Corrigendum: Development and Validation of a Prognostic Nomogram to Predict Cancer-Specific Survival in Adult Patients With Pineoblastoma
(Source: Frontiers in Oncology)
Source: Frontiers in Oncology - October 1, 2020 Category: Cancer & Oncology Source Type: research

Adverse Factors of Treatment Response and Overall Survival in Pediatric and Adult Patients with Pineoblastoma
(Source: Cancer Management and Research)
Source: Cancer Management and Research - August 17, 2020 Category: Cancer & Oncology Tags: Cancer Management and Research Source Type: research

Four Cases of Desquamative Esophagitis Occurring after Hematopoietic Stem Cell Transplantation.
Authors: Iwamuro M, Ennishi D, Matsuoka KI, Tanaka T, Okanoue S, Obayashi Y, Sakae H, Kawahara Y, Okada H Abstract We herein report four patients with desquamative esophagitis that developed one to nine days after peripheral blood stem cell transplantation (PBSCT). Three patients underwent allogeneic PBSCT for leukemia, and the other underwent autologous PBSCT for pineoblastoma. Esophagogastroduodenoscopy revealed mucosal sloughing and fresh blood in the esophagus. Fasting and intravenous proton pump inhibitor therapy in addition to blood transfusion improved the esophageal lesions within five to seven day...
Source: Internal Medicine - August 14, 2020 Category: Internal Medicine Tags: Intern Med Source Type: research

WNT-activated embryonal tumors of the pineal region: ectopic medulloblastomas or a novel pineoblastoma subgroup?
(Source: Acta Neuropathologica)
Source: Acta Neuropathologica - August 7, 2020 Category: Neurology Source Type: research

Development and Validation of a Prognostic Nomogram to Predict Cancer-Specific Survival in Adult Patients With Pineoblastoma
Pineoblastoma (PB) is a rare neoplasm of the central nervous system. This analysis aimed to identify factors and establish a predictive model for the prognosis of adult patients with PB. Data for 213 adult patients with PB (Surveillance, Epidemiology, and End Results database) were randomly divided into primary and validation cohorts. A predictive model was established and optimized based on the Akaike Information Criterion and visualized by a nomogram. Its predictive performance (concordance index and receiver operating characteristic curve) and clinical utility (decision curve analyses) were evaluated. We internally and ...
Source: Frontiers in Oncology - July 23, 2020 Category: Cancer & Oncology Source Type: research

Simultaneous single-trajectory endoscopic biopsy and third ventriculostomy in pediatric pineal region tumors
AbstractPineal region tumors have different pathological tumors and their optimal management remains controversial. Advancements in neuroendoscopy have led to the ability to simultaneously treat the hydrocephalus and obtain a tissue diagnosis. A retrospective review of 34 patients with pineal region tumors in Beijing Tiantan hospital from the year 2016 to 2018 was undertaken. A single bur hole for both procedures was used successfully in all patients. Once pathologic diagnosis is made, the subsequent management of different tumors is dependent on response to therapy, the tumor markers and original pathology. Follow-up peri...
Source: Acta Neurologica Belgica - June 5, 2020 Category: Neurology Source Type: research

Advances in Diagnostic Immunohistochemistry for Primary Tumors of the Central Nervous System
This article reviews novel immunohistochemical markers that may be used to determine mutation status, activation of signaling pathways, druggable targets, and cell lineage in many diverse tumor types. In particular, recently added entities to the 2016 WHO classification of central nervous system tumors will be addressed, including IDH-mutant gliomas, diffuse midline glioma, epithelioid glioblastoma, angiocentric glioma, RELA-rearranged ependymoma, embryonal tumors (medulloblastoma, atypical teratoid/rhabdoid tumor, pineoblastoma, embryonal tumor with multilayered rosettes, and other genetically defined high-grade neuroepit...
Source: Advances in Anatomic Pathology - April 10, 2020 Category: Pathology Tags: Review Articles Source Type: research

Embryonal Tumors of the Central Nervous System
Embryonal tumors of the central nervous system (CNS) are rare, high-grade neoplasms predominantly affecting the pediatric population. Well-defined embryonal tumors include medulloblastoma, atypical teratoid/rhabdoid tumor, embryonal tumor with multilayered rosettes, C19MC-altered and embryonal tumor with multilayered rosettes, not otherwise specified, pineoblastoma, pituitary blastoma, CNS neuroblastoma, and ganglioneuroblastoma. Although their prognosis is nearly uniformly poor, the rapidly evolving understanding of their molecular biology contributes to diagnosis, prognosis, treatment, and clinical trial participation. K...
Source: Surgical Pathology Clinics - April 6, 2020 Category: Pathology Authors: Melissa M. Blessing, Sanda Alexandrescu Source Type: research

Pineoblastoma is uniquely tolerant of mutually exclusive loss of DICER1 , DROSHA or DGCR8
(Source: Acta Neuropathologica)
Source: Acta Neuropathologica - March 1, 2020 Category: Neurology Source Type: research

Pediatric embryonal brain tumors in the molecular era.
Authors: Li BK, Al-Karmi S, Huang A, Bouffet E Abstract INTRODUCTION: Embryonal brain tumors (EBTs) are highly aggressive malignancies predominantly affecting children. They include medulloblastoma (MB), atypical rhabdoid/teratoid tumors (ATRT), pineoblastoma (PB), embryonal tumor multiple rosettes (ETMR)/C19MC-altered tumors, and newly recognized embryonal tumors with FOXR2 activation or BCOR alteration. AREAS COVERED: This review will provide a comprehensive overview and updated of the literature on each of these EBTs. The evolution from location- and histopathology-based diagnosis to more specif...
Source: Expert Review of Molecular Diagnostics - January 11, 2020 Category: Laboratory Medicine Tags: Expert Rev Mol Diagn Source Type: research

Correction to: Risk-adapted therapy and biological heterogeneity in pineoblastoma: integrated clinico-pathological analysis from the prospective, multi-center SJMB03 and SJYC07 trials
The original version of this article unfortunately contained a typesetting error in Fig 3c. The corrected Fig. 3 is given in the following page. (Source: Acta Neuropathologica)
Source: Acta Neuropathologica - December 20, 2019 Category: Neurology Source Type: research

Pineoblastoma segregates into molecular sub-groups with distinct clinico-pathologic features: a Rare Brain Tumor Consortium registry study
AbstractPineoblastomas (PBs) are rare, aggressive pediatric brain tumors of the pineal gland with modest overall survival despite intensive therapy. We sought to define the clinical and molecular spectra of PB to inform new treatment approaches for this orphan cancer. Tumor, blood, and clinical data from 91 patients with PB or supratentorial primitive neuroectodermal tumor (sPNETs/CNS-PNETs), and 2 pineal parenchymal tumors of intermediate differentiation (PPTIDs) were collected from 29 centres in the Rare Brain Tumor Consortium. We used global DNA methylation profiling to define a core group of PB from 72/93 cases, which ...
Source: Acta Neuropathologica - December 8, 2019 Category: Neurology Source Type: research

Risk-adapted therapy and biological heterogeneity in pineoblastoma: integrated clinico-pathological analysis from the prospective, multi-center SJMB03 and SJYC07 trials
AbstractPineoblastoma is a rare embryonal tumor of childhood that is conventionally treated with high-dose craniospinal irradiation (CSI). Multi-dimensional molecular evaluation of pineoblastoma and associated intertumoral heterogeneity is lacking. Herein, we report outcomes and molecular features of children with pineoblastoma from two multi-center, risk-adapted trials (SJMB03 for patients  ≥ 3 years; SJYC07 for patients <  3 years) complemented by a non-protocol institutional cohort. The clinical cohort consisted of 58 patients with histologically diagnosed pineoblastoma (SJMB03 = 30, SJYC07 = 12, ...
Source: Acta Neuropathologica - December 3, 2019 Category: Neurology Source Type: research

Molecular subgrouping of primary pineal parenchymal tumors reveals distinct subtypes correlated with clinical parameters and genetic alterations
AbstractTumors of the pineal region comprise several different entities with distinct clinical and histopathological features. Whereas some entities predominantly affect adults, pineoblastoma (PB) constitutes a highly aggressive malignancy of childhood with a poor outcome. PBs mainly arise sporadically, but may also occur in the context of cancer predisposition syndromes includingDICER1 andRB1 germline mutation. With this study, we investigate clinico-pathological subgroups of pineal tumors and further characterize their biological features. We performed genome-wide DNA methylation analysis in 195 tumors of the pineal regi...
Source: Acta Neuropathologica - November 24, 2019 Category: Neurology Source Type: research

MRI Features of Histologically Diagnosed Supratentorial Primitive Neuroectodermal Tumors and Pineoblastomas in Correlation with Molecular Diagnoses and Outcomes: A Report from the Children's Oncology Group ACNS0332 Trial [PEDIATRICS]
CONCLUSIONS: In nonpineal locations, only a minority of histologically diagnosed primitive neuroectodermal tumors are embryonal tumors; therefore, high-grade glioma or ependymoma should be high on the radiographic differential. An understanding of molecularly defined tumor entities and their relative frequencies and locations will help the radiologist make more accurate predictions of the tumor types. (Source: American Journal of Neuroradiology)
Source: American Journal of Neuroradiology - November 12, 2019 Category: Radiology Authors: Jaju, A., Hwang, E. I., Kool, M., Capper, D., Chavez, L., Brabetz, S., Billups, C., Li, Y., Fouladi, M., Packer, R. J., Pfister, S. M., Olson, J. M., Heier, L. A. Tags: PEDIATRICS Source Type: research