Large penile plexiform neurofibroma in an 11-year old boy (original) (raw)
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Two cases of plexiform neurofibromatosis at unusual sites
IP innovative publication pvt. ltd, 2019
Plexiform neurofibromatosis (PNF) is a potentially devastating type of neurofibromatosis, but is rare to occur. PNFs are benign tumours that originate from nerve sheath cells, visceral or subcutaneous peripheral nerves that can involve multiple fascicles and present as large tumours attached to major nerves. They are associated with NF-1 lesions in majority of cases and present with bulging and deforming masses involving skin folds and connective tissue most commonly at sites of trigeminal nerve distribution, and may also present as isolated lesions. There are high chances of malignant transformation in these lesions and thus should not be left untreated. Due to paucity of options available for treatment, surgical debulking remains the most important management. Being an autosomal dominant disorder, genetic counseling is necessary. We are presenting two cases of PNFs, occurring at unusual sites like sole of foot and forearm.
Giant plexiform neurofibroma of digital nerves: A case report and review of the literature
Neurofibroma is a benign tumor arising from nerve sheath of peripheral nerves, and may present as an isolated lesion or may present as part of von Recklinghausen’s disease. Although plexiform neurofibromas usually affect the face, neck, back, and inguinal region, however other areas may rarely be affected. Plexiform neurofibroma of the palm is a very rare entity, which may affect the fine motor functions of the hand. Surgery is the only treatment option available, but due to diffuse involvement of the nerves and infiltrating nature of the lesions, complete resection may be impossible in many of the cases. We are reporting a case of giant neurofibroma of the palm affecting digital nerves in a 37-year-old patient presenting with features of Von Recklinghausen’s disease. He presented with pain and swelling over left palm of 8 years duration. We carried out the surgical excision of the mass, and the individual has been followed-up on out-patient basis for about 3 months without showing any evidence of local recurrence.
Management of large dorsal diffuse plexiform neurofibroma
European Journal of Plastic Surgery, 2014
Neurofibromas are considered one of the hallmark signs of neurofibromatosis type 1(NF1). Diffuse plexiform neurofibroma is a congenital NF1-associated tumor, characterized by overgrowth and interference with function of the affected area. The rich vascular plexus associated with neurofibromas, together with their infiltrative pattern, makes them difficult to eradicate. Complications of neurofibromas are rare but include malignant transformation and potentially lifethreatening hemorrhages. The use of the term "giant" to define a neurofibroma is controversial because there is no clear consensus and descriptions are limited to few case reports. We report a case of a large dorsal diffuse plexiform neurofibroma responsible for significant worsening of a patient's quality of life. These features make us believe that the mass can be defined as "giant." Comprehensive perioperative management, including arterial embolization of the feeding vessels and stabilization of the patient's conditions, is required to perform a safe surgical procedure in such a complicated case.
Extensive Plexiform Neurofibroma Presenting as Clitoromegaly in Neurofibromatosis Type 1
Journal of Dr. Behcet Uz Children s Hospital, 2021
Neurofibromatosis type 1 (NF1) is an autosomal-dominant disorder with multisystem involvement. Genitourinary involvement of neurofibromatosis type 1 is rare condition and involvement of plexiform neurofibroma can cause painful clitoromegaly. A 9-year-old girl with neurofibromatosis type-1 referred with clitoromegaly to our endocrinology clinic. Pelvic magnetic resonance imaging (T2W images) showed that plexiform neurofibroma was found on the pelvic floor and peripubic region, which was 8x7x13 cm in size, extending to the external genital region and progressing to subcutaneous soft tissue. Cranial and lumbosacral magnetic resonance imaging revealed two neurofibromas in the cerebellar region and the basal ganglia. We emphasize it should be kept in mind that suspicious genitalia may develop due to infiltration of space occupying formations such as neurofibromas.
PlexiformNeurofibromatosis of Vulva; A case report
Plexiform Neurofibromatosis of vulva is a rare, benign tumor of genital tract arising from nerve sheath of peripheral nerve. It may present as solitary lesion or as part of Von Recklinghausen’s disease. Genitourinary neurofibroma is rare, however clitoris and labia majus happen to be the most frequent location of neurofibromatosis involving female genital tract. We present a case of plexiform neurofibroma arising from left labia majora in a young patient having features of Von Recklinghausen's disease. She complained of discomfort while walking due to rubbing of pedunculated mass arising from her labia majora. We carried out the surgical excision of the mass followed by primary repair of labia majora. She has been followed up on out-patient basis for about three months without any recurrence so far.
Cervical and Spinal Plexiform Neurofibroma: A Case Report
Journal of Pediatrics Research Reviews & Reports, 2021
Plexiform neurofibroma is a rare benign tumor of the peripheral nerves at the expense of perineural connective cells. It is pathognomonic of neurofibromatosis type 1 (NF1 or Von Recklinghausen disease). MRI is of great help in the diagnosis of this pathology. Anatomopathological confirmation is sometimes necessary, especially in the absence of a context suggestive of NF1. We report the observation of an oung boy with a cervical plexiform neurofibroma revealing a neurofibromatosis Type 1
Facial plexiform neurofibroma in a child with neurofibromatosis type I: A case report
Journal of Indian Society of Pedodontics and Preventive Dentistry, 2007
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. The diffuse and soft nature of plexiform neurofi broma is often compared to 'a bag of worms' and is difficult to distinguish from a vascular malformation or a lymphangioma, thus necessitat ing thorough clinical and histopathological examination and imaging of the lesion. We present a case of plexiform neurofibroma in a 12-year-old male child.