Trilateral Retinoblastoma
Trilateral retinoblastoma (TRb) is a malignant midline primitive neuroectodermal tumor occurring in patients with inherited uni- or bilateral retinoblastoma. In most cases trilateral retinoblastoma presents itself as pineoblastoma Pineoblastoma is a malignant tumor of the pineal gland. A pineoblastoma is a supratentorial midline primitive neuroectodermal tumor. Pineoblastoma can present at any age, but is most common in young children. They account for 0.001% of all primar ... (pineal TRb). In about a fourth of the cases the tumor develops in another intracranial region, most commonly supra- or parasellar (non-pineal TRb), but there are reported cases with non-pineal TRb in the 3rd ventricle. In most cases pineal TRb is diagnosed before the age of 5, but after the diagnosis of retinoblastoma. Non-pineal TRb, however, is often diagnosed simultaneous with retinoblastoma. Prognosis of patients with trilateral retinoblastoma is dismal, only a few patients have survived more than 5 ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
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Oncology
Oncology is a branch of medicine that deals with the study, treatment, diagnosis, and prevention of cancer. A medical professional who practices oncology is an ''oncologist''. The name's Etymology, etymological origin is the Greek word ὄγκος (''ónkos''), meaning "tumor", "volume" or "mass". Oncology is focused on the diagnosis of cancer in a person, therapy (e.g., surgery, chemotherapy, radiotherapy and other modalities), monitoring of patients after treatment, palliative care of people with advanced-stage cancers, Ethics, ethical questions surrounding cancer care, Screening (medicine), screening of patients, and the study of cancer treatments through clinical research. An oncologist typically focuses on a specialty area in cancer treatment, such as surgery, Radiation therapy, radiation, gynecology, gynecologic oncology, geriatrics, geriatric oncology, pediatrics, pediatric oncology, and various organ-specific disciplines (breast, brain, liver, among others). The exp ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
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Primitive Neuroectodermal Tumor
Primitive neuroectodermal tumor is a malignant (cancerous) neural crest tumor. It is a rare tumor, usually occurring in children and young adults under 25 years of age. The overall 5 year survival rate is about 53%. It gets its name because the majority of the cells in the tumor are derived from neuroectoderm, but have not developed and differentiated in the way a normal neuron would, and so the cells appear "primitive". PNET belongs to the Ewing family of tumors. Genetics Using gene transfer of SV40 large T-antigen in neuronal precursor cells of rats, a brain tumor model was established. The PNETs were histologically indistinguishable from the human counterparts and have been used to identify new genes involved in human brain tumor carcinogenesis. The model was used to confirm p53 as one of the genes involved in human medulloblastomas, but since only about 10% of the human tumors showed mutations in that gene, the model can be used to identify the other binding partners of SV40 ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
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Retinoblastoma
Retinoblastoma (Rb) is a rare form of cancer that rapidly develops from the immature cells of a retina, the light-detecting tissue of the eye. It is the most common primary malignant intraocular cancer in children, and 80% of retinoblastoma cases are first detected in those under 3 years old. Though most children in high income countries survive this cancer, they may lose their vision in the affected eye(s) or need to have the eye removed. Almost half of children with retinoblastoma have a hereditary genetic defect associated with it. In other cases, retinoblastoma is caused by a congenital mutation in the chromosome 13 gene 13q14 ( retinoblastoma protein). Signs and symptoms Retinoblastoma is the most intrusive intraocular cancer among children. The chance of survival and preservation of the eye depends fully on the severity. Retinoblastoma is extremely rare as there are only about 200 to 300 cases every year in the United States. Globally, only 1 in about 15,000 childre ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
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Pineoblastoma
Pineoblastoma is a malignant tumor of the pineal gland. A pineoblastoma is a supratentorial midline primitive neuroectodermal tumor. Pineoblastoma can present at any age, but is most common in young children. They account for 0.001% of all primary CNS neoplasms. Epidemiology Pineoblastomas typically occur at very young ages. One study found the average age of presentation to be 4.3 years, with peaks at age 3 and 8. Another cites cases to more commonly occur in patients under 2 years of age. Rates of occurrence for males and females are similar, but may be slightly more common in females. One study found incidence of pineoblastoma to be increased in black patients compared to white patients by around 71%. This difference was most apparent in patients aged 5 to 9 years old. Pathophysiology The pineal gland is a small organ in the center of the brain that is responsible for controlling melatonin secretion. Several tumors can occur in the area of the pineal gland, with the most ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
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Sella Turcica
The sella turcica (Latin for 'Turkish saddle') is a saddle-shaped depression in the body of the sphenoid bone of the human skull and of the skulls of other hominids including chimpanzees, gorillas and orangutans. It serves as a cephalometric landmark. The pituitary gland or hypophysis is located within the most inferior aspect of the sella turcica, the hypophyseal fossa. Structure The sella turcica is located in the sphenoid bone behind the chiasmatic groove and the tuberculum sellae. It belongs to the middle cranial fossa. The sella turcica's most inferior portion is known as the hypophyseal fossa (the "seat of the saddle"), and contains the pituitary gland (hypophysis). In front of the hypophyseal fossa is the tuberculum sellae. Completing the formation of the saddle posteriorly is the dorsum sellae, which is continuous with the clivus, inferoposteriorly. The dorsum sellae is terminated laterally by the posterior clinoid processes. Development It is widely believed th ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
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Endocrine Neoplasia
The endocrine system is a messenger system in an organism comprising feedback loops of hormones that are released by internal glands directly into the circulatory system and that target and regulate distant organs. In vertebrates, the hypothalamus is the neural control center for all endocrine systems. In humans, the major endocrine glands are the thyroid, parathyroid, pituitary, pineal, and adrenal glands, and the (male) testis and (female) ovaries. The hypothalamus, pancreas, and thymus also function as endocrine glands, among other functions. (The hypothalamus and pituitary glands are organs of the neuroendocrine system. One of the most important functions of the hypothalamusit is located in the brain adjacent to the pituitary glandis to link the endocrine system to the nervous system via the pituitary gland.) Other organs, such as the kidneys, also have roles within the endocrine system by secreting certain hormones. The study of the endocrine system and its disorders is kn ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |