Cordoma de clivus pdf

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. Typically the mass projects posteriorly at midline, indenting the pons. Matriz acuosa gelatinosa es remplazada por cartilago mejor pronostico. Clivus is also the site for chordoma a rare malignant tumour. The clivus is an important landmark for checking for anatomical atlantooccipital alignment. Primary intraosseous cavernous hemangioma of the clivus.

Chordoma definition of chordoma by medical dictionary. Eight of the patients were men and four were women. It most often forms where the skull sits atop the spine skull base or at the bottom of the spine sacrum. At the clivus, the occipital bone has articulations with the petrous part of temporal bone at the petroclival fissure. 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. Spontaneous csf rhinorrhea as a presenting symptom of a. Primary intraosseous hemangiomas are benign, vascular malformations that account for approximately 1% of all primary bone neoplasms. Pdf adenoma invasor en seno esfenoidal y clivus hector. 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.

The authors report a case of a congenital clivus chordoma that caused cranial nerve palsy and hydrocephalus. Here we report on a patient that presented with an incidental clival mass thought to be a chordoma. Endonasal resection, tumor pathology, and endocrinology workup revealed a prolactinoma. Chordoma is the most common primary malignant sacral tumor 9,10. Cordoma genetic and rare diseases information center. Chordoma is a rare slowgrowing neoplasm thought to arise from cellular remnants of the notochord. Twelve patients with histologically confirmed clivus chordoma were treated at the johns hopkins hospital between 1971 and 1989. Clival ectopic pituitary adenoma mimicking a chordoma. Specifically, it is formed by the sphenoid body and the basiocciput, which join at the sphenooccipital synchondrosis. The mid clivus extends from the trigeminal nerve down to the exit foramina pars nervosa of the jugular foramen.

These tumors are mostly found in vertebral bodies and are rarely seen in the calvarium, where they represent 0. Clivus chordomas are more common inmales and occur most ofteninthethirtiesandforties. Chordoma is a rare type of bone cancer that happens most often in the bones of the spine or the skull. The chordoma foundation works to accelerate the development of new treatments for chordoma, while helping chordoma patients get the best care possible. Cordoma causas, sintomas, diagnostico e tratamento. The mean age at first operation was 51 years range, 10 to 80. The clival region is the second most common location, accounting for 3035% of cases 2,3.