The Role of CD133+ Cells in a Recurrent Embryonal Tumor with Abundant Neuropil and True Rosettes (ETANTR)

Abstract

Embryonal tumor with abundant neuropil and true rosettes (ETANTR) is a recently described embryonal neoplasm of the central nervous system, consisting of a well-circumscribed embryonal tumor of infancy with mixed features of ependymoblastoma (multilayer ependymoblastic rosettes and pseudorosettes) and neuroblastoma (neuroblastic rosettes) in the presence of neuropil-like islands. We present the case of a young child with a very aggressive tumor that rapidly recurred after gross total resection, chemotherapy, and radiation. Prominent vascular sclerosis and circumscribed tumor led to the diagnosis of malignant astroblastoma; however, rapid recurrence and progression of this large tumor after gross total resection prompted review of the original pathology. ETANTR is histologically distinct with focal GFAP and synaptophysin expression in the presence of neuronal and ependymoblastic rosettes with focal neuropil islands. These architectural features, combined with unique chromosome 19q.13.42 amplification, confirmed the diagnosis. In this report, we describe tumor stem cell (TSC) marker CD133, CD15, and nestin alterations in ETANTR before and after chemotherapy. We found that TSC marker CD133 was richly expressed after chemotherapy in recurrent ETANTR, while CD15 is depleted compared to that expressed in the original tumor, suggesting that CD133+ cells likely survived initial treatment, further contributing to formation of the recurrent tumor.

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] .…”

“…The majority of patients in the literature were treated with photons. Two patients in the literature were treated craniospinal proton therapy [9] , [68] .…”

Battle of the sexes: contrasting roles of testis-specific protein Y-encoded (TSPY) and TSPX in human oncogenesis

“…Further, TSPY is also expressed in nongonadal germ cell tumors, such as the intracranial germ cell tumors, of male patients 65. TSPY expression pattern is correlated with those of various CIS and germ cell markers, such as placental alkaline phosphatase (PLAP), octamer-binding transcription factor 4 (OCT4), proto-oncogene c-Kit (c-Kit),63 and CD133 (prominin 1), a consensus marker for cancer stem cells66676869 capable of self-renewal and tumorigenesis ( Figure 3 ). These findings suggest the potential role of TSPY in the early tumorigenic initiation and progression of gonadoblastoma and TGCTs and somatic cancers of germ cell origin.…”