a median age at diagnosis of 35.4 months (range 7–139.2 months), and a median OS of 77.25 months (range 37.2–183.9 months) [7] , [50] , [55] , [66] , [67] , [69] , [78] , [80] . Sixteen of these eighteen patients received trimodality therapy with surgery, chemotherapy and radiotherapy; one patient received surgery and chemotherapy [55] , and one received surgery and radiation [69] . Therefore, 94% of long-term survivors (17 of 18) in the literature received radiotherapy as part of their primary treatment [55] .…”

“…Similarly, Ding [49] found that the addition of radiotherapy, chemotherapy or combined chemo-radiotherapy had a significant survival benefit compared to patients who did not receive radiotherapy, chemotherapy or combined post-operative chemo-radiotherapy. Horwitz reported a statistically significant OS benefit on multivariate analysis of patients who received complete surgical resection, radiotherapy and high dose chemotherapy [69] . In a series of 11 EPL patients treated on the prospective HIT-trials [66] , the only two long-term survivors (follow up of 12.7 and 9.4 years, respectively) received CSI therapy with involved field boost as part of their first line-therapy; a third patient who received radiation had stable disease at 2.3 years.…”

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

“…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.…”

Rare Brain Tumor in a Neonate

“…They occur mostly in children aged less than 2 years old and their prognosis is poor. In the literature, the localization of the majority of ETMR is supratentorial with signs of increased intracranial pressure (66%) [ 7 ]. Our case showed a infratentorial tumor with hydrocephalus.…”