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The Journal of Dermatology Feb 2020Neurofibromatosis 1 (NF1) is a genetic disease characterized by cutaneous, neurological and osseous abnormalities. Approximately 20% of patients develop plexiform...
Neurofibromatosis 1 (NF1) is a genetic disease characterized by cutaneous, neurological and osseous abnormalities. Approximately 20% of patients develop plexiform neurofibroma (PN), resulting in impaired quality of life. To evaluate distribution of diffuse PN on the body surface, a retrospective study was conducted for 354 patients with NF1 from 2007 to 2018 in Japan. We investigated a total of 40 patients with clinically apparent superficial diffuse PN. In the cases evaluated, 57.4% of the diffuse PN were located on the trunk, 19.2% the head and neck, 12.8% the lower limbs and 10.6% the upper limbs. Remarkably, 75.0% of the diffuse PN were located on the dorsal side. The frequency was significantly higher on the trunk than on the head and neck (P = 0.026). Our findings provide useful information for giving attention to the high possibility of diffuse PN on the dorsal side before progression in childhood and for future treatment in NF1.
Topics: Adolescent; Adult; Aged; Child; Child, Preschool; Disease Progression; Female; Head; Humans; Infant; Infant, Newborn; Lower Extremity; Male; Middle Aged; Neck; Neurofibroma, Plexiform; Neurofibromatosis 1; Quality of Life; Retrospective Studies; Skin; Torso; Upper Extremity; Young Adult
PubMed: 31849109
DOI: 10.1111/1346-8138.15194 -
Expansion Technique for Reconstruction of Craniofacial Defect after Plexiform Neurofibroma Excision.The Journal of Craniofacial SurgeryReconstruction of large craniofacial defect after plexiform neurofibroma excision poses a continuous challenge for plastic surgeons, on account of characteristics of...
Reconstruction of large craniofacial defect after plexiform neurofibroma excision poses a continuous challenge for plastic surgeons, on account of characteristics of plexiform neurofibroma and patients' aesthetic requirements. Skin graft or free flap is hard to obtain satisfactory results or may pose technical challenges. In an attempt to provide the coverage with 'like tissue', we chose local tissue expansion technique. The expansion period was about an average of 3.4 months. We performed 19 expanded flaps located in the head, face, neck, forearm and superclavical regions to reconstruct the craniofacial defect and achieved satisfactory results. Preoperative endovascular embolism in some cases and several intraoperative hemostatic methods for all cases were undertaken to control the perioperative bleeding. For patients who request aesthetic results and are allowed two-staged operations, our method is viable.
Topics: Humans; Plastic Surgery Procedures; Neurofibroma, Plexiform; Esthetics, Dental; Skin Transplantation; Free Tissue Flaps
PubMed: 37291729
DOI: 10.1097/SCS.0000000000009364 -
Urology Journal 2007
Topics: Child, Preschool; Humans; Male; Neurofibroma, Plexiform; Penile Neoplasms
PubMed: 17514614
DOI: No ID Found -
Indian Pediatrics Jun 2004
Topics: Child; Humans; Male; Neurofibroma, Plexiform; Neurofibromatosis 1; Skin Neoplasms
PubMed: 15235175
DOI: No ID Found -
Current Problems in Cancer 2018Plexiform neurofibroma (PNF) is a rare variant of neurofibromatosis type1 (NF-1), which histopathologically, is a subtype of benign nerve sheath tumors, neurofibromas...
Plexiform neurofibroma (PNF) is a rare variant of neurofibromatosis type1 (NF-1), which histopathologically, is a subtype of benign nerve sheath tumors, neurofibromas (NF). It develops as a result of proliferation in all parts of peripheral nervous system and can cause the functional damage, deformities, pain, considerable mortality, and morbidity and even the increasing risk of malignant transformation in some critical cases. Currently, the surgical intervention is the treatment of choice for PNF patients, which due to the tumor invasion, massive growth, and the chance of postoperative regrowth is not possible. The diagnosis of isolated tumor is an uncommon event. Considering the rarity of this neoplasm, herein, we describe a case of isolated PNF, so the purpose of this presenting is the rarity of recording. We describe a case of isolated plexiform neurofibroma presented with 7-year history of a slowly growing postauricular soft subcutaneous mass in a 14-year-old boy, which caused the right auricular deformity. After initial evaluation by imaging studies, the patient underwent to surgical resection of the mass and the diagnosis of plexiform neurofibroma was confirmed by histopathologic examination. Surgical excision of the mass had been done before which concluded the satisfactory result and based on oncologist diagnosis, further intervention such as radiotherapy or chemotherapy was not needed. The patient left the hospital with a clinical stability and was suggested to continue the regular follow-up. In conclusion, considering neurofibroma (NF) as differential diagnosis for subcutaneous masses in head and neck area is critical for early diagnosis and treatment procedure.
Topics: Adolescent; Diagnosis, Differential; Humans; Male; Neurofibroma, Plexiform; Skull Neoplasms
PubMed: 29449010
DOI: 10.1016/j.currproblcancer.2018.01.007 -
The American Journal of Emergency... Jun 2021
Topics: Adult; Antifibrinolytic Agents; Blood Transfusion; Combined Modality Therapy; Embolization, Therapeutic; Hemorrhage; Hemostatics; Humans; Male; Neurofibroma, Plexiform; Radiography, Interventional; Resuscitation; Tomography, X-Ray Computed; Tranexamic Acid
PubMed: 33221111
DOI: 10.1016/j.ajem.2020.10.080 -
Clinical Medicine & Research Mar 2015We describe a premature neonate with an extensive plexiform neurofibroma. Prenatal ultrasound at 32 weeks of gestation was normal. Postnatal examination was significant...
We describe a premature neonate with an extensive plexiform neurofibroma. Prenatal ultrasound at 32 weeks of gestation was normal. Postnatal examination was significant for a palpable left neck mass. Magnetic resonance imaging (MRI) of the head demonstrated a mass involving the left cavernous sinus with spreading to the left orbital region. MRI of the neck was positive for extensive adenopathy, left more than right, with extension into the deep face region and infratemporal fossa on the left side. MRI of the chest, abdomen, and pelvis demonstrated a mass extending from the superior mediastinum to the left pelvic retroperitoneal region, including the mesenteric vasculature and spinal canal at multiple levels with compression of the spinal cord. Biopsy of the left neck mass confirmed for plexiform neurofibroma. A careful search of the literature revealed no previous report of such an extensive neurofibroma in a premature neonate. Surgical decompression in this premature neonate was not possible because of the extensive nature of the disease; it is known that neurofibroma is non-respondent to chemotherapy and radiotherapy. Therefore, alternative treatment is needed to improve the outcome.
Topics: Humans; Infant, Newborn; Infant, Premature; Magnetic Resonance Imaging; Male; Neurofibroma, Plexiform
PubMed: 25380612
DOI: 10.3121/cmr.2014.1224 -
Giornale Italiano Di Dermatologia E... Oct 2018
Topics: Adult; Female; Humans; Male; Middle Aged; Neurofibroma, Plexiform; Neurofibromatosis 1
PubMed: 30246960
DOI: 10.23736/S0392-0488.17.05678-4 -
Current Oncology Reports Dec 2023Neurofibromatosis type I (NF1), neurofibromatosis type 2 (NF2), and schwannomatosis represent a diverse group of genetic tumor predisposition syndromes with a shared... (Review)
Review
Neurofibromatosis type I (NF1), neurofibromatosis type 2 (NF2), and schwannomatosis represent a diverse group of genetic tumor predisposition syndromes with a shared feature of tumors affecting the peripheral nerve sheaths. PURPOSE OF REVIEW: Many advancements have been made in understanding the biologic underpinnings of these conditions, and in 2016 the first drug was approved by the FDA to treat pediatric symptomatic unresectable plexiform neurofibromas. RECENT FINDINGS: Mek inhibitors have provided a much-needed therapeutic avenue for NF1 patients with unresectable plexiform neurofibromas (PN), both for reduction of tumor bulk and for improvement in symptoms. Selumetinib is the first FDA approved drug for PN, but is only approved for children. Some research suggests that alternative Mek inhibitors and other mixed tyrosine kinase inhibitors may have better efficacy in adults. Vascular endothelial growth factor (VEGF) inhibitor bevacizumab can prolong hearing and delay the need for surgery in NF2 patients with bilateral vestibular schwannomas. This article provides an update regarding considerations and approaches when treating the tumors associated with the neurofibromatoses (NF), including risk and prognosis metrics, clinical trial results, surgical techniques, and radiation therapy recommendations.
Topics: Adult; Humans; Child; Neurofibroma, Plexiform; Vascular Endothelial Growth Factor A; Neurofibromatoses; Neurofibromatosis 1; Peripheral Nervous System Neoplasms; Genetic Predisposition to Disease; Protein Kinase Inhibitors; Mitogen-Activated Protein Kinase Kinases
PubMed: 37906356
DOI: 10.1007/s11912-023-01451-z -
The Pan African Medical Journal 2018Plexiform neurofibroma is a rare benign tumor of the peripheral nerves involving the conjunctiva cells of the perineurium. It is pathognomonic of neurofibromatosis type1...
Plexiform neurofibroma is a rare benign tumor of the peripheral nerves involving the conjunctiva cells of the perineurium. It is pathognomonic of neurofibromatosis type1 (NF1 or Von Recklinghausen disease). MRI is a great help in the diagnosis of this pathology. Anatomopathological confirmation is sometimes necessary, in particular in patients with no signs of NF1. We here report the case of a little girl with cervical plexiform neurofibroma revealing neurofibromatosis type 1.
Topics: Child; Female; Head and Neck Neoplasms; Humans; Magnetic Resonance Imaging; Neurofibroma, Plexiform; Neurofibromatosis 1
PubMed: 30167068
DOI: 10.11604/pamj.2018.30.41.14446