Evaluation of the good tumor response of embryonal tumor with abundant neuropil and true rosettes (ETANTR)

Abstract

The embryonal tumor with abundant neuropil and true rosettes is a rare and highly malignant variant of embryonal brain tumors. It usually affects infants and young children under the age of 4 years and exhibits a very aggressive course with a dismal prognosis. For the 68 cases reported to date the mean age at diagnosis was 25.42 months (range 3-57 months). Survival data are available for 48 children (including our case): the median overall survival is 13.0 months, though 6 (9%) of the children have had a relative long survival (>30 months). The aggressive combined treatment, involving primary surgical tumor removal, adjuvant polychemotherapy, including high-dose chemotherapy with stem cell transplantation, radiotherapy and radiochemotherapy, might play an important role in the longer survival. We have performed a literature review and we present here a multimodal-treated case of a 2- year-old girl with a long survival, who was reoperated when recurrence occurred. The residual tumor demonstrated a good response to temozolomide radiochemotherapy (craniospinal axis + boost) and followed by maintenance temozolomide. The described complex aggressive treatment option might be considered for future cases of this tumor entity.

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] .…”

Preclinical drug screen reveals topotecan, actinomycin D, and volasertib as potential new therapeutic candidates for ETMR brain tumor patients

“…While conventional high-dose chemotherapy and radiotherapy may give a good initial tumor response, the very aggressive nature of ETMRs is still poorly controlled by the current therapies, leading to a high risk of early relapse with very poor overall survival. [9][10][11] Good preclinical ETMR models are needed to evaluate standard and novel treatment options. The only available preclinical model so far is the human ETMR cell line BT183.…”

Embryonal Tumour With Multi-Layered Rosette- A Rare Case Report

“…Instead it has very characteristics ependymoblastic rosettes in both highly cellular as well as acellular area. Recently amplification of C19MC region on chromosome 19(19 q 13.42) (3) has been identified as a characteristic of these tumours (4,5) . As a result, both ETANTR & CNS PNET has been removed from 2016 updates of WHO classification of CNS tumour and has been replaced by the term ETMR, C 19 MC-altered or ETMR -NOS if C19MC amplification is absent.…”