case_images: PMC5982496_01
case_images: Axial post-contrast T1-weighted MRI showing homogeneously enhancing soft tissue along the course of right trochlear nerve entering the superior orbital fissure. Multiple homogeneously enhancing plexiform, soft-tissue masses are seen in bilateral cavernous sinus region obscuring the course of 3rd, 4th, 5th, and 6th cranial nerves
case_images: JPN-13-74-g001.jpg
case_images: PMC5982496_01_JPN-13-74-g001.jpg
case_images: F1
case_images: It exhibited enhancement of its solid mural nodular component, which was compressing on brain stem, craniovertebral, and cervicomedullary junction [Figure 1].
case_images: (A) Axial post-contrast T1-weighted MRI image showing homogeneously enhancing soft-tissue mass in bilateral cerebellopontine angle extending into internal auditory canal along the course of 7th and 8th cranial nerve complex giving a bilateral "ice-cream cone" appearance suggestive of schwannomas.: (B) Coronal post-contrast T1-weighted image with a heterogeneously enhancing solid cystic mass of the nucleus of lower cranial nerve projecting in extra-axial spaces compressing and distorting left cervicomedullary junction along the course of the 9th cranial nerve toward jugular foramen. In addition, multiple lobulated enhancing masses are seen along the course of exiting lower cranial nerves (10th, 11th, and 12th cranial nerves) in upper cervical foramina. (C) Sagittal T2-weighted image with mass in cervicomedullary region as detailed above. Additionally, intramedullary T2 hyperintensity at C2-C3 level (compatible with ependymoma or astrocytoma) with intradural extramedullary neural lesion at C6 level displacing the cord anteriorly with intramedullary hyperintensity is seen
case_images: JPN-13-74-g002.jpg
case_images: PMC5982496_01_JPN-13-74-g002.jpg
case_images: F2
case_images: Both vestibulocochlear nerves in CP angle regions were involved with intracanalicular extension of lesions [Figure 2A]. Paracavernous region exhibited bilateral involvement of multiple cranial nerves namely 3rd, 4th, and 6th; extra-calvarial retrocerebellar, retro-occipital, and suboccipital regions also exhibited multiple nodular-enhancing lesions of variable sizes representing neurocutaneous nodules with a possibility of plexiform involvement [Figure 2B]. There were multiple enhancing extradural nodular lesions with broad base at C6-C7 and D5-D6 levels, extending through both lateral neural regions and C5-C6 levels, and occupying right lateral aspect of spinal canal at D5-D6 levels. There was perineural involvement of caudal filum [Figure 2C].
case_images: Hematoxylin and eosin stain of the biopsy of the exophytic growth over the trunk, demonstrating picture consistent with neurofibroma
case_images: JPN-13-74-g003.jpg
case_images: PMC5982496_01_JPN-13-74-g003.jpg
case_images: F3
case_images: Biopsy of the cutaneous lesion was consistent with neurofibroma [Figure 3].