Publications by authors named "A Hirbe"
Synovial sarcoma (SS) is a rare soft tissue sarcoma characterized by high-grade malignancy and poor prognosis. Preliminary research indicates that apoptosis evasion is a key factor in SS progression, primarily attributed to the overexpression of anti-apoptotic genes. However, the mechanisms underlying this phenomenon are still not fully understood.
View Article and Find Full Text PDFBrain Metastases in Sarcomas: A Multicenter Retrospective Cohort Study.
Cancers (Basel)
November 2024
Purpose: Brain metastases (BM) in sarcomas occur rarely and are associated with poor prognosis. This study is a large retrospective cohort describing the demographic and clinical characteristics of these patients, treatment strategies, and survival outcomes.
Methods: In total, 81 patients with BM from sarcomas were identified across five sarcoma centers.
Purpose: Pharmacologic therapies for neurofibromatosis type 1-associated plexiform neurofibromas (NF1-PNs) are limited; currently, none are US Food and Drug Administration-approved for adults.
Methods: ReNeu is an open-label, multicenter, pivotal, phase IIb trial of mirdametinib in 58 adults (≥18 years of age) and 56 children (2 to 17 years of age) with NF1-PN causing significant morbidities. Patients received mirdametinib capsules or tablets for oral suspension (2 mg/m twice daily, maximum 4 mg twice daily), regardless of food intake, in 3 weeks on/1 week off 28-day cycles.
Article Synopsis
- The study focuses on early detection of peripheral nerve sheath tumors (PNST) associated with neurofibromatosis type 1 (NF1) using a cell-free DNA (cfDNA) fragmentomic approach, which can improve clinical decision-making and treatment outcomes.
- Researchers isolated cfDNA from plasma samples of 101 NF1 patients and 21 healthy controls, employing whole-genome sequencing and analyzing various fragmentomic signatures to differentiate between benign, premalignant, and malignant tumors.
- Results showed that fragmentomic methods successfully distinguished atypical neurofibromas (premalignant) from benign forms and malignant PNST, offering potential for non-invasive diagnostics and better management of NF1-related tumors.*
Background: Malignant peripheral sheath tumor (MPNST) is a rare, aggressive form of soft-tissue sarcoma that presents a unique set of diagnostic and treatment challenges and is associated with major unmet treatment medical needs.
Objective: The chief aim of this review is to consider the epidemiology, histology, anatomic distribution, pathologic signaling pathways, diagnosis, and management of MPNST, with a focus on potential targeted therapies. A subordinate objective was to establish benchmarks for the antitumor activity of such treatments.