Clinical characteristics, late effects and outcomes in pineoblastomas in children: a single center experience
CONCLUSIONS: Pineoblastomas are rare but very aggressive tumors; more effective treatment strategies are needed. Survivors should be followed up for late effects such as second malignancies and endocrine deficiencies.PMID:35023643 | DOI:10.24953/turkjped.2021.06.002 (Source: The Turkish Journal of Pediatrics)
Source: The Turkish Journal of Pediatrics - January 13, 2022 Category: Pediatrics Authors: Ömer Görgün Ba şak Koç Rejin Kebudi Johannes E Wolff Abut Kebudi Emin Darendeliler Source Type: research
Non-invasive preoperative imaging differential diagnosis of pineal region tumor: A novel developed and validated multiparametric MRI-based clinicoradiomic model
Pineal region tumors are relatively rare accounting for approximately 5% of all intracranial tumors in children and represent less than 1% of brain tumors in adults [1 –3]. Pineal region tumors are histologically divided into germ cell tumors (GCTs) (including germinomas, nongerminomatous GCTs and teratomas), pineal parenchymal tumor (including pineocytomas and pineoblastomas), and miscellaneous tumors arise in this region. Pathologic diagnosis is necessary to d etermine the optimal treatment strategy because different types of tumors have reported varying responses to radiotherapy, chemotherapy, surgical resection, or c...
Source: Radiotherapy and Oncology - January 13, 2022 Category: Radiology Authors: Yanghua Fan, Xulei Huo, Xiaojie Li, Liang Wang, Zhen Wu Tags: Original Article Source Type: research
Role of proliferative marker index and KBTBD4 mutation in the pathological diagnosis of pineal parenchymal tumors
This study included 19 cases of PPTs [3 pineocytomas (PCs), 10 PPTs of intermediate differentiation (PPTID), and 6 pineoblastomas (PBs)]. Immunohistochemistry for Ki-67, PHH3, and DICER1, as well as Sanger sequencing analysis forKBTBD4 mutations, was performed using formalin-fixed paraffin-embedded tissue specimens that were resected during surgery. Tumor cell proliferation was quantified using an image analysis software. For the PHH3 and MIB-1 indices, a significant difference was observed between the PPTIDs and PBs (P < 0.05). Loss of DICER1 was not specific for PB; 0/3 PCs (0.0%), 2/9 PPTIDs (22.2%), and 2/4 PBs...
Source: Brain Tumor Pathology - January 9, 2022 Category: Neurology Source Type: research
Report of clinical presentations and two novel mutations in patients with Wiskott-Aldrich syndrome/X-linked Thrombocytopenia
We reported 7 male patients, 2 with WAS and 5 with XLT, from 6 different families. Two novel mutations, p.Gly387GlufsTer58 and p.Ala134Asp, were identified in patients with WAS. Both patients had severe clinical phenotypes compatible with classic WAS and developed lethal outcomes with intracranial hemorrhage. Other than that, one patient with XLT developed pineoblastoma.PMID:34705590 | DOI:10.1080/09537104.2021.1988549 (Source: Platelets)
Source: Platelets - October 27, 2021 Category: Hematology Authors: Natsumon Udomkittivorakul Duangrurdee Wattanasirichaigoon Wiparat Manuyakorn Pongpak Pongphitcha Arthaporn Khongkraparn Padcha Tunlayadechanont Nongnuch Sirachainan Source Type: research
Prognostic factors and outcome of pineoblastoma: 10 years single-center experience
CONCLUSIONS: A multimodality approach is needed to treat this aggressive disease. Inadequate dose intensity affected our patients' outcome negatively. A more aggressive approach using high-dose chemotherapy or CSI may be required to improve infantile pineoblastoma's dismal outcome. Focal radiotherapy is not an efficacious treatment in infants due to its high-metastatic potential. Molecular typing should be considered to label patients who need a more intensified approach.PMID:34541625 | DOI:10.1186/s43046-021-00083-3 (Source: J Egypt Natl Canc In...)
Source: J Egypt Natl Canc In... - September 20, 2021 Category: Cancer & Oncology Authors: Ahmed Elhemaly Mohamed S Zaghloul Soha Ahmed Hala Taha Amal Refaat Eslam Maher Mohamed El-Beltagy Wael Zekry Source Type: research
Prognostic factors and outcome of pineoblastoma: 10 years single-center experience
CONCLUSIONS: A multimodality approach is needed to treat this aggressive disease. Inadequate dose intensity affected our patients' outcome negatively. A more aggressive approach using high-dose chemotherapy or CSI may be required to improve infantile pineoblastoma's dismal outcome. Focal radiotherapy is not an efficacious treatment in infants due to its high-metastatic potential. Molecular typing should be considered to label patients who need a more intensified approach.PMID:34541625 | DOI:10.1186/s43046-021-00083-3 (Source: J Egypt Natl Canc In...)
Source: J Egypt Natl Canc In... - September 20, 2021 Category: Cancer & Oncology Authors: Ahmed Elhemaly Mohamed S Zaghloul Soha Ahmed Hala Taha Amal Refaat Eslam Maher Mohamed El-Beltagy Wael Zekry Source Type: research
