The most frequent skeletal abnormality is scoliosis. Theoretically, differentiation of neurofibroma from neurilemoma should be possible because of their differences in location relative to the normal nerve. Photomicrograph (original magnification, approximately ×20; Bielschowsky silver stain) of an axial section of normal sural nerve. Malignant PNSTs account for 5%–10% of all soft-tissue sarcomas and usually affect adult patients 20–50 years of age with an approximately equal sex distribution (,3,,4). Neural fibrolipoma in a 34-year-old man with macrodactyly. (c, d) Axial T1-weighted (500/20) MR images of the wrist (c) and phalanges (d) reveal adipose tissue causing thickened nerve, surrounding fascicles (arrowheads) both proximally (median nerve) and distally (interdigital nerve branches), and overgrowth of the second and third digits with predominance of fat (arrow). Figure 11b. 3, 4 May 2013 | Skeletal Radiology, Vol. 5, Physical Medicine and Rehabilitation Clinics of North America, Vol. Figure 6f. NF1 and neurofibromatosis type 2 (NF2) account for 99% of cases. 44 chromosomes dits autosomes, identiques deux à deux et 2 chromosomes sexuels (XX pour les femmes et XY pour les hommes). 2, Current Medical Imaging Formerly Current Medical Imaging Reviews, Vol. La neurofibromatose (NF) est une maladie génétique qui touche le système nerveux. The MR imaging appearance is pathognomonic, consisting of longitudinally oriented cylindric foci (about 3 mm in diameter) of low signal intensity surrounded by fatty signal intensity representing nerve fascicles (,,,,,,,,,Fig 6c, ,,,,,,,,,6d) (,50,,52,,53). The proximal neuroma (terminal type) has an entering tubular tibial nerve (arrowhead) ending in a bulbous expansion (*). 4, 1 March 2005 | Radiology, Vol. 4, Current Problems in Diagnostic Radiology, Vol. The proximal neuroma (terminal type) has an entering tubular tibial nerve (arrowhead) ending in a bulbous expansion (*). En effet, les neurofibromes apparaissent plus tard au moment de la pré-adolescence. 45, No. Nerve sheath ganglion in a 25-year-old man with peroneal nerve distribution symptoms. (d, e) Intraoperative photographs of the distal thigh initially (d) show the bag-of-worm (arrows) appearance of serpentine plexiform neurofibromas. Subsequent incision (e) of the distal sciatic nerve epineurium (arrowhead) reveals markedly thickened nerve branches (*).
(c, d) Axial T1-weighted (500/20) MR images of the wrist (c) and phalanges (d) reveal adipose tissue causing thickened nerve, surrounding fascicles (arrowheads) both proximally (median nerve) and distally (interdigital nerve branches), and overgrowth of the second and third digits with predominance of fat (arrow). Viewer. A large amount of intermetatarsal bursal fluid (>3 mm in transverse diameter) or fluid in the fourth intermetatarsal space should suggest an associated Morton neuroma (,36). *Two or more of these criteria are required for diagnosis.

(g, h) Photograph of a coronally sectioned whole-mount specimen (hematoxylin-eosin stain) (g) of the second digit and photomicrograph (h) (original magnification, ×200; hematoxylin-eosin stain) demonstrate osseous and soft-tissue hypertrophy with predominance of fat (*) surrounding nerve fascicles (arrow).Download as PowerPointOpen in Image 133, No. Il existe différentes formes de neurofibromatoses. 2, European Annals of Otorhinolaryngology, Head and Neck Diseases, Vol. A Morton neuroma appears pathologically as fusiform enlargement of the plantar digital nerve at its bifurcation, with thickening of the epineural fascicles, perineural fibrosis with high collagen content (Renaut bodies), and loss of the myelinated fibers (,36–,38). (a) Chest radiograph shows multilobulated extrapleural masses (arrowheads) and scalloping of all ribs with a ribbonlike appearance.

Neural fibrolipoma in a 34-year-old man with macrodactyly. 8, American Journal of Roentgenology, Vol. Surgical resection of the neuroma and involved nerve segment is the most successful treatment. Chez l’adulte, le diagnostic est habituellement assez facile à faire sur les manifestations cliniques. Figure 15a. Traumatic neuromas have been divided into two major categories based on the anatomic location of the tangled, multidirectional, regenerating axonal mass with respect to the proximal nerve end (,2,,18). Spindle neuromas are internal, focal, fusiform swellings secondary to chronic friction or irritation to a nondisrupted, injured but intact nerve trunk. Calcification (osteoid, chondroid, or amorphous) is uncommon and mild in extent when present (,70,,80). 3, Diagnostic and Interventional Imaging, Vol. A characteristic at angiography that should suggest a neurogenic neoplasm is the identification of corkscrew-type vessels at the upper or lower poles of the tumor, which represent hypertrophy of nutrient nerve vasculature (,,,Fig 9b) (,81,,82). (a, b) Photograph (a) and anteroposterior radiograph (b) of the hand show soft-tissue and osseous enlargement (in length and width) of the second and third digits with ulnar deviation and secondary degenerative changes (arrowhead in b). Viewer. Elle concerne une naissance sur 3 500 dans le monde et est la plus fréquente de ce type de maladie. (a, b) Forearm radiograph (a) and photograph of the amputation specimen (b) show massive enlargement of the upper extremity with dysplastic changes of the radius and ulna, including dislocation (arrowhead). The diagnosis of neurogenic tumors can be suggested from their imaging appearances, including lesion shape and intrinsic imaging characteristics. Figure 16d. Les enfants atteints de la maladie rencontrent des troubles de l'apprentissage notamment à cause des problèmes oculaires. 87, No. (a, b) Photograph (a) and anteroposterior radiograph (b) of the hand show soft-tissue and osseous enlargement (in length and width) of the second and third digits with ulnar deviation and secondary degenerative changes (arrowhead in b). 51, No. Tibial nerve neurilemoma in a 29-year-old man. Figure 9b. However, lesions demonstrating the target sign typically enhance more prominently centrally. 94, No. Il peut être plus difficile à établir chez l’enfant de moins de 5 ans qui peut n’avoir que des tâches café au lait. Ces taches sont très fréquentes dans la population saine (plus de 10 % de la population en possède une ou deux), mais dans le cas de la NF-1 leur nombre est de 6 au minimum, certains malades en ayant parfois plusieurs dizaines. Plexiform neurofibromas about the elbow and distal thigh in a 21-year-old woman with NF1. 3, 28 November 2013 | American Journal of Neuroradiology, Vol. Figure 14a.

1, Journal of Clinical Neuroscience, Vol. La dernière modification de cette page a été faite le 14 juin 2020 à 09:58. 207, No. A firm soft-tissue mass at a focal pressure site may be apparent. 12, Seminars in Roentgenology, Vol. Chacune des méthodes disponibles actuellement ne permet de détecter qu’un certain pourcentage d’anomalies, ce qui oblige les laboratoires à coupler plusieurs techniques pour accroître le taux de détection. (b, c) Short axis fat-suppressed contrast material-enhanced T1-weighted (700/15) MR (b) and power Doppler US (c) images show marked enhancement and increased vascularity of the lesion (*). CHU Henri Mondor. 177, No. 3, 29 January 2018 | Aesthetic Plastic Surgery, Vol. Figure 19d. Males and females are equally affected, and there is no familial predisposition (,48). 34, No. suppl_1, Seminars in Ultrasound, CT and MRI, Vol. Nodular area represents intact sciatic nerve (open arrows). 40, No. 52, No. The histologic hallmark of neurilemoma is identification of Antoni A and Antoni B regions (,,,,,Fig 11). (a, b) Photograph (a) and anteroposterior radiograph (b) of the hand show soft-tissue and osseous enlargement (in length and width) of the second and third digits with ulnar deviation and secondary degenerative changes (arrowhead in b). The degree of increased vascularity is variable and more prominent in malignant PNSTs (,81–,85). (b, c) Intraoperative photographs initially (b) reveal the lesion (solid black arrow) and entering nerve (white arrow) both within the epineurium. In one study, MR imaging had an accuracy of 90%, positive predictive value of 100%, and negative predictive value of 60% in identification of Morton neuroma (,37). (a) Axial CT scan shows a low-attenuation well-defined mass (arrow) with a center of slightly increased attenuation (white arrowheads) and incomplete fat rim (black arrowheads) (target sign). (a) Coronal T1-weighted (500/15) MR image shows a paraspinal mass (*) with entering nerve in the neural foramen (arrowhead). 0000040878 00000 n

Spinal neurofibroma in a 31-year-old woman. (a, b) Coronal T1-weighted (634/18) (a) and inversion recovery (3,000/60; inversion time, 160 msec) (b) MR images show a thickened sciatic nerve entering and exiting (straight solid arrows in a) a focal heterogeneous mass with a rim of fat (split-fat sign) (curved arrow).

40, No. Figure 4c. Skeletal abnormalities are common in NF1 (,,,Fig 15), occurring in approximately 25%–40% of cases, again reflecting the multiorgan effects of mesodermal dysplasia (,9,,70). (b) Intraoperative photograph reveals the paraspinal mass (arrow) and entering nerve (arrowhead). This finding can be quite subtle in muscle supplied by the affected nerve, may require comparison with the normal side, and is best seen on T1-weighted MR images. Malignant tumors, Differentiation of soft tissue benign and malignant peripheral nerve sheath tumors with magnetic resonance imaging, A possible cause of multiple intramuscular masses: Mazabraud’s syndrome (2007: 11b), Differentiation Between Schwannomas and Neurofibromas in the Extremities and Superficial Body, Primary Paraspinal Malignant Peripheral Nerve Sheath Tumor, Fibrolipomatous Hamartoma of the Median Nerve, Usefulness of muscle denervation as an MRI sign of peripheral nerve pathology, High-resolution ultrasound of peripheral neurogenic tumors, Sacral Nerve Sheath Tumor Simulates a Pedunculated Fibroid on Sonography, Multicentric malignant peripheral nerve sheath tumor, MRI appearances of common benign soft-tissue tumours, Prevalence and diagnostic significance of fluid–fluid levels in soft-tissue neoplasms, Treatment of Morton's Neuroma with Alcohol Injection Under Sonographic Guidance: Follow-Up of 101 Cases, Nerve sheath tumor surgery: case-guided discussion of ambiguous findings, appropriateness of removal, repeated surgery, and nerve repairs, Échographie des nerfs des membres inférieurs, Intramuscular Neurilemoma of the Lower Limb, An extrarenal rhabdoid tumor of the cervical spine with bony involvement, Soft-tissue masses in the shoulder girdle: an imaging perspective, Epithelioid sarcoma of the median nerve mimicking a peripheral nerve sheath tumour, A giant malignant peripheral nerve sheath tumour of the breast: CT and pathological findings, Soft-tissue tumors update: MR imaging features according to the WHO classification, MRI of soft tissue masses of the hand and wrist, Traumatic Neuroma around the Celiac Trunk after Gastrectomy Mimicking a Nodal Metastasis: A Case Report, Peripheral Neuropathies of the Median, Radial, and Ulnar Nerves: MR Imaging Features1, Imaging and diagnostic strategy of soft tissue tumors in children, MRI Findings of Subcutaneous Epidermal Cysts: Emphasis on the Presence of Rupture, Unexpected diagnosis of superficial neurofibroma in a lesion with imaging features of a vascular malformation, Foot and Ankle Disorders: Radiographic Signs, The soft-tissue manifestations of neurofibromatosis type 1, Abdominal Neoplasms in Patients with Neurofibromatosis Type 1: Radiologic-Pathologic Correlation1, Tumours and tumour-like lesions in the foot and ankle, Tumors and Tumor-Like Lesions of Soft Tissues, Estudo retrospectivo do tratamento cirúrgico do neuroma de morton por via plantar, Imaging of peripheral nerve sheath tumors with pathologic correlation, Best Cases from the AFIPPlexiform Neurofibroma of the Bladder1, Traumatic neuroma: unusual benign lesion occurring in the mastectomy scar, From the Archives of the AFIPBenign Musculoskeletal Lipomatous Lesions1, Extraaxial Neurofibromas Versus Neurilemmomas: Discrimination with MRI, Foci of decreased signal on T2-weighted MR images in leiomyosarcomas of soft tissue: correlation between MR and histological findings, Diagnosis of Peripheral Nerve Sheath Tumors around the Pelvis, MR imaging of tumors and tumor-like lesions of the upper extremity, Neuroimaging findings in neurofibromatosis type 1 and 2, Sonographic Characteristics of Peripheral Nerve Sheath Tumors, Peripheral Schwannoma Lacking Enhancement on MRI, Thallium-201 single-photon emission computed tomography in the detection of retroperitoneal schwannoma, Peripheral Nerve Lesions: Role of High-Resolution US1, Additional Value of Magnetic Resonance with Spin Echo T1-Weighted Imaging with Fat Suppression in Characterization of Soft Tissue Tumors, Imaging of Soft-Tissue Myxoma with Emphasis on CT and MR and Comparison of Radiologic and Pathologic Findings1, Soft tissue tumors of the lower extremities, Pedal plexiform neurofibroma: Review of the literature and case report, Síndrome del túnel carpiano por hamartoma fibrolipomatoso del nervio mediano, IRM et névromes des moignons dˈamputation des membres inférieurs, MR Imaging of Soft-Tissue Masses of the Extraperitoneal Spaces1, Magnetic Resonance Imaging of the Peripheral Nervous System, Hamartoma fibrolipomatoso del nervio mediano: descripción de un caso y revision de la bibliografía, Cross-Sectional Imaging of Peripheral Nerve Sheath Tumors, US of Nerve Entrapments in Osteofibrous Tunnels of the Upper and Lower Limbs1.
Localized neurofibromas in NF1 often affect the dermis and subcutaneous tissue and are referred to as fibroma molluscum when pedunculated (,2,,9,,70). (d-f) Intraoperative photographs of lesion resection initially (d) reveal the fusiform mass (M) with entering and exiting peroneal nerve (*). (a-c) Coronal T1-weighted (435/40) (a) and axial T2-weighted (2,000/80) (b) MR images and longitudinal US scan (c) (photographically spliced together to show both ends of the lesion) reveal a well-defined fusiform mass (M) with entering and exiting nerve (*). Growth of neurofibromas is usually slow; however, more rapid episodes of growth can be associated with pregnancy, puberty, or malignant transformation. 1, Journal of the American Academy of Orthopaedic Surgeons, Vol. Elle peut-être cancéreuse ou non, on la qualifie alors de bénigne. US and MR imaging are superior to CT for identifying Morton neuroma (,40). 0000011568 00000 n 111 0 obj <> endobj 2Current address: Charleston Radiologists, North Charleston, SC. Viewer. 38, No. (b) Photograph of the sectioned gross specimen reveals fascicular morphology (*) of the malignant PNST, corresponding to the MR imaging appearance. Figure 12b. CT scan shows bilateral paraspinal masses (*) with nerves entering from the neural foramina (arrowheads). Patients typically present before 30 years of age, often at birth or early childhood, with a soft, slowly enlarging mass in the volar aspect of the hand, wrist, or forearm (,47). Cet article porte sur la neurofibromatose de type I qui n'a aucun rapport, clinique ou génétique, avec la, Tache cutanée « Café au lait » caractéristique de la, Le gène NF1 est un des gènes dont le taux de, A genetic study of von Recklinghausen neurofibromatosis in south east Wales, prevalence, fitness, mutation rate, and effect of parental transmission on severity, Identification of the neurofibromatosis type 1 gene product. Viewer. Viewer.