scholarly journals Central Nervous System Embryonal Tumor, Not Otherwise Specified with Leptomeningeal Spread

2020 ◽  
Author(s):  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Embryonal Tumor ◽  
Leptomeningeal Spread ◽  
Not Otherwise Specified

Hemorrhagic Suprasellar Central Nervous System Embryonal Tumor in an Adult: Uncommon Features of an Extremely Rare Neoplasm

2021 ◽  
Author(s):  
Martina Piloni ◽  
Filippo Gagliardi ◽  
Michele Bailo ◽  
Lina Raffaella Barzaghi ◽  
Marcella Callea ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Who Classification ◽  
Optic Chiasm ◽  
World Health ◽  
Embryonal Tumors ◽  
Embryonal Tumor ◽  
First Case ◽  
Diffusion Pattern

Abstract Background Occurrences of suprasellar central nervous system (CNS) embryonal tumors in adults are extremely rare. Hemorrhagic onset is further uncommon, with only anecdotic cases reported in the literature. The authors describe the case of a 57-year-old man affected by a suprasellar CNS embryonal tumor, with hemorrhagic onset and a unique diffusion pattern along the optic pathways. Material and Methods A 57-year-old man presenting with acute visual acuity worsening and left homonymous hemianopia was referred to our hospital. Neuroradiologic studies demonstrated an infiltrating, high-grade lesion involving the optic chiasm and right retrochiasmatic pathways with a hemorrhagic area in the ipsilateral pulvinar. Results The patient underwent microsurgical biopsy. Pathologic assessment confirmed the diagnosis of CNS embryonal tumor, not otherwise specified (NOS) according to the 2016 World Health Organization (WHO) classification of CNS tumors. The patient was referred to a multimodal adjuvant treatment; he eventually died 4 months after surgery. Competent literature has been systematically reviewed in the light of the relevant changes made in the last version of the WHO classification. Conclusion Embryonal tumors should be considered in the differential diagnosis for sellar and suprasellar space-occupying lesions, despite the rarity of the disease and the uncommon features at time of presentation. As per our knowledge, this is the first case ever described of hemorrhagic suprasellar embryonal tumor with a diffusion pattern along white matter fibers. Histogenesis, biomolecular and neuroradiologic features, and classification of embryonal tumors are an open field of research, with considerable implications for the definition of better diagnostic pitfalls and therapeutic regimens.

scholarly journals Atypical teratoid/rhabdoid tumor: A rare case report

2017 ◽  
Vol 5 (3) ◽  
pp. 70-72
Author(s):  
R Shrestha ◽  
S Gauchan ◽  
A K Jha
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Rare Case ◽  
Primitive Neuroectodermal Tumor ◽  
Atypical Teratoid Rhabdoid Tumor ◽  
Embryonal Tumor ◽  
Rare Case Report ◽  
Atypical Teratoid ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors

Atypical Teratoid/Rhabdoid Tumors (AT/RTs) is rare, highly malignant embryonal tumor of central nervous system that predominantly occurs in infancy and young children. The majority of tumors arise (approximately two-third) in the posterior fossa. The diagnosis of AT/RTs relies predominantly on morphologic and immunohistochemical criteria. The most common differential diagnosis are Primitive Neuroectodermal Tumor and malignant glioma. Herein we present a case of 5 year old child who initially presented with headache and vomiting.

Pontine Embryonal Tumor With Multilayered Rosettes: An Autopsy Case Exhibiting Extensive Posttreatment Glial and Neuronal Maturation

2020 ◽  
Vol 23 (4) ◽  
pp. 326-331
Author(s):  
Adrian Levine ◽  
Juliette Hukin ◽  
Christopher Dunham
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Diffuse Intrinsic Pontine Glioma ◽  
Neurological Deficits ◽  
Neuronal Maturation ◽  
Mirna Cluster ◽  
Central Nervous System Tumor ◽  
Embryonal Tumor ◽  
Tumor Biopsy ◽  
History Of

Embryonal tumor with multilayered rosettes (ETMR) is a rare and highly aggressive embryonal central nervous system tumor that primarily affects young children. It is characterized by (1) amplification of the C19MC miRNA cluster at 19q13.42 and (2) immunohistochemical tumor cell positivity for LIN28A. We describe the case of a 3-year-old girl who presented with a 2-week history of multiple neurological deficits. Based primarily on imaging findings that revealed a large pontine tumor, biopsy was not performed and the patient was clinically diagnosed with a “diffuse intrinsic pontine glioma.” She was subsequently treated with radiation and concurrent adjuvant temozolomide, but unfortunately there was minimal response and the patient died 6 months after diagnosis. Autopsy revealed an ETMR that was confirmed via C19MC fluorescence in situ hybridization and LIN28 immunohistochemistry. Although widespread central nervous system dissemination was observed, large portions of the main pontine mass exhibited evidence of extensive glial and neuronal maturation (ie, differentiation). We consider this tissue “maturation” to have been induced by chemotherapy and radiation. Herein, we discuss the importance of antemortem biopsy of intrinsic pontine tumors and the clinical significance of glial and neuronal maturation post therapy in the context of ETMR.

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