Results 51 to 60 of about 47,993 (230)

Malignant peripheral nerve sheath tumor of the cervical vagus nerve in a neurofibromatosis type 1 patient - An unusual presentation [PDF]

, 2010
Malignant peripheral nerve sheath tumors (MPNST’S) of the head and neck comprise 2% to 6% of head and neck sarcomas. These tumors may arise as sporadic variants or in patients with neurofibromatosis (NF).
Bahl, A, Bansal, S, Bhagat, S, Gupta, A
core   +1 more source

Rapid Response to Trametinib Combined With Chemotherapy for Infant BRAF‐Fused Chiasmatic Glioma

Pediatric Blood &Cancer, EarlyView.
ABSTRACT Infants, less than 1 year, with chiasmatic gliomas (ICG) present a major therapeutic challenge due to large tumor size, decreased vision, rapid progression, and poor response to vincristine/carboplatin chemotherapy. The majority have a BRAF fusion, which may respond to downstream MEK inhibitors but response time is slow. There are no safety or
Helen Toledano, Alexander Yelak, Walid Kaadan, Shlomo Caduri, Rivka Friedland, Orli Michaeli, Gad Dotan, Osnat Konen   +7 more
wiley   +1 more source

A case of neurofibromatosis type 1 with neurofibromatosis type 1-related and neurofibromatosis type 1-unrelated tumors: a case report

Journal of Medical Case Reports
Background Neurofibromatosis type 1 is an autosomal dominantly inherited disorder caused by pathogenic variants in the neurofibromatosis type 1 gene, resulting in a predisposition to multiple tumors.
Tabea I. Hartung, Qais Karimi, Lan Kluwe, Said C. Farschtschi   +3 more
doaj   +1 more source

Estrogen activation of microglia underlies the sexually dimorphic differences in Nf1 optic glioma-induced retinal pathology [PDF]

, 2016
Children with neurofibromatosis type 1 (NF1) develop low-grade brain tumors throughout the optic pathway. Nearly 50% of children with optic pathway gliomas (OPGs) experience visual impairment, and few regain their vision after chemotherapy.
Anne C. Solga, Arnold, Avery, Azevedo, Bajenaru, Bondesson, Compton, Daginakatte, Daginakatte, David H. Gutmann, Dennis, Diggs-Andrews, Diggs-Andrews, Fisher, Fisher, Green, Gu, Guadagno, Hegedus, Hyman, Joseph A. Toonen, Kalin-Hajdu, Kappeler, Kuiper, Listernick, Listernick, Neher, Parrilla-Reverter, Revankar, Saijo, Schneider, Simmons, Solga, Tapia-Gonzalez, Tikka, Wu, Yu Ma, Zhao   +37 more
core   +2 more sources

Organoids in pediatric cancer research

FEBS Letters, EarlyView.
Organoid technology has revolutionized cancer research, yet its application in pediatric oncology remains limited. Recent advances have enabled the development of pediatric tumor organoids, offering new insights into disease biology, treatment response, and interactions with the tumor microenvironment.
Carla Ríos Arceo, Jarno Drost
wiley   +1 more source

Multiple CNS Tumors in a Patient With Neurofibromatosis Type 2: Classical Presentation of a Rare Diseas

Acta Medica Iranica, 2020
Neurofibromatosis type 2 is a genetic autosomal dominant disorder, caused by spontaneous mutation in the gene located on chromosome 22 q11-13.1,which usually emerges in adolescence or early adulthood and is characterized by development of bilateral ...
Aamer Ubaid, Farishta Waheed, Awais Naeem   +2 more
doaj   +1 more source

Whole tumor RNA-sequencing and deconvolution reveal a clinically-prognostic PTEN/PI3K-regulated glioma transcriptional signature [PDF]

, 2017
The concept that solid tumors are maintained by a productive interplay between neoplastic and non-neoplastic elements has gained traction with the demonstration that stromal fibroblasts and immune system cells dictate cancer development and progression ...
Bush, Erin C, Gutmann, David H, Ma, Yu, Pan, Yuan, Sims, Peter A, Solga, Anne C, Toonen, Joseph A   +6 more
core   +2 more sources

Pathogenic Neurofibromatosis type 1 gene variants in tumors of non‐NF1 patients and role of R1276

FEBS Open Bio, EarlyView.
Somatic variants of the neurofibromatosis type 1 (NF1) gene occur across neoplasms without clinical manifestation of the disease NF1. We identified emerging somatic pathogenic NF1 variants and hotspots, for example, at the arginine finger 1276. Those missense variants provide fundamental information about neurofibromin's role in cancer.
Mareike Selig, Swanhild Lohse, Sara Elahi, Nils Hartmann, Stefanie Deckert, Shutian Si, Alexander Desuki, Anja Harder   +7 more
wiley   +1 more source

Neurofibromatosis type 2 (NF2): A clinical and molecular review

Orphanet Journal of Rare Diseases, 2009
Neurofibromatosis type 2 (NF2) is a tumour-prone disorder characterised by the development of multiple schwannomas and meningiomas. Prevalence (initially estimated at 1: 200,000) is around 1 in 60,000. Affected individuals inevitably develop schwannomas,
Evans D Gareth R
doaj   +1 more source

Lethal Hemomediastinum due to Spontaneous Rupture of an Aberrant Bronchial Artery in a Patient with Neurofibromatosis Type 1: Successful Treatment with Embolization

대한영상의학회지, 2020
Spontaneous bleeding due to vascular involvement of neurofibromatosis type 1 is rare but potentially fatal. Herein, we report a case of a lethal spontaneous hemomediastinum in a patient with neurofibromatosis type 1.
Yun Jin Im, Min Jeong Choi, Bong Man Kim
doaj   +1 more source