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Disorders of Keratinization and Other Genodermatoses
Published in Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang, Roxburgh's Common Skin Diseases, 2022
Roselyn Stanger, Nanette Silverberg
Course and/or Prognosis: When the seizures are well-managed from an early age, neurologic prognosis is improved long term. Cutaneous lesions, such as neurofibromas, can be surgically removed to reduce cosmetic burden.
Benign tumors
Published in Rashmi Sarkar, Anupam Das, Sumit Sethi, Concise Dermatology, 2021
The neurofibromas can be solitary or multiple. Multiple neurofibromas are present in several distinct genetic disorders. The two main forms are NF1 (Neurofibromatosis 1 or von Recklinghausen’s disease) and NF2 (Neurofibromatosis 2). It is inherited as an autosomal dominant condition, but 30–50% of patients do not give a family history, suggesting that there is a high rate of new gene mutation.
Neoplasia
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
Examples include colorectal adenomas, which may progress to become adenocarcinomas. This relationship is described as the adenoma–carcinoma sequence, the molecular basis of which is dealt with in Chapter 10. Polyps may be solitary (or few) in sporadic cases or more numerous in inherited susceptibility syndromes (FAP). Other benign tumours that may undergo malignant change are neurofibromas (see Chapter 12).
Endovascular embolization of spontaneous massive hemorrhage of a facial plexiform neurofibroma: case report and literature review
Published in Brain Injury, 2022
Ying Jiang, Zheng Xu, Jin-Xiang Huang, Dan-Qing Yu, Cheng-Guang Huang
Neurofibromatosis is a benign, autosomally inherited disorder affecting approximately 1 in every 3000 people worldwide (1,2). Plexiform neurofibroma belongs to one subtype of neurofibromatosis, which is seen in about one-third of patients with neurofibromatosis (1,2) and features a predisposition to tumour formation (3). Although there is currently no known effective therapy, the plexiform neurofibroma grows slowly and does not require frequent and emergent medical treatment. Here, we reported a rare case as a demonstration of the potentially serious nature of plexiform neurofibroma, whose size increased dramatically in a short period due to haemorrhage. Written informed consent was voluntarily provided by the patient and patient’s families. To report this study, the CARE guidelines were followed (4).
Solitary neurofibroma of the orbit with intracerebral extension associated with ocular surface melanocytoma: a case report
Published in Orbit, 2020
Mansooreh Jamshidian-Tehrani, Raziyeh Mahmoudzadeh, Esmaeil Asadi Khameneh, Fahimeh Asadi Amoli, Morteza Faghih Jouibari, Abolfazl Kasaee, Hadi Ghadimi
Neurofibroma is a benign peripheral nerve sheath tumor, composed of variable mixtures of Schwann cells, perineural cells, and fibroblasts.1 Neurofibromas are classified as plexiform, diffuse, localized, or myxoid. Localized neurofibromas are rarely encountered in the orbit (accounting for 0.6% of all orbital tumors)2, but plexiform neurofibromas are more common and account for 2% of orbital masses.3 While plexiform neurofibroma is strongly associated with neurofibromatosis type 1 (NF-1), diffuse and localized orbital neurofibromas have a more variable association with neurofibromatosis.4–6 Solitary or isolated neurofibroma (in the absence of systemic neurofibromatosis) in the orbit is particularly uncommon.7 Herein, we present a case of solitary diffuse orbital neurofibroma with intracerebral extension and associated melanocytoma of ocular surface.
Emerging therapeutic targets for neurofibromatosis type 1
Published in Expert Opinion on Therapeutic Targets, 2018
James A. Walker, Meena Upadhyaya
Neurofibromas usually arise during puberty, with their growth being influenced by hormones and increase in number with age. Understanding the mechanism that underlies the transition of NF1−/+ SCs into tumors may provide potential therapeutic interventions. For example, recently, EGFR-STAT3 signaling has been shown to be involved in the self-renewal of SC precursors (SCPs), which result in PNF initiation [127], raising the possibility that it might be possible to devise a pre-emptive strategy to inhibit PNF formation. The recent development of three-dimensional (3D) cell culture models of PNFs may facilitate pre-clinical identification of potential targeted therapeutics for these tumors [128].