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The Journal of Foot and Ankle Surgery :... 2002Plexiform neurofibroma typically occurs in the setting of neurofibromatosis type 1, and is included in the diagnostic criteria for neurofibromatosis. Plexiform... (Review)
Review
Plexiform neurofibroma typically occurs in the setting of neurofibromatosis type 1, and is included in the diagnostic criteria for neurofibromatosis. Plexiform neurofibromas generally affect larger peripheral nerves and are uncommon in the foot and ankle. While there are several reports of large neurofibromas involving the foot, they have been described on the plantar aspect. We report the first known case of plexiform neurofibroma involving the deep peroneal nerve of the foot. A review of the literature is also presented.
Topics: Adult; Female; Foot; Foot Diseases; Humans; Magnetic Resonance Imaging; Neurofibroma, Plexiform; Peripheral Nervous System Neoplasms; Peroneal Neuropathies; Radiography
PubMed: 11995832
DOI: 10.1016/s1067-2516(02)80036-9 -
The Journal of Laryngology and Otology Aug 2000A rare plexiform neurofibroma of the submandibular gland in a patient with neurofibromatosis is presented. The clinical manifestations of the disease are reviewed. The... (Review)
Review
A rare plexiform neurofibroma of the submandibular gland in a patient with neurofibromatosis is presented. The clinical manifestations of the disease are reviewed. The need for early diagnosis of neurofibroma is emphasized because of malignant transformation.
Topics: Adult; Humans; Male; Neurofibroma, Plexiform; Neurofibromatoses; Submandibular Gland Diseases
PubMed: 11027061
DOI: 10.1258/0022215001906390 -
Medicine Feb 2016Plexiform neurofibromas represent an uncommon variant (30%) of neurofibromatosis type 1 (NF-1) in which neurofibromas arise from multiple nerves as bulging and deforming...
Plexiform neurofibromas represent an uncommon variant (30%) of neurofibromatosis type 1 (NF-1) in which neurofibromas arise from multiple nerves as bulging and deforming masses involving also connective tissue and skin folds.We report a rare case of a 30-year-old man who presented with a progressive facial deformity that began in early childhood. Skin examination also revealed multiple neurofibromas and café-au-lait macules on the trunk and arms. Histopathological examination on biopsy samples showed overgrowth of peripheral nerve components and connective tissue. Two diagnostic criteria for NF-1 (plexiform variant) were met, the patient did not accept to undergo genetic testing. Craniofacial MRI confirmed the presence of a deforming mass arising from the left side of his face giving homolateral eye dislocation.Surgery is the mainstay of the treatment. However, the patient expressed the preference to avoid surgery and chose to undergo clinical follow-up every 6 months.Diagnosis of plexiform neurofibromas is usually made clinically, especially if classical hallmarks of NF-1 are present. Therapy is surgical, aiming at resecting deforming masses and cancerous tissue when malignant transformation occurs.
Topics: Adult; Facial Neoplasms; Humans; Male; Neurofibroma, Plexiform
PubMed: 26871793
DOI: 10.1097/MD.0000000000002663 -
Skeletal Radiology Feb 2020Plexiform neurofibromas are an uncommon variant of neurofibromas that are described as being essentially pathognomonic of neurofibromatosis type 1 (NF1). Plexiform...
Plexiform neurofibromas are an uncommon variant of neurofibromas that are described as being essentially pathognomonic of neurofibromatosis type 1 (NF1). Plexiform neurofibromas in the absence of NF1 are extremely rare. We present the case of a 38-year-old woman with a large multilobulated lumbosacral mass extending into the pelvis and proximal thigh. Histopathology of a CT-guided biopsy of the mass revealed it to be a neurofibroma. The imaging findings were consistent with a plexiform subtype. Further imaging and clinical workup showed that the patient had no other identifiable neurofibromas and did not meet criteria for the diagnosis of NF1.
Topics: Adult; Female; Humans; Image-Guided Biopsy; Magnetic Resonance Imaging; Neurofibroma, Plexiform; Radiography, Interventional; Spine; Tomography, X-Ray Computed
PubMed: 31342092
DOI: 10.1007/s00256-019-03281-2 -
Clinical Journal of Gastroenterology Dec 2020Plexiform neurofibroma is mainly associated with neurofibromatosis type 1 and is seldom observed in the liver. Its occurrence in the liver without neurofibromatosis type...
Plexiform neurofibroma is mainly associated with neurofibromatosis type 1 and is seldom observed in the liver. Its occurrence in the liver without neurofibromatosis type 1 is even rarer. We report an extremely rare case of plexiform neurofibroma of the liver diagnosed by laparoscopic biopsy in a patient without neurofibromatosis type 1. The patient was a 35-year-old man who had neither clinical signs nor any family history of neurofibromatosis type 1. Abdominal ultrasonography, as part of a health screening, had detected a hepatic tumor. Subsequent contrast ultrasonography, computed tomography, and magnetic resonance imaging showed the tumor extending from the retroperitoneal space around the aorta to the hepatic hilum and distal portal branches in the right hepatic lobe, gallbladder, and left hepatic lobe. F-fluorodeoxyglucose positron emission tomography showed no abnormal accumulation. Histopathological examination of the tumor obtained laparoscopically led to a diagnosis of plexiform neurofibroma. Because the patient was asymptomatic with no features of malignancy, he was only monitored and managed. At follow-up 10 years later, computed tomography showed a decrease in tumor size. It is important to recognize that, while rare, plexiform neurofibroma can occur without neurofibromatosis type 1. We recommend follow-up instead of unreasonable surgery in such cases.
Topics: Adult; Humans; Liver; Male; Neurofibroma, Plexiform; Neurofibromatosis 1; Tomography, X-Ray Computed; Ultrasonography
PubMed: 32918724
DOI: 10.1007/s12328-020-01227-z -
JAMA Dermatology Mar 2024
Topics: Humans; Child; Neurofibromatosis 1; Neurofibroma, Plexiform; Cafe-au-Lait Spots; Benzimidazoles
PubMed: 38198164
DOI: 10.1001/jamadermatol.2023.5338 -
The British Journal of Ophthalmology Aug 1993
Topics: Child; Female; Humans; Neurofibroma, Plexiform; Orbital Neoplasms; Tomography, X-Ray Computed
PubMed: 8025053
DOI: 10.1136/bjo.77.8.527 -
World Neurosurgery Mar 2022A 16-year-old male presented with solitary occipital plexiform neurofibroma, which had grown slowly over several years to reach giant size, thereby mimicking a "double...
A 16-year-old male presented with solitary occipital plexiform neurofibroma, which had grown slowly over several years to reach giant size, thereby mimicking a "double head." There were no features of neurofibromatosis type 1. Total excision of the neurofibroma was done by infiltrating adrenaline circumferentially around the tumor before scalp incision. The feeding arteries were identified in the dense connective tissue above the aponeurosis and ligated or cauterized. Dissection was then carried out in the loose areolar tissue preserving the periosteal layer. There was no evidence of malignant transformation on histopathology, and 5-year follow-up showed no tumor recurrence.
Topics: Adolescent; Humans; Male; Neoplasm Recurrence, Local; Neurofibroma; Neurofibroma, Plexiform; Neurofibromatoses; Neurofibromatosis 1; Scalp
PubMed: 34958991
DOI: 10.1016/j.wneu.2021.12.071 -
Diagnostic Cytopathology Jul 2024Isolated intraparotid neurofibromas are exceptionally rare and often associated with neurofibromatosis type 1 (NF1). Diagnosing these tumors proves challenging because...
Isolated intraparotid neurofibromas are exceptionally rare and often associated with neurofibromatosis type 1 (NF1). Diagnosing these tumors proves challenging because of the clinical resemblance to primary salivary gland masses. This case report details an 18-year-old with a painful, enlarging parotid mass, diagnosed through fine needle aspiration biopsy (FNAB) revealing myxoid stroma and spindle cells. Magnetic resonance imaging confirmed a plexiform neurofibroma involving the parotid gland and facial nerve. Histopathology validated the diagnosis, emphasizing the importance of cytological and radiological correlation. Notably, the absent NF1 association makes this case unique. Surgical excision with facial nerve reconstruction was performed, highlighting the complexity of managing such rare intraparotid neurofibromas. Awareness of this entity is crucial for accurate diagnosis and appropriate management.
Topics: Humans; Adolescent; Neurofibroma, Plexiform; Parotid Neoplasms; Biopsy, Fine-Needle; Male; Parotid Gland; Female
PubMed: 38595111
DOI: 10.1002/dc.25322 -
Medicina Oral : Organo Oficial de La... 2004The case reported deals with a solitary plexiform neurofibroma affecting the cheek submucosa. Neurofibroma is an uncommon tumor which rarely appears in oral cavity but... (Review)
Review
The case reported deals with a solitary plexiform neurofibroma affecting the cheek submucosa. Neurofibroma is an uncommon tumor which rarely appears in oral cavity but it represents the most common neurogenic tumor. Furthermore, plexiform variety is less frequent. Clinically, oral neurofibromas usually appears as anodyne and asintomatic lesions. Sometimes, they produce nervous compression. In this case, tumor is big but asintomatic. There is no definitive radiologic image. It has association with polyglandular syndromes and phacomatosis. The treatment of choice is excision. There are doubts of the surgical results so that some authors are looking for new non-surgical treatments. The clinical characteristics, epidemiology, diagnosis and treatment are described as soon as a bibliographic revisión.
Topics: Aged; Female; Humans; Mouth Mucosa; Mouth Neoplasms; Neurofibroma, Plexiform
PubMed: 15122129
DOI: No ID Found