Plexiform neurofibroma (Hamartoma) of the median nerve: A two-case report.

Plexiform neurofibroma is a benign peripheral nerve-sheath tumor, rarely involving major nerves of the extremities. In the literature, there are no clear treatment strategies for plexiform neurofibroma of major peripheral nerves. Our experience encountered two patients with plexiform neurofibroma of the median nerve, presenting with a palmar mass and symptoms of carpal tunnel compression. Preoperatively, plexiform neurofibroma was diagnosed on MRI and clinical examination. Both patients also experienced significant neurological deterioration, with finger numbness and increased nerve/tumor size. Potential malignant transformation was also considered. For these reasons, resection of the involved area of the nerve and repair were indicated. In both patients, intraoperative pathological diagnosis was plexiform neurofibroma. The 45-year-old male patient refused further surgery after carpal tunnel release, which was performed under axillary block. One year postoperatively, nerve compression symptoms decreased moderately. In the other patient, a 7-year-old boy, a significantly enlarged area of the median nerve was resected, and neurorrhaphy was performed. One year postoperatively, median nerve motor-sensory functions recovered completely. Four years postoperatively, no enlargement of the residual tumor was observed.

Multiple Small Bowel Gastrointestinal Stromal Tumors Associated with Neurofibromatosis Type 1 that Were Not Detected by Endoscopy: A Case Report.

We treated a 39-year-old Japanese man who was admitted for an abdominal mass. He had had neurofibroma-like skin lesions since childhood. Computed tomography and endoscopic ultrasound results were consistent with a tumor in the small intestine. Although the tumor was undetectable by single-balloon endoscopy, the patient's background and imaging results led us to suspect a gastrointestinal stromal tumor (GIST). He also met the diagnostic criteria for neurofibroma type 1 (NF1). We performed a surgical removal of the tumor, and the biopsy results led to a definitive diagnosis of GIST. Small bowel GISTs should be considered in cases of NF1.

Modified facelift in severe plexiform neurofibromatosis associated with venous vascular malformation: Case report.

Neurofibromatosis is an autosomal dominant disorder characterized by tumors of the nervous system and skin. Plexiform neurofibromas are common complications of neurofibromatosis type 1 and can cause large facial deformities. Vascular anomalies are in turn a rare manifestation of neurofibromatosis. We present the case of a 48-year-old female patient with right hemifacial neurofibromatosis associated with venous vascular malformation, previously treated surgically and then with sclerosing agents, determining severe residual facial deformity. Her surgical approach using a modified facelift technique associated with partial tumor debulking and lipofilling seems to be a valid technical alternative for these highly complex cases that require a customized approach after exhaustive preoperative evaluation.

[Management of cervico-cephalic plexiform neurofibromas: About 35 cases]. / Prise en charge des neurofibromes plexiformes cervico-céphaliques : à propos de 35 cas.

OBJECT: Plexiform neurofibroma is a characteristic lesion of Von Recklinghausen's disease. Conservative surgery is the most widely adopted treatment. However, it is very challenging because of its hemorrhagic nature and the infiltrative aspect of the lesions. The aim of this study was to evaluate our management. PATIENTS AND METHOD: A retrospective study over 16 years was realized and during this period 35 patients with neurofibroma with cervico-facial location were included. RESULTS: There were 18 men and 17 women with an average age of 23 years (3-50 years). The familial form was found in 9% of patients. Aesthetic discomfort was noted in all patients and functional impairment only occurred in 10% of patients. The NFP was localized at the hemiface in 11 cases, periorbital in 6 cases, naso-labial in 5 cases, scalp in 4 cases, jugal in 4 cases and cervico-chin in 5 cases. Size of the lesions averaged 11.6cm (4-45cm). Eighteen patients (51.4%) were operated including 10 by modeling resection, 05 cervico-facial lifting and 3 complete resections. Complication rate was 28% dominated by disunion wound. The average number of procedures was 1.6 (1 to 5). After 3 years average follow-up, aesthetic et functional results was assessed as good over 75 per cent of patients. CONCLUSION: Cervico-facial plexiform neurofibromas is challenging. Conservative surgery should be the gold standard and long time follow-up is recommended.

The role of imaging in isolated benign peripheral nerve tumors: A practical review for surgeons.

The diagnosis of nerve tumor(s) must be suspected in all cases of tumefaction or pain on the path of a nerve exacerbated by percussion. Solitary nerve tumors are primarily schwannomas, but other rare tumors may be present such as intraneural ganglion cysts of controversial origin. Preservation of nerve continuity is the underlying goal for any surgical procedure, irrespective of the type of tumor. Therapeutic outcomes are closely linked to tumor resectability; in most patients, the resectability of the tumor, its type and benignity can be predicted based on medical imaging. Comparison with the clinical examination and case-based reasoning is crucial. Consequently, the aim of this review was to examine the role of imaging in isolated benign peripheral nerve tumors, and provide the surgeon with a practical guide for its application in predicting the nature and resectability of nerve tumors.

[Neurogenic mediastinal tumors in adults]. / Tumeurs neurogènes du médiastin de l'adulte.

INTRODUCTION: Neurogenic mediastinal tumors are tumors develop at the expense of peripheral nerve elements of the mediastinum, the sympathetic nervous tissue and paraganglia. The series that we propose has the distinction of a high frequency of malignant forms and a wealth of clinical symptomatology. METHODS: This is a retrospective study of 9 patients collected over a period of 6 years. The epidemiological aspect, clinical, radiological, surgical treatment and his results, histological diagnosis and prognosis were reviewed. RESULTS: Five women and 4 men were identified. All patients were symptomatic at diagnosis. Tumor resection was complete in 8 cases. In 3 patients, there was a malignant tumor. Two of them had received chemo-radiotherapy. The others showed good clinical progress with a mean of 35 months. CONCLUSION: Neurogenic tumors in adults are usually discovered incidentally. When the malignant forms predominate or in the presence of large tumor, the symptoms can be quite marked. In all cases the treatment based on a complete surgical resection remains the only guarantee of a good prognosis.

[Fillet flap for axillary reconstruction]. / Utilisation d'un membre banque pour le comblement d'une perte de substance axillaire.

INTRODUCTION: Among all complications that affect patients with neurofibromatosis, type I (NF1) are very aggressive malignant nerve sheath tumors (MPNSTs). Surgery is their first line therapy. CLINICAL CASE: We report the case of a non metastatic neurofibrosarcoma of the axillary area in a 22-year-old male with NF1. An interscapulothoracic resection was performed to resect the tumor and had sacrificed the homolateral latissimus dorsi pedicled myocutaneous flap. In this extreme and not codified situation, the coverage of the tissue loss was possible in the same time using a free flap harvested on the amputated limb (fillet flap). The free flap based on the humeral pedicle was composed of the entire skin and muscles of the amputated forearm. It was anastomosed on the subclavian vessels. Only a free flap was suitable in this tissue loss. The use of a fillet flap allows a wide and reliable coverage without donor site morbidity.

[Nerve sheath tumours]. / Les tumeurs des gaines des nerfs périphériques.

Peripheral nerve sheath tumors are common neoplasms in daily practice. Diagnosis and classification of most conventional peripheral nerve sheath tumors are relatively straightforward for the experienced observer; but on occasion, they are diagnostically challenging (especially with locally aggressive and malignant tumors). This article aims to provide an update of the data (clinical, histological, immunohistochemistry and genomic) of benign, intermediate and malignant peripheral nerve sheath tumors, thanks to the latest WHO "Classification of Tumors of Soft Tissue and Bone", published in 2013, which includes a new chapter on "Nerve Sheath Tumors". Advances in molecular biology have provided new insights into the nature of the various peripheral nerve sheath tumors, and have begun to suggest novel targeted therapeutic approaches.

[Management of plexiform neurofibroma isolated in childhood: four patients]. / Prise en charge du neurofibrome plexiforme isolé de l'enfant : à propos de quatre observations.

Plexiform neurofibroma is a rare and benign tumor often associated with type 1 neurofibromatosis (NF1) or Von Recklinghausen's disease. Present in one third of cases of NF1, there are isolated forms where the diagnosis should remain a diagnosis of exclusion. We report four cases of isolated plexiform neurofibromas found in children from topographies and we discuss the pretherapeutic assessment, the surgical management and the long-term follow-up.

Doença de Recklinghausen: relato de caso e revisão de literatura / Recklinghausen disease: case report and literature review

JUSTIFICATIVA E OBJETIVOS: A neurofibromatose tipo 1 é uma doença genética autossômica dominante. Apresenta-se distribuída em diversas regiões do mundo, não possui distinção étnica e acomete igualmente ambos os sexos. O objetivo deste estudo foi relatar um caso de neurofibromatose tipo 1, descrevendo o quadro clínico desta doença. RELATO DO CASO: Paciente do sexo masculino, 60 anos, com diagnóstico de neurofibromatose tipo 1 estabelecido havia 22 anos. Fazia acompanhamento para exérese de nódulos. O paciente referiu uma filha com a mesma doença. CONCLUSÃO: É importante que o clínico saiba reconhecer a doença, para que seja estabelecido o diagnóstico precoce tendo em vista o risco da transformação sarcomatosa. O manuseio do paciente com essa afecção deve ser feito por equipe multidisciplinar familiarizada com a história natural da doença,tendo como objetivo proporcionar a melhor qualidade de vida possível aos seus portadores.

BACKGROUND AND OBJECTIVES: The neurofibromatosis type 1 is an autosomal dominant disorder. It is distributedin many regions of the world, without distinction of ethnic affecting both sexes equally. The objective of this study is toreport a case of neurofibromatosis type 1, describing the clinical course of this disease. CASE REPORT: Male patient, 60-year-old, has been diagnosedwith neurofibromatosis type 1 for 22 years. Exeresis has been performed in order to remove the nodules. The patient reported one daughter with the same disease. CONCLUSION: It is important that clinicians learn to recognize the disease for early diagnosis in view of the risk of sarcomatous transformation. The management of patients with this disease should be done by a multidisciplinary team familiarize with the history of the disease, aiming to provide the best possible quality of life for sufferers.