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Plexiform neurofibromas are peripheral nerve sheath tumors associated with neurofibromatosis type 1. The maxillary sinus is an extremely rare location of the plexiform neurofibroma and only two adult cases have been previously reported. We report the first case of plexiform neurofibroma of the maxillary sinus occurring in a child with neurofibromatosis type 1. This unusual location presents a management challenge considering the infiltrative nature and the potential malignant degeneration of this type of tumor. MRI is highly valuable to diagnose and plan the surgical approach of the plexiform neurofibroma of the maxillary sinus. Due to the location of the tumor and the patient's age, conservative surgery is highly recommended. We performed an endoscopic total en-bloc resection of the tumor with no recurrence after nine months of follow-up.

A 19-year-old woman with type I neurofibromatosis requested reconstruction of her severe facial disfigurement caused by the plexiform neurofibroma of the right upper eyelid. Previously, she had glaucoma surgery for buphthalmus and enucleation with dermis fat transplantation. She was unable to wear prosthesis in the last year. Transverse palpebral resection of the lesion, lateral canthal reconstruction, and frontal sling ptosis surgery were performed at the same session. Postoperatively, the cosmetic appearance of the patient was markedly improved. No complication or progression was observed during 2 years follow-up. We believe that an individual-based surgical plan may give acceptable results in these challenging cases.

Plexiform neurofibroma (PN) is a rare benign tumor and a special subtype of neurofibromatosis type 1 (NF1). Though the incidence is low, giant PN of the craniomaxillofacial region could result in severe hemifacial hypertrophy which is known as a typical manifestation of NF1 in young children. Here, we retrospectively reported a giant plexiform neurofibroma with hemorrhage in the cranio-maxillofacial region detected by CT and MRI. In addition, a brief review of the relevant literature is presented.

Introduction: We report a case of total oral rehabilitation with basal implants (cortically anchored disk-design implants) on a patient who received a facial allograft 1 year earlier., Observation: A 31-year-old patient was suffering from a plexiforme neurofibroma spread into the soft tissues of the oral cavity with huge deformations of the jaws. The operation consisted in pulling out numerous supernumerary impacted teeth, removing unnecessary soft tissues, settling six basal implants in the maxilla and seven in the mandible. The following day, two resin bridges were adjusted and cemented onto the implant abutments. The permanent bridges were settled 2 months and half later. The outcome was still unremarkable 2 years after the implant procedure., Discussion: This case report raises important issues, notably the relevance of an oral rehabilitation with implant-supported prostheses on an immunosuppressed patient. In this specific case, this was impossible to achieve using removable prostheses. A facial reconstruction with its plastic, functional, and social goals seemed inconsistent without an oral rehabilitation. Even if they have not been published, cases of grafted patients with dental implants are unexceptional. Lastly, this rehabilitation has been planned for a particularly monitored patient. The second important question regards the choice of the basal implantology. This option has been motivated by the possibility to complete the case in one sole operation with provisional prostheses cemented the day after that act like orthopaedic external fixators providing an exceptional primary stability. The cortical anchorage, which was the only reliable on this patient, allowed to avoid bone grafting. Finally, the particular thinness of the implant emergence limits to the minimum the communications between bone and oral cavity. We would like to stress that the only objective of this observation was to document a specific case and possibly to open the way to a scientific study in accordance with the regulations of clinical research.

Trigeminal plexiform neurofibromas involving the orbit and the infratemporal fossa are rare tumours. An uncommon case of a plexiform neurofibroma of the trigeminal nerve occurring in a 53-year-old female patient with severe cosmetic deformity is reported. The clinical findings, neuroradiographic features, surgical management, postoperative results and follow-up of trigeminal plexiform neurofibromas are reviewed.

Plexiform neurofibromas (PN) are one of the most common and severe types of neurofibroma that occur in neurofibromatosis type I. These tumours affect long portions of nerves, infiltrating the nerve and surrounding tissue thus causing significant pain, deformity and functional problems in the affected part of the body. Treatment of this variant of neurofibromas is currently surgical. The aim of this study was to analyze the surgical treatment of plexiform neurofibromas in the lower and upper extremities. The clinical pathological features of 29 neurofibromas, 12 in the upper extremities and 17 in the lower extremities, as diagnosed at the Department of Plastic and Reconstructive Surgery of University "La Sapienza" in Rome from 2000 to 2007, were reviewed. We established that subtotal and total resection without functional destruction is often possible for superficial PN.

Plexiform neurofibroma at the thoracic inlet has rarely been reported and to our knowledge, the use of a temporary middle claviculectomy approach for thoracic inlet tumors has never been reported. We report a case of plexiform neurofibroma of the first intercostal nerve resected using a temporary claviculectomy approach. An abnormal shadow detected radiographically in a 16-year-old boy led to a diagnosis of neurofibromatosis 1 (NF-1) with a chest wall tumor at the thoracic inlet. The patient underwent resection of the tumor with the right first rib. The resected clavicle was reapproximated with a plate and postoperative shoulder function was satisfactory. The tumor was diagnosed pathologically as a plexiform neurofibroma and the patient's postoperative course was uneventful. The temporary middle claviculectomy approach provides excellent exposure of the subclavian vessels and brachial plexus before resection of the tumor. We recommend this approach for tumors of the anterior thoracic inlet.

Genitourinary neurofibromas with clitoral involvement in neurofibromatosis type 1 are rare, and even more infrequent are the neurofibromas involving genitalia in males. The most frequent presenting sign of neurofibroma in females is clitoromegaly with pseudopenis, and enlarged penis is the most common sign in males. Labium majus neurofibroma not associated with clitoral involvement is extremely rare. Magnetic resonance imaging demonstration of the neurofibromas has seldom been reported. We report 4 children, 3 girls and 1 boy, with plexiform neurofibromas involving the external genitalia. Three of the 4 patients had histologic confirmation of neurofibroma. Two girls with clitoral hypertrophy had a neurofibroma that infiltrated the clitoris and extended unilaterally to the lower bladder wall. One girl had a plexiform neurofibroma that affected a labium. One boy with asymmetric penile hypertrophy since 2 years of age and ipsilateral gluteal hypertrophy had plexiform neurofibromas that extended between the left lumbogluteal and penile regions, infiltrating the left rectum wall and bladder with compression of both structures, the left prostate, and the left half of the cavernous corpi with hypertrophy of this part and asymmetry of the penis. Magnetic resonance imaging demonstrated in all patients that external genitalia and plexiform neurofibroma formed images of nondetachable structures. However, hermaphroditism was discarded by chromosomal study in all 3 girls before ratifying the diagnosis of external genitalia neurofibroma.

Background: The risk to benefit ratio of face transplantation with a composite tissue allograft remains debatable, although this procedure is technically feasible. We report here a 1-year follow-up of a patient who underwent face transplantation with a composite tissue allograft., Methods: On Jan 21, 2007, a 29-year-old man with neurofibromatosis type 1 underwent resection of a massive plexiform neurofibroma diffusely infiltrating the middle and lower part of his face. The main goal was to restore both the cutaneous appearance and function of the face, including, in particular, control of orbicularis oculi and oris muscle contraction. The issues of immunosuppressive therapy, psychological outcome, and social reintergration were addressed, together with the monitoring of graft rejection by biopsies of the skin and mucosa., Findings: The initial postoperative course was uncomplicated. Two episodes of clinical rejection occurred on days 28 and 64. The second episode was associated with cytomegalovirus infection. Both episodes resolved favourably, with no further clinical signs of rejection, making the reduction of immunosuppressive treatment possible. A year after surgery, the functional outcome was very good, with successful sensory and motor reinnervation in the transplanted territory. Psychological recovery was excellent, with complete social reintegration., Interpretation: This case demonstrates the feasibility of surgically removing a large part of the face and replacing it with a composite tissue allograft. This facial repair procedure, which seems to have a satisfactory risk to benefit ratio, could be offered in rare and selected cases.

Laryngeal neurofibromas are rare. The plexiform subtype is less common and often presents during childhood in association with neurofibromatosis type I. Because it is unencapsulated and more aggressive, plexiform neurofibroma presents a management dilemma. Imaging, particularly magnetic resonance imaging, can aid in diagnosing neurofibroma, differentiating the nonplexiform and plexiform subtypes, and planning the subsequent operative approach. The importance of conservative surgery to relieve symptoms, but preserve laryngopharyngeal function, and close follow-up are stressed. We report 2 adult cases of plexiform neurofibroma involving the larynx and hypopharynx and discuss the controversies in management.

Introduction: Primary neurogenic tumours of facial nerve are uncommon with the majority found intra-temporally. Intracranial and intra-parotid neoplastic involvement of cranial nerve VII is much less common. There are 11 reported cases, in the English-language literature, of intra-parotid facial nerve plexiform neurofibromas with eight of them associated with NF1., Materials and Methods: A child, 10 years old, with NF1, reached us for a cheek swelling, slowly increased in previous 8 years. At the age of 3 years, a plexiform neurofibroma was diagnosed by biopsy of the lesion. Clinical examination and NMR showed in the sub-cutaneous tissue of the right cheek, two contiguous nodular lesions, about 2 cm x 1.5 cm in diameter; a third neoformed lesion, about 1cm in diameter, was located above the ipsilateral labial commissure. No facial nerve impairment was seen. The patient underwent superficial parotidectomy with removal of the lesions and preservation of the facial nerve., Results: The patient had a considerable regional swelling in the immediate post-operative course; no facial nerve impairment was observed. The swelling of the cheek did not show a fully regression in the post-operative course. Ultrasonography at 3 months showed a recurrence of disease., Discussion: Plexiform neurofibromas should be distinguished due to their risk of malignant transformation seen in up to 15% of patients affected by NF1. Surgery is the only effective option currently available for the treatment of PNF. However, success of surgical intervention is limited by the infiltrating nature of the tumours, resulting in a high rate of tumour re-growth. Facial nerve preservation during surgery is unlikely and significant morbidity can result from their excision. The age of the patient at surgical resection seemed to influence outcome: tumours resected before age 10 years recurred in 60% of cases compared with only 30% recurrence in patients older than the age of 10 years., Conclusion: Indication and timing of surgery, in paediatrics patients with NF1, are complex. To avoid eventual physical and psychological consequences, it seems prudent to delay surgery as long as it is feasible for otherwise asymptomatic paediatric patients with facial plexiform neurofibroma.

A 32-year-old female patient visited the emergency room because of sudden onset of abdominal pain, which was found to be induced by hemorrhagic infarction of a plexiform neurofibroma arising from the lesser omentum. No other classic clinical characteristics of systemic von Recklinghausen's disease had been seen up until now. The tumor, classified as sporadic plexiform neurofibroma, rarely occurs in the digestive tract. This diagnosis was confirmed by exploratory laparotomy with complete resection of the tumor. Here, we describe the rare case of a sporadic intra-abdominal plexiform neurofibroma and discuss the clinical characteristics and management of plexiform neurofibroma.

The objective of this article was to describe a case of Von Recklinghausen's disease with plexiform neurofibroma of the penis. The patient was 26 years old and reported difficulty with sexual intercourse owing to a penile mass, despite experiencing normal sensation and erection. Physical examination demonstrated this penile mass with café au lait spots, subcutaneous neurofibromas, and lisch nodules. The treatment consisted of subcoronal incision and resection of the lesion. Pathologic examination revealed a plexiform neurofibroma. The patient recovered well with subsequent maintenance of penile sensation and erection. In cases of plexiform neurofibroma compromising penile function, total resection of penile mass offers a good therapeutic option.

Plexiform neurofibroma of the cauda equina is extremely rare. Herein, a case is reported in a 56-year-old man who also had muscle atrophy, severe motor disturbance, and mild sensory disturbance of the left leg due to childhood poliomyelitis. The clinical symptoms manifested insidiously until the patient developed cauda equina syndrome. At operation, the tumor mass was observed to engulf the nerve roots of the cauda equina. After pathological examination, the diagnosis of plexiform neurofibroma was made. Neither the patient described herein nor any previously reported patients had neurofibromatosis.

Cervical cord compression from cervical root neurofibromas represents an important clinical problem in patients with neurofibromatosis type 1 (NF1), but is rarely reported. The aim of this study was to describe the clinical presentation and follow-up of children and adults with NF1 and cervical cord compression. A retrospective review of clinical records and neuroimaging studies from two large tertiary care centres between 1996 and 2006 was performed. 13 patients with NF1 and cervical cord compression were identified. Age at presentation ranged from 9 to 61 years. The most common presentation was progressive quadriparesis. 11 of 13 patients underwent cervical decompression and subtotal resection of the associated neurofibroma. The majority of patients had recovery of neurological function and no further clinical progression. Progressive neurological deficit (typically quadriparesis), rather than neuroimaging appearances, should dictate the need for surgery.

Massive plexiform neurofibromatosis is an uncommon manifestation of Type 1 neurofibromatosis. These tumours result in functional disability and severe disfigurement. Due to the variable natural history od these lesions, the optimal management is not well defined and surgery is often delayed until significant disfigurement has occurred. The aim of this report is to discuss the optimum timing of surgical intervention and to highlight the difficulties in dealing with an advanced lesion.

Plexiform neurofibroma is generally considered as a component of neurofibromatosis 1, and a great majority of the cases with plexiform neurofibroma display other symptoms related to neurofibromatosis. Plexiform neurofibromas occur frequently in the head and neck region due to the rich innervation of the area, however appear rarely in the extremities. We report here an isolated giant plexiform neurofibroma involving the common peroneal nerve branches without symptoms related to neurofibromatosis in a 5-year-old case. Surgical excision was performed due to pain and numbness in the leg, and against the possibility of malignant transformation due to sudden growth observed in the tumor. One month following the excision of all the reachable tumoral tissues, tendon transfer surgery was performed for the ankle and toe extensions. Our case stands as the only reported case of isolated giant plexiform neurofibroma involving the common peroneal nerve in the pediatric age.

We experienced the case of an 11-year-old boy diagnosed as having type 1 neurofibromatosis with intraabdominal and pelvic masses. On physical examination, there were multiple café-au-lait spots larger than 15 mm in diameter scattered over the patient's entire body and axillary freckling, but no cutaneous neurofibromas were present. Lisch nodules were detected in the iris by a slit lamp. A large, firm mobile mass was palpated in the lower abdomen. Abdominal computed tomographic scan showed the hypodense masses in the lower abdomen and pelvic cavity. At laparotomy, a 16 x 9-cm, firm nodular mass along the mesentery of the terminal ileum and ascending colon was found. The mesenteric mass was encasing the superior mesenteric vessels and extending into the serosa of the intestine. The mass was incompletely excised together with affected intestine. However, resection of the rectum could not be performed because of the extensive involvement of the entire mesorectum. Histopathologic study revealed a plexiform neurofibroma involving the mesentery and intestine without evidence of malignant transformation. The postoperative course was uneventful. Ten months after the operation, a magnetic resonance imaging showed no interval change of the mesorectal mass.

A 12-year-old girl with neurofibromatosis type 1 underwent subtotal anterior exenteration of a blind, unsightly, buphthalmic eye and orbital/eyelid plexiform neurofibroma. Simultaneously, a channeled Medpor orbital implant was positioned to reduce a meningoencephalocele resulting from severe sphenoid wing dysgenesis. Two-stage osseointegration was subsequently performed to permit secure fitting of an orbital prosthesis. This patient's cosmesis and social interaction were improved markedly with this surgical approach for disfiguring ocular, eyelid, and orbital disease due to neurofibromatosis type 1.

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 neurofibroma is often compared to 'a bag of worms' and is difficult to distinguish from a vascular malformation or a lymphangioma, thus necessitating thorough clinical and histopathological examination and imaging of the lesion. We present a case of plexiform neurofibroma in a 12-year-old male child.

We describe a unique case of a 44-year-old gentleman who presented to our hand department with a history of a lump within his right palm that spanned several decades. Its gradual increase in size together with pain on palpation merited further investigation and subsequent surgical excision. Extensive tumour emanating from both ulnar and median nerves was resected, the histopathological diagnosis of which was plexiform neurofibroma. We believe this is the first description of an isolated plexiform neurofibroma of the hand with dual nerve origin.

Object: The operative management of combined intrapelvic and extrapelvic sciatic notch dumbbell-shaped tumors is challenging. The relatively rare occurrence of these tumors and the varied extent of disease have made it difficult for surgeons to establish definitive surgical indications or predict favorable neurological outcomes based on preoperative imaging data., Methods: In the past 3 years, the authors treated five patients presenting with radiating leg pain as a result of benign sciatic notch dumbbell-shaped tumors. These tumors in three patients with unilateral leg symptoms were considered unresectable by other neurosurgeons because of presumed direct intrinsic neural involvement. After high-resolution magnetic resonance (MR) imaging demonstrated that the extensive tumors were separate from the sciatic nerve and the lumbosacral plexus, however, these patients underwent a combined one-stage transabdominal and posterior transgluteal complete resection. Normal neurological status was maintained postoperatively in these three patients, and after more than 1 year of postoperative follow up, there were no tumor recurrences. In two patients with bilateral symptoms and extensive tumor burden, serial MR images showed that innumerable tumors directly involved the entire cross-sectional area of the sciatic nerves and extended longitudinally to the lumbosacral plexuses. Tumor debulking or resection in these patients would have resulted in neurological deficits and would not have addressed their neuropathic pain, and therefore no surgery was performed. These two patients were treated pharmacologically and advised to monitor their tumor status over the course of their lifetimes in case of malignant transformation of the tumor., Conclusions: A combined one-stage transabdominal and transgluteal approach allows safe resection of selected benign but extensive sciatic notch tumors. High-resolution MR imaging is a useful tool in the management of these tumors because it allows the surgeon to visualize the anatomical relationships of the tumor to the sciatic nerve. The authors believe that as this imaging technology advances, it will provide surgeons with a method to predict definitively which sciatic notch tumors displace rather than directly involve the sciatic nerve, and therefore indicate which tumors can be resected safely and completely.

We report an uncommon case of solitary subungual neurofibroma on the right first finger of a middle-aged healthy woman. Surgical excision was used for the treatment. Common clinical findings, treatment modalities, and reported recurrence rates in previous studies are reviewed.

Objective: Plexiform neurofibromas of the head and neck in neurofibromatosis type 1 (NF 1) carry a significant morbidity with substantial loss of function as well as significant cosmetic problems. We describe our experience with early aggressive surgical intervention in such patients in order to avert these problems., Methods: Retrospective review of four consecutive pediatric patients with massive head and neck plexiform neurofibromas who underwent single stage near total or sub-total tumor resections., Results: All four patients were referred for obstructive airway symptoms. Each patient experienced complete relief of symptoms and return of function without additional neurological deficits. There were two minor complications and no major complications of surgical resection. There have been no recurrences to date, with follow-up ranging from 15 months to 5 years., Conclusions: Early surgical intervention of NF 1 patients with plexiform neurofibromas of the head and neck with a goal of near total resection avoids the loss of function associated with these tumors, such as tracheostomy dependence, swallowing difficulty, and speech problems, and prevents the inexorable progression of substantial cosmetic deformity. Successful management of these complex lesions requires detailed preoperative planning, advanced surgical techniques, and vigilant postoperative care.

Plexiform neurofibromas traditionally have posed a surgical challenge in pediatric patients. Expert preoperative planning, advanced surgical techniques, and vigilant postoperative care results in minimal morbidity and resolution of tumor symptomatology. A retrospective review of four consecutive pediatric patients with massive head and neck neurofibromas who underwent single-stage total or near-total removal of their tumors was performed. All four patients had obstructive airway symptoms. There were two minor complications. All patients had complete relief of their symptoms and return of function without any additional neurologic deficits. It is possible to safely achieve total or near-total removal of extensive plexiform neurofibromas with minimal morbidity while restoring lost function.

A three-year-old girl with a lingual plexiform neurofibroma treated by total excision is presented. Despite their occurrence in the head and neck region, neural sheath tumors are rarely encountered in the oral cavity. It is reported that 4-7% of patients affected by neurofibromatosis display oral manifestations. Neurofibromatosis is characterized by café-au-lait spots and cutaneous neurofibromas. Plexiform neurofibroma is said to be indicative of von Recklinghausen's disease (VRD) even though it may be the only manifestation of the disease. Generally, surgical resection represents the treatment of choice and the diagnosis can only be confirmed after histological examination. Affected patients need regular follow-up to detect malignant degeneration, an early recurrence or appearance of other manifestations of VRD.

Background: Neurofibromatosis type 1 manifests itself by the development of plexiform neurofibromas, with craniofacial deformities caused by bone alterations and soft-tissue infiltration. The medical treatment of this disease is disappointing, and the surgical management of these deformities calls for aggressive procedures. There is a strong risk of recurrence., Methods: A pilot study was performed to test radiofrequency as a minimally invasive method with which to diminish the size of craniofacial neurofibromas either as an adjunct to craniofacial surgery (in cranio-orbital neurofibromas) or to avoid a visible scar in young patients (in infraorbital neurofibromas). Five patients ranging in age from 6 to 18 years were treated by three procedures 2 months apart using radiofrequency performed under local anesthesia or under sedation for the youngest patients., Results: The tolerance of the treatment was excellent, with no major side effects and no pain in the postoperative course. A diminution of the size of the lesion was noted clinically in four patients and on computed tomography in two patients. A biopsy performed in one case illustrated the effect of the treatment., Conclusions: A partial diminution or stabilization of plexiform neurofibromas may be obtained using radiofrequency. This treatment is well tolerated. The best effect can be observed in the early stages of the disease. The optimal dose and frequency of the procedure require further study.

The authors present clinical, imaging, and histological features of two adult patients found to harbor a conventional schwannoma contiguous with a deep plexiform schwannoma. One patient had neurofibromatosis (NF) Type 2 (NF2), and both intracranial (bilateral oculomotor, trigeminal, acoustic, and hypoglossal schwannomas as well as meningiomas) and intraspinal (schwannomas and meningiomas) lesions. The proximal forearm lesions consisted of a conventional schwannoma and an underlying plexiform component. The second patient, who did not have NF2, presented with a similar enlarging mass in the distal arm; two contiguous lesions were resected. Both patients exhibited distinct, readily identifiable, magnetic resonance imaging features. Plexiform schwannomas are rare lesions that occur sporadically or, on occasion, in association with NF2 or meningiomas with or without multiple schwannomas. The authors believe that a more careful examination of patients with NF2 may show that these people have a higher incidence of plexiform schwannoma than previously thought. Pathological confirmation is crucial in distinguishing plexiform schwannomas from plexiform neurofibromas, because the latter are directly associated with NF1 and have a significant tendency to undergo malignant transformation. To the best of the authors' knowledge, this is the first report of a conventional schwannoma contiguous with a deep plexiform schwannoma.

Objectives: The present case is a diffuse isolated plexiform neurofibroma of the tongue that was not associated with neurofibromatosis that we treated with intraoral surgery., Study Design: A case report., Methods: We present a 5-year-old girl with isolated plexiform neurofibroma of the tongue with infiltration of the tongue base and review treatment approaches for this very rare tumor., Results: An intraoral approach appears to be a good alternative method that allows both an acceptable exposure for total excision and limited postoperative sequelae., Conclusions: Isolated plexiform neurofibroma of the tongue is very rare. It causes a massive macroglossia that is progressive with advance of years and disturbs the patient's speech, occlusion, and tongue movements. Involvement of the tongue may be associated with infiltration into the deep structures of the neck by the tumor, thereby presenting considerable operative difficulties.

This disease was first described by von Recklinghausen and Festscher and has been known as the von Recklinghausen's disease or neurofibromatosis (NF). Numerous articles have been published on this subject where majority of the authors have stressed their difficulty in achieving favourable surgical results. This disease is pathologically accepted to be of neuroectodermal origin with a positive family history in approximately 50% of the cases, autosomal dominant trait and is known to involve the periorbital regions, orbit (preoperative CT scan: upper left and right photographs), temporal region to a variable extent, mid-facial region to the mandibular region. The indicated treatment for this disease is surgery including cranio-maxillofacial surgery even though re-evaluation of the conventional methods of surgery should be considered. This particular case is an extremely difficult and challenging case for any reconstructive plastic surgeon and required extensive preoperative planning. The systematic multiple stage surgical approach for an extremely severe von Recklinghausen's disease, elephantiasis neurofibromatosa is presented and discussed in detail.

Introduction: Orbito-temporal plexiform neurofibroma is characteristic of Von Recklinghausen neurofibromatosis. We report 6 cases of orbito-temporal plexiform neurofibromas and review different aspects of diagnosis, course and treatment., Patients and Methods: Six patients, 4 males and 2 females, with orbito-temporal plexiform neurofibromas were treated between 1986 and 2003. Patient age varied between 10 and 29 years (age average=19). Computed tomography and magnetic resonance imaging were performed in all 6 patients. Surgical resection of the neurofibroma was performed in all patients., Results: The tumor was located on the left in 5 cases and on the right in one. Spheno-orbital dysplasia was found in all patients and was associated with fronto-temporal meningoencephalocele and pulsate exophthalmia. Surgical resection enabled reduction of the neurofibroma. Exenteration was performed in all patients because of a nonfunctional eye. Two patients underwent cure of fronto-temporal meningoencephalocelus with reconstruction of the large wing of the sphenoid using an iliac bone graft and a titanium plate., Discussion: Surgery is essential for orbito-temporal plexiform neurofibroma but is usually difficult, particularly when the tumor has developed increasing the risk of hemorrhage. Bony reconstruction is difficult considering the risk of progressive osseous dysplasia. The cosmetic results achieved in this patient were considered good.

Objectives: To identify presenting symptoms, growth patterns, and outcomes of head and neck plexiform neurofibromas (PNs) in children with neurofibromatosis type 1 (NF-1); to determine which patients may benefit most from operative intervention in terms of duration of disease-free progression, perioperative morbidity, identification of malignancy, and symptom relief., Design: A retrospective review of 39 pediatric patients with NF-1 who had PNs of the head and neck managed at a single tertiary referral center., Results: Thirty-nine patients had 49 head and neck PNs, 11 small (5 cm and/or involving multiple deep neck sites). Thirty-nine surgical procedures were performed on 18 of 35 patients with massive disease, and 4 procedures were performed on 4 of 11 patients with small tumors. Tumors recurred in 1 (25%) of 4 patients with small tumors and in 18 (100%) of 18 patients with massive tumors (P = .001; mean time to regrowth, 3.1 years.), Conclusions: Size and location of PN tumors most influenced presentation of clinical symptoms. Complete tumor resection was possible only in patients with small PNs. Patients with PNs of the head and neck were more likely to benefit from surgery if the indications were to (1) exclude malignancy in a rapidly enlarging mass; (2) provide relief from neurogenic pain or motor weakness; (3) improve symptoms caused by airway compression; or (4) enhance cosmesis in those with disfiguring disease.

The female genital system is rarely affected in von Recklinghausen neurofibromatosis. The vulva is the most frequent genital location, but vaginal, cervical, uterine, and ovarian neurofibromas have rarely been reported. We describe a case of plexiform neurofibroma affecting the uterine cervix in a patient with chronic pelvic pain and menorrhagia who had multiple cutaneous neurofibromas and 1 large paraspinal neurofibroma. A small plexiform neurofibroma, which was not grossly visible, was confined to the uterine cervix and coexisted with a uterine leiomyoma and adenomyosis. There were no neurofibromas in the myometrium, fallopian tubes, or ovaries. Plexiform neurofibroma is a neoplasm that should be considered in the differential diagnosis of spindle cell neoplasms of the uterine cervix, especially in specimens from patients with neurofibromatosis.

Introduction: Plexiform neurofibromas are benign tumours of the peripheral nerves and connective tissue. They develop most often in patients with neurofibromatosis type 1 (NF1) and often grow continuously. Removal of plexiform neurofibromas is usually unsatisfactory because the network-like growth of these tumours often involves multiple nerve fascicles and other adjacent tissues. It has been previously shown that magnetic resonance tomography can distinguish the growth patterns of plexiform neurofibromas into three different categories: superficial, displacing and invasive., Patients and Methods: Three cases are described with successful subtotal resections of superficial plexiform neurofibromas, and one case with total resection following the diagnosis of tumour subtype using magnetic resonance imaging (MRI)., Results: There was a significant, lasting improvement in appearance which demonstrates that surgical intervention in the case of superficial plexiform neurofibroma is valuable., Conclusion: Careful classification of plexiform neurofibroma by means of MRI provides valuable information for the surgical management of patients. It enables the distinction to be drawn between this subtype and the other two subtypes of plexiform neurofibromas.

Background: Plexiform neurofibromas are rarely found in the cauda equina. The most recent report of a plexiform neurofibroma of the cauda equina noted only 2 previously described cases., Case Description: To these we add the current case, as well as 2 additional previously published cases. We report the case of a 44-year-old man with a sudden exacerbation of his long-standing lower-back and bilateral leg pain. An intradural lesion was seen on magnetic resonance imaging and he underwent surgery. Intraoperatively, there were swollen nerve roots and tumor insinuating itself between the roots. A biopsy was performed, and pathology findings were consistent with plexiform neurofibroma., Conclusions: Plexiform neurofibroma of the cauda equina is a rare tumor, with variable manifestations. These tumors are not amenable to complete resection. Surgical treatment consists of either partial resection or biopsy, possibly with dural grafting for decompression.

We report a successful application of Oberlin's procedure combined with transfer of trapezius and latissimus dorsi with the teres major for reconstruction of elbow flexion as well as abduction and external rotation of the shoulder to a ten-year-old patient, who had a long defect of the left brachial plexus upper trunk caused by resection of the plexiform neuroma.

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.

Multiple plexiform schwannomas (PS) or neurilemmomas are very rare nerve sheath tumors, showing multiple schwannomas of the skin and other soft tissues, brain, or spinal cord with histopathologic characteristic of interwoven Antonio-A type tissues(1) but without other signs of neurofibromatosis type 2 (NF2). Neurofibromatosis type 2 is much less common than neurofibromatosis type 1 (NF1), with a symptomatic prevalence of 1 in 210,000,(2) and, to the authors' knowledge, only six cases of NF2 with multiple plexiform schwannomas have been reported in the literature.(3-5) We report a rare case of multiple cutaneous plexiform schwannomas associated with characteristic features of NF2 including bilateral acoustic neurilemomas and intracranial meningioma.

A 10-year-old boy presented with a mass on the left side of his face for many years, left ear deafness, and limited vision in the left eye. Enhanced CT of the face and neck showed a multilobulated low attenuation mass in the left parotid space, with nodularity and involvement of branches of the left facial nerve. There was a mass in the left orbital apex that extended into the left cavernous sinus, exopthalmos of the left eye, and erosion of the medial portion of the greater wing of the left sphenoid. Partial removal of the mass in the left parotid space was performed. Histopathological examination revealed plexiform neurofibromas. CT and MR imaging findings in neurofibromatosis type 1 patients with craniofacial abnormalities are discussed.

Plexiform neurofibroma often causes significant pain, deformity, and functional problems in the affected part of the body. Surgical management of the condition can be challenging, and local recurrence is common because of inadequate resection of the tumor. One patient with a large recurrent plexiform neurofibroma of the foot and ankle was treated successfully with a complete resection of the tumor, followed by a free latissimus dorsi muscle transfer with a skin graft for soft-tissue reconstruction. The patient has regained good contour of the foot and ankle, and has been symptom-free with no evidence of local recurrence in the affected area for 18 months. The authors believe that thorough preoperative planning and state-of-the-art reconstructions, as described here, are the keys to their success., (Copyright 2003 Wiley-Liss, Inc.)

Solitary plexiform neurofibromas of the intraparotid facial nerve are extremely rare tumours that can present a diagnostic challenge. Knowledge of their existence is key to its proper management. Only two cases have been reported in the English literature. We describe the first paediatric case of an intraparotid facial nerve solitary plexiform neurofibroma.

The commonest skull manifestations in neurofibromatosis involve the orbit, with very few reports about occipital defects. We report a rare case of a 54-year-old lady with a massive plexiform neurofibroma extending from the auricular region down her left neck and into her shoulder, with an associated large left occipital and left petrous bone defect and extensive cerebellar meningo-encephalocoele, which presented with a relatively asymptomatic cervical mass and was treated with resection of the neurofibroma and advancement and rotational skin flaps.

Background: Neurofibromatosis type 1 (NF1) is a common autosomal dominant disorder in humans. The hallmark of NF1 is development of neural tumors. Plexiform neurofibromas are a major source of morbidity associated with NF1. Surgical removal of these particular tumors is associated with a high risk of damage of surrounding vital structures as well as a risk of significant hemorrhage. Surgical debulking of tumors also sometimes leads to extensive scarring, which may be very disfiguring., Objective and Methods: We report a novel approach to the surgical therapy of plexiform neurofibromas using liposuction in two patients., Results: Debulking of plexiform neurofibromas with liposuction was successful in both patients. Postoperative follow-up 6 months later showed no evidence of tumor regrowth in either case., Conclusion: Liposuction may be a preferable surgical method for debulking of superficial plexiform neurofibromas in patients with NF1. This method is less invasive than conventional surgical tumor debulking.