Female, 2 years old, with neurofibromatosis type I and plexiform neurofibroma of the jaw and face. Evaluate cavernous sinus and extent of jaw tumor. Also with large paraspinal and shoulder tumor increasing in size. Assess extent and boundaries of tumor. Brain:A small ill-defined T2 hyperintense lesion is evident within the left cerebral peduncle. There may be one or two similar small lesions along the left aspect of the fourth ventricle within the middle cerebellar peduncle.The left anterior hippocampus and amygdala are slightly larger and T2 hyperintense relative to the right.No other evidence of brain parenchymal edema is seen. No findings to suggest acute intracranial hemorrhage are demonstrated. The ventricles are normal in size and morphology.Although this examination is not specifically protocoled for assessment of the orbits and optic pathway, no definite lesions of the optic apparatus are seen.The CSF space anterior to the left temporal lobe is expanded. Along the medial aspect of this space, evidence is seen of a wispy T2 hyperintense, FLAIR hyperintense, mildly diffusion restricting lesion which demonstrates fairly robust but ill-defined and infiltrative enhancement. Face:An extensive, infiltrative plexiform neurofibroma is present centered on the right pterygopalatine fossa which is markedly expanded.Laterally, this lesion extends through the pterygomaxillary fissure to invade to the masticator space. A small tumor component even extrudes further laterally, anterior to the mandibular angle, into the right cheek subcutaneous space.Medially, the tumor traverses the sphenopalatine foramen to invade the right nasal cavity and the nasopharyngeal mucosal space. Tumor appears to extend into the Eustachian tube. As a result, obstructed secretions are evident within the right mastoid air cells and middle ear cavity.Superiorly, tumor invades the inferior aspect of the right orbit coming in contact with and mildly displacing the inferior and lateral recti muscles. No definite intraconal extension is seen and no obvious optic nerve compromise is demonstrated.Posteriorly, tumor invades and expands the right cavernous sinus but with preservation of the ICA flow void. Tumor is in fact evident tracking along the right ICA, through the cavernous and petrous segments, and down into the cervical region. From the cavernous sinus, tumor infiltrates along the V3 segment of the fifth cranial nerve through a widened foramen ovale and down into the masticator space. Tumor extends through the foramen rotundum, and posteriorly along the floor of the middle cranial fossa tracking along the anterior margin of the petrous temporal bone.The right sphenoid wing may be mildly dysplastic or simply chronically deformed from the extensive tumor.Neck and spine:Extensive infiltrative plexiform neurofibroma is evident involving the paraspinal tissues of the left neck, the left supraclavicular fossa, axilla, upper back, and significantly within the left upper thoracic cavity. Tumor is partly visualized extending distally into the left arm as well.Beginning at the C5-6 level and continuing down to the T5-6 level, tumor invades the left-sided neural foramina. At C7, substantial intracanalicular/intradural extension is seen resulting in rightward displacement of the spinal cord but without evidence of cord edema. Minimal intracanalicular extension is seen at T4 and T5 without significant canal narrowing or cord displacement.Bulky tumor extends through the widened thoracic inlet to completely fill the upper left thoracic cavity. The mediastinal structures are displaced towards the right but the airway seems to remain patent. Posterior lung atelectasis is seen bilaterally along with a small left pleural effusion.More inferiorly in the spine, no large lesions are suspected. Small foraminal tumors are present within the right T7-8 foramen extending along the T7 rib, and perhaps within the left L2-3 foramen.Angiographic imaging:Common origin of the brachiocephalic and left common carotid arteries is seen. The aorta and the origins of the great vessels are patent.The left common carotid artery drapes along the anterior medial aspect of the tumor without evidence of encasement or significant narrowing.The proximal left subclavian artery passes through and is encased by tumor though it does remain patent throughout. The origins of the major proximal branches of the subclavian, including the vertebral artery, thyrocervical and costocervical trunks, are encased by tumor. The vertebral artery remains encased by tumor until it enters the transverse foramen at approximately the C5 level, but the vertebral artery remains patent throughout.On delayed venous phase imaging, the left internal jugular vein tapers and then fails to be visualized as it approaches the large thoracic cavity mass. An enlarged left external jugular vein is noted which anastomoses across midline with the right-sided jugular system. Distal to the mass, the left subclavian vein opacifies, but in the vicinity of the mass, it does not. The subclavian system seems to drain via a network of chest wall and shoulder collaterals.
An extensive, infiltrative neurofibroma is evident involving the deep spaces of the right face. Tumor invades the inferior extraconal orbit, the masticator space and cheek, the left nasal cavity and nasopharyngeal mucosal space. Tumor invades the right Eustachian tube with obstruction of the mastoid air cells and middle ear cavity.This tumor, centered within the pterygopalatine fossa, extensively invades the right cavernous sinus tracking along V3 through the foramen ovale, through the foramen rotundum, and along the floor of the middle cranial fossa following the petrous apex. Tumor also tracks down along the right ICA through the skull base and into the upper neck.A wispy, ill-defined enhancing lesion is evident within the left middle cranial fossa where the CSF space is expanded. This lesion may represent an additional neurofibroma, though the imaging characteristics, and particularly the presence of restricted diffusion, differ significantly from those of the right-sided tumor. This may therefore represent another lesion, perhaps a slightly atypical epidermoid cyst.Multiple additional sequelae of neurofibromatosis are demonstrated intracranially including scattered areas of nonspecific white matter signal abnormality, as well as thickening and T2 hyperintensity in the left mesial temporal lobe. No definite lesions of the optic pathway are seen.Extensive infiltrative plexiform neurofibroma is evident involving the left neck, upper back, supraclavicular fossa and axilla, and extensively the upper left thoracic cavity. Tumor invades numerous left-sided neural foramina in the cervical region with significant intracanalicular extension at C7 resulting in rightward displacement of the spinal cord.The thoracic component of this tumor displaces the mediastinal structures towards the right. The great vessels are however patent throughout. In particular, the left common carotid artery drapes along the anterior medial margin of the tumor without encasement or narrowing. The left subclavian artery passes through and is encased by the tumor but it, along with its major branches including the vertebral artery, remain patent. The vertebral artery is encased by tumor up to the C5 level where it enters the transverse foramen.The left internal jugular vein is patent until it reaches the thoracic tumor at which point it narrows and fails to be visualized. A large left external jugular vein is likely compensating and drains across midline via collaterals to the right jugular system. The distal left subclavian vein is patent, but at the level of the tumor, it is not. Subclavian drainage is likely via chest wall and shoulder collaterals.