Results 41 to 50 of about 3,865 (167)
ABSTRACT Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive soft tissue sarcomas and the most common cause of disease‐associated death for neurofibromatosis type 1 (NF1) patients. In the context of NF1, MPNSTs develop from benign premalignant precursors and the transition to malignancy is typically accompanied by loss of the polycomb ...
Madilyn R. Stahl +4 more
wiley +1 more source
A toddler with neurofibromatosis type 1 (NF1) was evaluated for tuberculosis (TB) after exposure. Chest X-ray (CXR) revealed a mediastinal mass indicating lymphadenopathy. However, magnetic resonance imaging showed a large plexiform thoracic neurofibroma.
Renate Krüger +4 more
doaj +1 more source
Selumetinib as a Target Therapy in Progressive Paediatric Low‐Grade Gliomas—Case Series (pLGG)
ABSTRACT Background Optic pathway gliomas (OPGs) occur in 15%–20% of children with neurofibromatosis type 1 (NF1). While smaller gliomas may be only monitored, the current standard of care for symptomatic ones relies on chemotherapy, most commonly carboplatin and vincristine.
Laura Trapani +12 more
wiley +1 more source
Bilateral Giant Plexiform Neurofibromas In Neurofibromatosis Type 1
A single plexiform neurofibroma or two of any type are considered diagnostic of neurofibromatosis type 1 (NF). Plexiform neurofibromas are congenital and pathognomonic for NF-1.
Thappa D . M +2 more
doaj
Ancient Schwannoma in the Nasal Cavity—A Rare Case Report With Brief Literature Review
Eye &ENT Research, EarlyView.
Shikhar Chohan +2 more
wiley +1 more source
ABSTRACT Objective To evaluate selumetinib exposure using therapeutic drug monitoring (TDM) in pediatric patients with neurofibromatosis type 1 (NF1) and plexiform neurofibromas (PN), assess interpatient pharmacokinetic variability, and explore the relationship between drug exposure, clinical response, and adverse effects.
Janka Kovács +8 more
wiley +1 more source
BRAF inhibitors and MEK inhibitors (MEKi) have reshaped the treatment of BRAFV600‐mutant malignancies; however, cutaneous adverse drug reactions (ADRs) remain a frequent and clinically impactful toxicity. Although clinical trials provide insight into their safety profiles, real‐world data on dermatologic ADRs are limited.
Natalia Sauer +3 more
wiley +1 more source
Facial plexiform neurofibroma in a child with neurofibromatosis type I: A case report
Plexiform neurofibroma is a non-circumscribed, thick, and irregular benign tumor of the peripheral nerve sheath. It is a virtually pathognomonic and often disabling feature of neurofibromatosis type I.
Patil K, Mahima V, Shetty S, Lahari K
doaj
Background A large plexiform neurofibroma in patients with neurofibromatosis type I can be life threatening due to possible massive bleeding within the lesion.
Taro Mikami +11 more
doaj +1 more source
ABSTRACT Introduction Neurofibromatosis type 1 (NF1) is a genetic disorder that increases the risk of various tumors. However, its association with testicular germ cell tumors (GCTs) is rare. We report a case of metastatic GCT in an NF1 patient treated with precision medicine.
Keita Goto +9 more
wiley +1 more source

