Central nervous system tumor with BCOR internal tandem duplication (CNS tumor with BCOR-ITD) constitutes a molecularly distinct entity, characterized by internal tandem duplication within exon 15 of the BCOR transcriptional co-repressor gene (BCOR-ITD). The current study aimed to elucidate the clinical, pathological, and molecular attributes of CNS tumors with BCOR-ITD and explore their putative cellular origin. This study cohort comprised four pediatric cases, aged 23 months to 13 years at initial presentation.
View Article and Find Full Text PDFSolitary fibrous tumor (SFT) is an uncommon fibroblastic neoplasm which may arise in a wide range of anatomic location and can occur across all ages. Fat-forming SFT is a rare morphological variant of SFT. Primary breast fat-forming SFT is exquisitely rare.
View Article and Find Full Text PDFMedulloblastoma (MB) is one of the most common childhood malignant brain tumors (WHO grade IV), traditionally divided into WNT, SHH, Group 3, and Group 4 subgroups based on the transcription profiles, somatic DNA alterations, and clinical outcomes. Unlike WNT and SHH subgroup MBs, Group 3 and Group 4 MBs have similar transcriptomes and lack clearly specific drivers and targeted therapeutic options. The recently revised WHO Classification of CNS Tumors has assigned Group 3 and 4 to a provisional non-WNT/SHH entity.
View Article and Find Full Text PDFSolitary fibrous tumor/hemangiopericytomas (SFT/HPCs) are mesenchymal tumors characterized by "staghorn" blood vessels and collagen deposition. Little is known about SFT/HPCs with papillary architecture. We summarized the clinicopathologic features of 12 patients with papillary SFT/HPCs (8 males and 4 females; median age: 59 years), including 8 previously reported cases.
View Article and Find Full Text PDFIntraorbital meningoencephalocele is a rare manifestation of neurofibromatosis type 1 (NF1) caused by secondary changes in sphenoid dysplasia, and it seriously affects patients' vision and facial appearance. We retrospectively analyzed the clinical data of 23 patients with NF1 and intraorbital meningoencephalocele, summarized the surgical strategies employed, and evaluated their clinical efficacy in order to better understand its management in clinical practice, establish a reasonable surgical strategy, and assess prognosis. Before surgery, 22 patients had unilateral pulsatile exophthalmos, 18 patients had significant visual impairment, and 13 patients had ptosis associated with an orbital plexiform neurofibroma (PNF).
View Article and Find Full Text PDFAberrant expression of thyroid transcription factor-1 (TTF-1) has been observed in tumors arising in locations other than thyroid gland, lung and ventral forebrain. However, TTF-1 expression in schwannomas has not yet been studied. Meanwhile, a few inconsistent changes in protein expression have been identified between schwannomas and other peripheral nerve sheath tumors.
View Article and Find Full Text PDFObjective: To evaluate the H. pylori and Epstein-Barr virus infection in cardiac and distal gastric adenocarcinoma tissues in residents in Cixian county, a high risk area of esophageal cancer in Hebei province, and to explore the putative role of H. pylori and Epstein-Barr virus infection in the carcinogenesis of adenocarcinoma at different subsites of stomach.
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