Retinal Tumorigenesis Von Hippel Lindau Disease : A Review

Glioblastoma (pronounced like gleO blastoma) is an incurable brain cancer,Survival rate is usually measured in months.This became a word that was instantly defined, researched, dissected, feared, and tried to comprehend and understand by family,friends and acquaintances of Larry McKee (McKee)who was personally affected from this single word, it was quickly added to their vocabulary, because of the of the events that transpired on October 22, 2011 that forever changed lives “Glioblastomas are tumors that arise from the astrocytes- the star-shaped cells that make up the “glue-like,” or supportive tissue of the brain. These tumors are usually highly malignant (cancerous) because the cells reproduce quickly and they are supported by a large network of blood vessels.”(A.B.T A..) He was diagnosed with stage four.

Tumors can be classified into three types: 1) benign 2) pre malignant 3) malignant tumor. Benign tumors are those which are incapable of abrupt expanding and affecting the other healthy brain tissues. Premalignant tumor is a pre cancerous stage, if not treated properly it may lead to cancers. It is often considered as a disease. Malignant tumor grows rapidly with time an ultimately leads to death of patient. Malignant is a medical term describing a sever growth of a disease. The most common primary brain Tumors are gliomas, wherein 70% are in the group of malignant gliomas, glioblastoma multiform (GBM).The GBM is one of the highest malignant human

An abdominal ultrasound was performed on September 13, 2017 that indicated a small hemangioma in the left hepatic lobe (Ex. B20F).

Ependymoma is a type of brain tumor and while it is rare in adults, accounting for 2-3% of brain tumors, ependymoma is mainly found in children and accounts for around 5% of all childhood brain cancers (American Cancer Society [ACS], 2014, January 31; American Brain Tumor Association [ABTA], n.d.). The survival rate of patients with ependymoma who have survived five years after their tumor is detected is 75% Tumors occur when cells grow to excess and form a mass. While some tumors are benign, meaning that they are harmless, others are malignant, or cancerous. Ependymoma is a malignant cancerous brain tumor (“American Society of Clinical Oncology [ASCO]– Childhood,” n.d.). The tumor appears as soft, grey or red tumors and could comprise of cysts or mineral calcifications.

The diseases associated with FUBP1 include oligodendrogliomas, astrocytomas, and oligoastrocytomas. Oligodendrogliomas are primary glial brain tumors and can be either low-grade (grade II) or high-grade (grade III). [2] Since oligodendrogliomas have a slow growth rate, they are often present for years before they are diagnosed. Nevertheless, the most common symptoms include: seizures, headaches, and personality changes. Altogether, the symptoms vary by location and size of the tumor. About 66 to 78 % of people with grade II oligodendroglioma survive for about 5 years after diagnoses, while 30 to 38 % of people with grade III will survive for about 5 years after they are diagnosed. [3] Astrocytoma is another type of brain cancer that stats in the cerebrum, which is the largest part of the brain, but can also appear in the cerebellum, which is the back of the brain. It is more common in men than in women. Like oligodendrogliomas, the most common symptoms of astrocytomas include: headaches, seizure, changes in behavior and memory loss. [4] Prognosis of astrocytomas depends on the type of astrocytoma. Altogether, a low-grade astrocytoma (LGA) has an 83 % 10-year overall survival, while the overall survival rate of a high-grade astrocytoma (HGA) range between 15-20%. In both oligodendrogliomas and astrocytomas, the FUBP1 locus is mutated which leads to the inactivation of

A tumor in the brain is dangerous if it starts to compress parts of the brain causing neurological symptoms. This action can destroy the brain tissue it surrounds or it can block fluid in the brain from flowing. Although it is not cancerous it can also compress parts of the spinal cord depending where it is in the brain and can also stop the flow of blood in veins surrounding it.

SEGAs originate within the wall of the lateral ventricle, most frequently in the region of the foramen of Monro, commonly resulting in obstructive hydrocephalus and symptoms of elevated intracranial pressure, the extent of which is proportional to tumor diameter [7]. Owing to their slow growth rate, SEGAs are often 2 to 3 cm at the time of presentation. Sudden death from acute hydrocephalus can occur, with the risk directly proportional to tumor volume [7]. Surgical resection has been the standard treatment for SEGA and is generally curative with gross total excision.

Hemangioma or Strawberry mark is a growth that can form before birth or during infancy that is a collection of abnormal blood vessels. They are very common with more than 200,000 cases per year or 3% of people. It is still not clear what causes blood vessels to form and group together It can be found on the skin or internal organs, mainly found on face, scalp, chest or back. The cells multiply and makes the Hemangioma thicker causing it to swell. Hemangioma are a type of tumor but they are non-cancerous and cause little to no discomfort, this growth may block vision, breathing, and hearing but that is very rare.

Brain tumors grow from abnormal cells which can be cancerous or noncancerous. Symptoms of brain tumors are headaches, muscular weakness like difficulty walking, whole body balance disorder, gastrointestinal (nausea or vomiting), sensory disorder like pins and needles, cognitive or mental confusion, speech (impaired voice), and very common blurred vision. There are various treatments for brain tumors. One includes chemotherapy which kills cells growing or multiplying too quickly. Surgery like craniotomy, this is brain surgery where a piece of bone is removed from the skull. And radiation therapy that uses X-rays to kill abnormal cells. Cancer cells can break off from the tumor into the bloodstream where they can go anywhere in the body. Noncancerous and cancerous brain tumors occur in about 12 in 100,000 people a year.

(Medscape) All ages can be affected but those between twenty and fifty are the most commonly affected. (Mayo Clinic) These rare tumors can either secrete hormones continuously or just sometimes. They primarily occur with “certain familial syndromes”. (Medscape) These autosomal dominant syndromes include two types of “multiple endocrine neoplasia, von Hippel-Lindau disease, and Von Recklinghausen disease”. (Medscape) Norepinephrine is the predominant catecholamine released. (Medscape) The increased blood pressure and cardiac contractibility is directly caused by alpha-adrenergic receptor stimulation, whereas the increased heart rate is caused by beta-adrenergic receptor stimulation (Medscape) The release of catecholamine’s is not caused be neural stimulation in these tumors and it has been stated that is it unknown what triggers the release. (Medscape) The tumors are encapsulated, vascularized and appear reddish brown. Figure 1 is an image of a pheochromocytoma tumor.

Cancer comes in many forms and can attack just about anyone. Some cancers take more time to grow, spread and show signs of attack, but it is very important for everyone to be very careful and cautious because anyone can be a victim for this cruel cancer. Retinoblastoma is one of the cancers that can become very fatal to someone’s vision if not treated. Retinoblastoma is caused when the cancer cells start to grow with no control. Cells can become cancer cells in any part of the body so it is very essential for it to be treated as soon as possible. It can also extend to any other parts of the body. Retinoblastoma is started in the retina of the eye and is most common in children.

Medulloblastoma, also called cerebellar primitive neuroectodermal tumor (PNET), is a fast growing embryonal tumors[A] located in the cerebellum caused by a glial cell (1)(4). The seven types of medulloblastoma are, classic,desmoplastic, nodular,, large-cell,(anaplastic), neuroblastic, glial differentiation, medullomyoblastoma, and melanotic. According to American Brain Tumor Association, medulloblastoma is relatively rare, and only accounts for two percent of all primary brain tumors(1).However, medulloblastoma is common in children making up to twenty percent of childhood brain tumors(2), while it is less common in adults(1). It is also more common in men than in women(3). (Thesis)

Leucocoria is often the first visible sign of retinoblastoma in 60% of cases [1]. The retinoblastoma tumour usually presents a distinct growth pattern; exophytic tumours extend into the subretinal space and progressively detach from the retina, causing a loss of central vision which induces symptoms of secondary strabismus in 20% of cases [1]. Failure to treat patients in early life will compromise vision, as seeding of the tumour within the eye disrupts fluid flow and increases the intraocular pressure. Secondary tumours in the optic nerve, central nervous system, lungs, liver and bone marrow are also common consequences following systemic metastasis of the tumour [1].

Common malignant brain cancers increase significantly according to statistical data collected by the National Cancer Institute. In 1984, the annual incidence rates of primary brain tumor and primary brain lymphoma also increased notably, the rate of lymphoma almost tripling,

Cavernous Angioma also referred to as cavernous malformations (CMs), cavernous hemangiomas, or cavernomas are a type of intracranial vascular malformation with developmental origin. The terms 'haemangioma' and 'cavernoma' are a misnomer as the lesions are non neoplastic.