The chordoma foundation works to accelerate the development of new treatments for chordoma, while helping chordoma patients get the best care possible. Clivus is also the site for chordoma a rare malignant tumour. Matriz acuosa gelatinosa es remplazada por cartilago mejor pronostico. Chordoma begins in cells that once made up a collection of cells in the developing embryo that go on to become the disks of the spine. Clival ectopic pituitary adenoma mimicking a chordoma.
The mean age at first operation was 51 years range, 10 to 80. The mid clivus extends from the trigeminal nerve down to the exit foramina pars nervosa of the jugular foramen. It most often forms where the skull sits atop the spine skull base or at the bottom of the spine sacrum. The authors report a case of a congenital clivus chordoma that caused cranial nerve palsy and hydrocephalus. Clinical, radiological and followup data of patients who had undergone surgery for clivus tumours at our institution between january 1995 and. Eight of the patients were men and four were women. Specifically, it is formed by the sphenoid body and the basiocciput, which join at the sphenooccipital synchondrosis. At the clivus, the occipital bone has articulations with the petrous part of temporal bone at the petroclival fissure. The clival region is the second most common location, accounting for 3035% of cases 2,3. Here we report on a patient that presented with an incidental clival mass thought to be a chordoma. Purely ectopic pituitary adenomas are exceedingly rare. Primary intraosseous hemangiomas are benign, vascular malformations that account for approximately 1% of all primary bone neoplasms.
These tumors are mostly found in vertebral bodies and are rarely seen in the calvarium, where they represent 0. Chordoma definition of chordoma by medical dictionary. The clivus is an important landmark for checking for anatomical atlantooccipital alignment. The lower clivus is the area below the ninth, tenth, and eleventh nerves and includes the jugular tubercle, occipital condyles, the foramen magnum, and the hypoglossal canals.
The clivus of blumenbach is the sloping midline surface of the skull base anterior to the foramen magnum and posterior to the dorsum sellae 1. Primary intraosseous cavernous hemangioma of the clivus. Chordoma is a rare type of bone cancer that happens most often in the bones of the spine or the skull. Endonasal resection, tumor pathology, and endocrinology workup revealed a prolactinoma. Pdf adenoma invasor en seno esfenoidal y clivus hector. Cordoma genetic and rare diseases information center. The evidence for this is the location of the tumors along the neuraxis, the similar immunohistochemical staining patterns, and the demonstration that notochordal cells are preferentially left behind in the clivus and sacrococcygeal regions when the remainder of the notochord regresses during. Spontaneous csf rhinorrhea as a presenting symptom of a. Tumours of the clivus are rare and metastases involving this area have been previously described only as single case reports or included in series with other skull base tumours. Clivus chordomas are more common inmales and occur most ofteninthethirtiesandforties. Congenital clivus chordoma eva neumaier probst, friedheim e. Typically the mass projects posteriorly at midline, indenting the pons.