Chordoma Research Paper Outline

Chordomas are considered rare neoplasms with an incidence of 0.08/100 000.(1) chordomas arise from remnant notochord cells found mainly within the clivus and sacrococcygeal regions . But also, can be found at any site along the vertebral column (2). The presentation of clival chordomas tend to be late, associated neurological impairment due to involvement of the lower cranial nerves (3). Clival chordomas are also challenging to treat due to their site , anterior to the brainstem, and their aggressive locally invasive nature (4,5) and they are radioresistant lesions . clival chordomas have relatively poor survival of 0.9 years without treatment (6). Extended open skull base approaches to the clival region are traditionally the mainstay for achieving gross total resection in chordoma. Extended subfrontal transbasal, anterior transfacial, and lateral transtemporal or far-lateral approaches have been described, as well as staged surgical approaches. (7-9) . These approaches to the anterior midline skull base often require extensive removal of skull base bone and brain retraction, and they put critical neurovascular structures between the operating surgeon and the pathology. With the advent and incorporation of the rigid endoscope into neurosurgical practice and the active collaboration between ENT surgeons and neurosurgeons, extended endonasal endoscopic approaches have become well-accepted, minimally invasive routes to the midline and paramedian skull base. The endoscopic

The aim of the practical is to investigate the nature of the chromosomal changes or rearrangements in this cancer using FLPTer.

     Recent studies show that this syndrome may be associated with changing craniofacial and skeletal muscle metabolism, such as blood flow, which causes the chronic fatigue and severe weakness. Another hypothesis is that an infectious trauma to the body, such as a virus, triggers the illness. However, with this syndrome being new, no specific virus has been identified. There is though a plot study that shows that there is possible inherited tendency toward the disease. This disease/syndrome has increased

It is an autoimmune condition. It is also inherited and also can be associated with medical conditions.

As it is cited in the references there are review articles about the subject of this manuscript. Authors should explain what is their case report adds to the current literature.

A chordoma is a rare type of bone tumor that grows along the spine or skull base. Since it originates from stems notochord cell lines, chordomas are normally present in the midline on the axial skeleton and become very difficult to detect during the early stages of life. Generally, 50% of cases occur in the sacrococcygeal region, 35% in Spenoccipital region and 15% in the rest of the column, especially in the 2nd cervical vertebra transverse process level and sinuses. Chordomas represent less than 1% of all tumors of the central nervous system and between 2% and 4% of all primary bone tumors. Each patient has a unique kind of chordoma, consequently, different treatment approaches may be appropriate for different patients. The initial manifestations

Intradiploic epidermoid cysts, derived from the ectodermal cells of the cranium, are lined by stratified squamous epithelium. They account for 1% of all intracranial tumors [1]. Although rare, their common locations include the frontal, parietal and occipital bones. These cysts grow very slowly. They usually present as painless bony swelling under the scalp. Headaches may occur due to erosion of the calvarium, and seizures due to local pressure. They may perforate the dura, rupture into the subarachnoid space or involve the brain parenchyma. Microscopically the wall of the cystic tumor is lined by squamous epithelium and the cyst is filled with lamellated keratin content.

Cell replication and division is vital for growth and development. The mechanisms associated with replication of DNA are very precise. However, there can be errors at times and when these errors occur there could be problems in reproduction process. Some of these errors can cause uncontrolled cell growth causing tumor formation. There are genes within the human genome that prevent this uncontrolled growth from occurring. These genes are known as tumor suppression genes. They work in a very specific way and are vital for proper growth. Though they fix errors, the tumor suppression genes can malfunction or be malformed at times as well. When these errors occur, there can be serious consequences.

address whether the disease is inherited by the parents, or if it is a chromosomal error. Finally,

Chordomas usually cause extensive bone destruction of the skull base, best analyzed on CT. CT will also show frequent intratumoral sequestered bone fragments. MRI typically shows a destructive invasive soft tissue mass arising from the clivus. At an early stage, bone expansion indicates that the tumor originates from bone and not adjacent structures. The extension of chordomas is primarily posterior, with involvement of the pontine cistern and, occasionally, of the premedullary cistern. On MRI, posterior extension may show as a thumb indenting the pons and/or of the medulla (fig 37-1). Extension can be upward to the sella turcica, displacing the pituitary gland, downward to the nasopharynx or laterally to the middle cranial fossa. Most chordomas demonstrate T1 signal hypointensity and T2 signal

| * located on chromosome 17p13.1, is the single most common target for genetic alteration in human tumors * tumor suppression protein that is dysfunctional in many types of cancer. One highly studied function of pRb is to prevent excessive cell growth by inhibiting cell cycle progression until a cell is ready to divide. It is also a recruiter of several chromatin remodelling enzymes such as methylases and acetylases.

These cells continuously grow and multiply as healthy cells die. This causes a mass cells to form a tumor. In most cases it not clear what causes the mutation, but it is possible to inherit a mutation from a parent. It is possible for mutated genes to be passed from parents to offspring’s which can cause retinoblastoma in children. Retinoblastoma is an autosomal dominant pattern, which means that if a parent has one copy of the dominant trait then there is a greater risk that their offspring will receive the mutated gene. If both parents are carriers have one of the mutated genes then there is a twenty five percent chance that the offspring will have both copies of the mutated gene. Just because someone has both mutated genes doesn’t mean that that person will have retinoblastoma, it just means there is a greater risk of it occurring. If a child has the inherited form of the gene, they typically develop retinoblastoma earlier, and tends to occur in both eyes rather than in one eye. There are some complications that can occur when you treating people with retinoblastoma. When you are treated for retinoblastoma there is a risk that the cancer can return to the treated eye or around it. Due to this, the doctor will schedule follow up appointments to check for the cancer reappearing. “In most cases, this will likely involve eye exams every few months for the first few years

There was a study of this disease that had patients tell their side of having the disease and how some had to stay home from work and how bad it changed some of their lives. The study is really neat to hear about and even probably watch. They would use the fingertips, fingers, palms, back of hands, and wrist to get the results for the study. They use a scale on every part they used to see how much skin or even no skin was

CASE DISCUSSION:Desmoplastic small round cell tumour (DSRCT) belongs to the family of ‘‘small round blue cell tumours’’, which include DSRCT,Wilms’ tumour, rhabdomyosarcoma, , primitive neuroectodermal tumour, ,neuroblastoma, Ewing sarcoma ,small cell osteosarcoma, poorly differentiated synovial cell sarcoma and lymphomas which are characterised by sheets of small cells round nuclei. The primary tumour typically arises in the peritoneal cavity of the pelvis, although DSRCT arising from brain, lung, pleura, stomach, pancreas, ovary, paranasal sinuses and scrotum has been described.[5]It is a very aggressive neoplasm with mean age of survival of 23 months.[6] CT frequently shows multiple bulky, lobulated, heterogeneous,and peritoneal soft-tissue masses with a predilection for spread to distant organs without obvious primary organinvolvement. On histology these tumors show sharply demarcated,angulated, cohesive nests of uniform tumor cells with round to oval nuclei, inconspicuous nucleoli, and scant cytoplasm; surrounded by abundant fibrous stroma. DSRCT immunohistochemistry shows

causes many issues and symptoms to certain individuals, the disease can be serious and deadly.