Giant Plexiform Neurofibroma Research Articles

Emergency management and perioperative strategies for intra-tumoral hemorrhage in neurofibromatosis type 1-related giant plexiform neurofibroma

To review the emergency management and perioperative strategies for ruptured neurofibromatosis type 1 (NF1)-related giant plexiform neurofibroma (PNF), providing a systematic treatment protocol to improve the therapeutic outcomes and quality of life for patients with giant PNF. The literature on the management of giant PNF rupture and hemorrhage was reviewed, and the diagnosis, treatment, and perioperative management were summarized based on clinical experiences. By implementing an integrated diagnostic and treatment strategy that includes early diagnosis, imaging evaluation, emergency ultra-selective arterial embolization combined with surgical excision, acute hemorrhage can be effectively controlled while also reducing the risk of major intraoperative bleeding and minimizing postoperative complications. As a result, this approach significantly improves treatment success rates and patient quality of life. For ruptured NF1-related giant PNF, employing emergency ultra-selective arterial embolization combined with surgical excision, under the collaboration of a multidisciplinary team, can effectively improve treatment success rates, rapidly control bleeding, reduce tumor size, and lower mortality. Future research should focus on assessing the long-term quality of life of patients treated for ruptured and hemorrhaging giant PNF and on further optimizing treatment protocols.

Giant plexiform neurofibroma of the eyelid

Giant plexiform neurofibroma of the eyelid

Multiple Strategies to Avoid Life-threatening Blood Loss During Intralesional Resection of a Giant Plexiform Neurofibroma: A Case Report

Giant plexiform neurofibroma (PNs) are benign peripheral nerve sheath tumors known to contain multiple fascicles of nerve and numerous friable vascular components. Most consult due to significant disfigurement and functional deficit. Though surgery is the current standard of therapy, there is high reservation in pushing through with resection in most cases. The reservation stems from the recognized difficulty in controlling intraoperative life-threatening hemorrhage. A 25-year-old female came in our institution due to multiple debilitating giant PNs on her scalp, back, neck, shoulder, and chest. She opted for debulking surgery despite possible complications and recurrence. Multiple modalities used to prevent massive bleeding in this case included preoperative arterial embolization, energy sealing device, cutting linear stapler, and interlocking retention sutures. The aim of this case report was to discuss the utility of each of these techniques, the advantages and disadvantages of each approach based on our experience. Key words: Neurofibromatosis type-1, plexiform neurofibroma, hemorrhage, neurofibroma resection, preoperative arterial embolization

Giant plexiform neurofibroma of lower limb: Importance of radiology in diagnosis, complications and management

Plexiform neurofibromatosis is reported to occur in 26.7% of patients with type I neurofibromatosis. Plexiform neurofibromas present at, or soon after, birth as areas of hyperpigmentation, thickening of the skin and excess hair. In 50 percent of cases, it results from autosomal dominant transmission and in the remaining 50 percent of cases it arises as a spontaneous genetic mutation with no known cause. It affects all three germ layers and it is capable of involving any organ system. Plexiform neurofibroma pathognomonic of von Recklinghausen disease (neurofibromatosis type 1) and the gene responsible for the disorder is located in the chromosome region 17q11.2. The aim of this article is to report a rare case of giant plexiform neurofibromatosis in 15 year old Nigerian boy, its complications, its association with birth mark and hyperpigmentation, and its management.

The mystery of brown shawl over back: a rare case of giant plexiform neurofibroma underlying a giant café-au-lait macule

Neurofibromatosis is a rare autosomal dominant disorder characterized by café-au-lait macules and cutaneous neurofibromas. Café-au-lait spots are sharply defined, light-brown colored homogenous macules observed in more than 90% of cases. Plexiform neurofibromas are benign tumors originating from nerve sheath cells, subcutaneous or visceral peripheral nerves involving multiple fascicles often described on palpation as a bag of worms. We describe a case of 25-year-old male who presented with a giant plexiform neurofibroma underlying a giant café-au-lait macule.

Giant Plexiform Neurofibroma

We report a rare case of giant plexiform neurofibroma in a patient affected by type-1 neurofibromatosis and we describe the correct surgical management of such lesions in order to avoid intra- and post-op blood loss related complications.

Isolated giant plexiform neurofibroma of lower back: a rare case presentation

Plexiform neurofibroma is a rare benign tumor of the peripheral tissue cells that develops in the perineurium that is often considered pathognomonic of neurofibromatosis type 1 (NF1 or von Recklinghausen disease). They occur most frequently in the craniomaxillofacial region, rarely on back and extremities. They are extremely vascular and there is 15-20% potential for malignant transformation. A 35-year-old married female presented with painless swelling in left lower back that began at around 8 years of age. The swelling gradually increased in size. The patient reported embarrassment over the disfigurement caused by the mass. Her medical history was unremarkable and none of the relatives was known to be affected. Surgical resection of the swelling with primary closure was done. Histopathology findings were consistent with neurofibromatosis. Hereby reporting a rare case of isolated plexiform neurofibroma of lower back which was surgically cured as a perusal of rare entity. We also try to emphasize on the need of sprightful clinical diagnosis with multidisciplinary approach in the management of these type of tumors. Finally, we insist on the need of a long term clinical and radiological follow-up of these patients to assess post resection recurrence or malignant transformation.

Giant plexiform neurofibroma of thigh in a young woman

A 30-year-old woman presented to orthopaedic clinic with a 5-year history of severe pain and oedema of left lower limb. The patient denied experiencing any other constitutional symptoms and no...

Giant Plexiform Neurofibroma of the Pelvic Region: Unusual Presentation in an Uncommon Location (About a Case)

Giant Plexiform Neurofibroma of the Pelvic Region: Unusual Presentation in an Uncommon Location (About a Case)

Giant lumbosacral plexiform neurofibroma without associated neurocutaneous syndrome: An unusual presentation

Benign retroperitoneal and presacral neural sheath tumors of lumbosacral plexus in patients without Von-Recklinghausen's disease are quite rare with only few sporadic case reports. We report a case of giant unilateral plexiform neurofibroma of lumbosacral plexus extending into the thigh up to the level of knee joint in a patient without neurofibromatosis-1. Large unilateral retroperitoneal neurofibromas are rare while extension of the same up to the level of knee joint has not been reported yet. The purpose of the report is to highlight the characteristic imaging features in an unusual case of retroperitoneal neurofibroma extending up to the knee joint and thus avoiding unnecessary biopsy. Further imaging helps in defining presurgical extent of lesion, which in our case is essential as the lesion is seen extending along the entire course of the involved femoral nerve.

Giant Plexiform Neurofibroma of the Urinary Bladder

Giant Plexiform Neurofibroma of the Urinary Bladder

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.

Lower limb giant plexiform neurofibroma: preoperative evaluation and surgical planning

Few cases of lower limb giant plexiform neurofibroma have been reported in literature and there are no available guidelines for defining its preoperative evaluation and surgical approach . Lower limb giant plexiform neurofibroma is a rare condition that requires clinical and radiological preoperative evaluation, mainly with MRI. Surgery is the only treatment with no preoperative trans-arterial embolization or vessel ligation. Early excision of superficial lesions may prevent further progression.

Giant plexiform neurofibroma of the upper limb and anterior chest wall: case report and review of the literature

Plexiform neurofibroma is generally accepted as a component of neurofibromatosis type 1. It is frequently seen on the head and neck regions, and extremity involvement is rare. In this report, a case of giant plexiform neurofibroma located on the upper extremity and anterior chest wall is presented.

Pregnancy with Elephantiasis Neurofibromatosa—A Rare Presentation

Neurofibromatosis (NF1) is an autosomal dominant disorder primarily affecting the development and growth of nerve cell tissues. We report a rare case of pregnancy with giant plexiform neurofibroma involving the genital tract with adverse perinatal outcome. A 26-year-old female presented at term in labour with NF1 and a huge plexiform neurofibroma arising from sacral region, gluteal region, pelvis and involving left labium majus. She delivered by cesarean section and the baby had congenital NF1. The baby expired on day 4 of life. To the best of our knowledge, this large size neurofibroma involving this site in pregnancy has never been reported in English literature. Both pregnancy and NF1 have reciprocal adverse effects on each other. Congenital neurofibromatosis can be fatal. Hence, all pregnancies associated with NF1 should be considered as high risk and monitored carefully.

Congenital giant plexiform neurofibroma with occipital calvarial dysplasia in association with meningoencephalocele in neurofibromatosis Type 1 and segmental neurofibromatosis

Giant plexiform neurofibroma (GPNF) of the scalp is an extremely rare lesion reported in association with neurofibromatosis. Occipital location of GPNF is even more infrequent, especially in association with occipital dysplasia (OD). The authors report 2 pediatric cases of GPNF associated with OD. The first case had an associated meningoencephalocele, and the second had large vascular channels within the lesion and the dominant ipsilateral transverse sinus lying in the center of the calvarial defect. The authors present these 2 unusual cases with a review of literature and discuss the radiological findings, theories of etiopathogenesis of the OD, and management dilemmas.

Giant Solitary Intramuscular Cystic Plexiform Neurofibroma of the Thigh: A Case Report and Literature Review

Objective: To report a rare occurrence of giant, solitary, cystic, intramuscular plexiform neurofibroma in the thigh. Case Report: A 45-year-old architect presented with a 5-year history of painless, progressively enlarging soft tissue mass in the right thigh, which was diagnosed on histology as cystic plexiform neurofibroma after excision biopsy. Conclusion: A plexiform neurofibroma should be considered in the differentials of a solitary cystic mass in the thigh even in the absence of usual manifestations of Neurofibromatosis.

Multidisciplinary management of a giant plexiform neurofibroma by double sequential preoperative embolization and surgical resection.

Plexiform neurofibromas are benign tumors originating from subcutaneous or visceral peripheral nerves, which are usually associated with neurofibromatosis type 1. Giant neurofibromas are very difficult to manage surgically as they are extensively infiltrative and highly vascularized. These types of lesions require complex preoperative and postoperative management strategies. This case report describes a 22-year-old female with a giant plexiform neurofibroma of the lower back and buttock who underwent pre-operative embolization and intraoperative use of a linear cutting stapler system to assist with haemostasis during the surgical resection. Minimal blood transfusion was required and the patient made a good recovery. This case describes how a multidisciplinary management of these large and challenging lesions is technically feasible and appears to be beneficial in reducing perioperative blood loss and morbidity. Giant neurofibroma is a poorly defined term used to describe a neurofibroma that has grown to a significant but undefined size. Through a literature review, we propose that the term “giant neurofibroma” be used for referring to those neurofibromas weighing 20% or more of the patient's total corporal weight.

Surgical treatment of giant plexiform neurofibroma associated with pectus excavatum

Plexiform neurofibromas are benign tumors originating from subcutaneous or visceral peripheral nerves, which are usually associated with neurofibromatosis type 1. They are almost always congenital lesions and often cause the surrounding soft tissue and bone to grow aberrantly. We treated a 12-year-old boy who presented with asymmetric pectus excavaum and an anterior chest wall plexiform neurofibroma. The pectus excavaum was corrected by modified Nuss procedure, followed by simultaneous resection of the giant mass. The patient is doing well at the 4 years follow-up visit.

Giant plexiform neurofi broma presenting with craniovertebral junction instability

Giant plexiform neurofi broma presenting with craniovertebral junction instability