CNRN-Tumors Exam 21 Questions with Verified Answers
Astrocytoma - CORRECT ANSWER Star-shaped glioma derived from astrocytes (maintain health of nerve
... [Show More] cells). Astrocytomas are frequently found in the cerebrum, cerebellum, hypothalamus, optic nerve, pons and also the spinal cord.
Several types:
Brain stem gliomas - rare, in adults and hard to cure
Pineal Astrocytic tumors - form around the Pineal Gland
Pilocystic astrocytomas - Mostly in children and young adults (and those with neurofibromatosis)
Diffuse astrocytomas - mostly in young adults (especially with those Li-Fraumeni syndrome)
Anaplastic astrocytomas and glioblastomas - Most common in adults.
Mixed gliomas are composed of two types of tumor cells (astrocytes and oligodendrocytes)
Low grade astrocytomas tend to occur more often in children.
Chordoma - CORRECT ANSWER Tumor derived from embryonic notochord cells. Rare; usually found at the base of the spine; invade both bone and soft tissues, causing pain.
Dermoid - CORRECT ANSWER Teratoma; Benign Cyst; Most occur in young adults, found in sacrococcygeal area, associated with spina bifida.
Ependymoma - CORRECT ANSWER Primary glioma derived ependymal cells that line the ventricles. Typically found in the cerebrum, but can also spread to the spine via the CSF; occur in children and young adults;
Often occur in the fourth ventricle and lead to obstructive hydrocephalus.
Spinal ependymomas occur more often in men.
Glioma - CORRECT ANSWER Tumor derived from the glial tissue. Glia include astrocytes, oligodendrocytes and ependymal cells. Gliomas are the most common type of primary brain tumor. Spinal gliomas are usually cervical.
Meningioma - CORRECT ANSWER Tumor that arises from the meninges; spinal meningiomas occur more often in women. They originate in the arachnoid layer and are often attached to the dura. Most are slow-growing and benign and do not elicit any symptoms. They do however, compress brain and spinal cord tissue and may block the flow of CSF and/or erode into bone.
With faster-growing, malignant meningiomas, symptoms are pronounced. Brain meningiomas tend to be located between the cerebral hemispheres or uner the top of the skull, whereas spinal meningiomas are found along the spinal column.
Neurofibroma - CORRECT ANSWER Heterogenous tumor derived from Schwann cells surrounding peripheral nerves; cannot be resected without damage to the underlying nerve; infiltrates surrounding tissues; genetic origin; in the spine most are located above L1.
Neurofibromatosis Type I - CORRECT ANSWER Genetic disorder in which neurofibromas develop on peripheral nerves and also in the skin. Also called Von Recklinghausen's Disease; genetic disorder associated with a mutation on chromosome 17.
Skin hyperpigmentation, brwon or tan patches, may be apparent at birth or develop within a few years. Tumors associated with problems occur in childhood and are found in the iris of the eye or along the optic nerve and are associated with visual disturbances.
Neurofibromas in the periphery affect bone, casuing disfigurement, including scoliosis.
Many of these children also have learning disabilities.
Neurofibromas tend to be resistant to radiation.
Neurofibromatosis Type II - CORRECT ANSWER Genetic disorder in which a schwannoma develops on the acoustic (vestibulocochlear) nerve. Also called an acoustic neuroma; auditory and vestibular symptoms arise such as hearing loss, tinnitus and vertigo.
If the tumor spreads beyond the auditory area, function of the facial, trigeminal, glossopharyngeal and vagus nerves may also be affected.
Prognosis is generally good, however hearing and facial sensation may be permanently affected.
Oligodendroglioma - CORRECT ANSWER Glioma derived from oligodendrocytes. Found in the cerebrum; tumors are often calcified; seizure is often the first symptom.
Sarcoma - CORRECT ANSWER Tumor derived from the connective tissue or bone; typically extradural.
Embryonic Brain Tumor - CORRECT ANSWER Consists of rapidly growing cells in masses, originating in the embryonic or fetal tissue of the brain and spinal cord during fetal development.
Most are malignant; however some may be benign.
Meningeal Brain Tumor - CORRECT ANSWER These tumors form in the thin tissue layers covering the brain and spinal cord. They are most common in adults and include three grades of tumors:
1) Grade I Tumors - Most common, prevalent in women; benign and slow-growing; typically form in the dura mater covering the brain near the skull.
2) Grade II and III Tumors - Prevalent in men and include rare, malignant, fast-growing tumors that spread within the brain and spinal cord.
Capillary Hemangioblastoma - CORRECT ANSWER Classified as WHO grade I and associated with von Hippel-Lindau (VHL) disease, which is a familial tumor syndrome. Occurs sporadically, typically in adults at an average of 29 years. May develope in various sites of the central nervous system such as the cerebellum, the brain stem and the spinal cord.
Median life expectancy of a patient with VHL disease is 49 years; the capillary hemangioblastoma is the most likely cause of death.
Pineal Parenchymal Brain Tumor - CORRECT ANSWER Tumor originating in the pineocytes or parenchymal cells and classified into three types:
1) Pineocytomas - WHO Grade II; slow-growing with variable prognosis, occuring primarily in young adults
2) Pineoblastomas - WHO Grade IV; rare, primative embyonal tumors that are highly malignant, rapid-growing and more likely to spread, often with a grim prognosis compared to pineocytomas; occuring primarily in children
3) Pineal tumors of intermiediate differentiation, which are considered diverse, monomorphous tumors that are difficult to assign a prognosis becuase of their unpredictable clinical behavior and growth; they occur in all age groups.
Lymphoma - CORRECT ANSWER Arise from lymphatic cells, occuring at nodes, but also in other areas of lymph distribution or the brain. More common in immunocompromised patients such as organ transplant recipients and AIDS patients.
Usually malignant, often recur after treatment and do occur in children.
Hemangioblastoma - CORRECT ANSWER Derived from capillary endothelial cells and typically occur in the cerebellum. They occur sporadically or as part of von Hippel-Lindau disease, which is an inherited syndrome in which tumors develop in several other tissues including the kidneys and adrenal glands.
Pituitary Tumors - CORRECT ANSWER Tumors of this origin may secrete hormones, and tend to do so excessively. Non-secretory tumors cause symptoms due to compression of adjacent nerves, most notibly the optic nerve, causing visual disturbances (scotoma, optic atrophy, paresis of extraocular muscles).
Most common secretion is prolactin, which can cause infertility and amenorrhea. Growth hormone oversecretion can giantism or acromegaly.
Prolactin, groth hormone and cortisol levels are tested in the diagnosis of pituitary hormone.
Brain and Spinal Cord Metasteses - CORRECT ANSWER Metastatic brain tumors originate away from the cells of the brain; usually composed of cells that escaped from lung tumors, or sometimes breast, GI or skin tumors. Most brain tumors do not metastesize elsewhere in the body, but medulloblastomas may migrate to the spinal cord.
Spinal Cord tumors may come from the lungs, breast, prostate, colon, kidneys or uterus. The location of the primary organ tends to determine the location of the spinal metastesis because invasion occurs through adjacent vertebrae (the thoracic area is most commonly involved).
Craniopharyngioma - CORRECT ANSWER Congenital tumor arising from Rathke's puch of the pituitary. It occurs predominantly in children , but is sometimes viewed in adults. Like other pituitary tumors, it affects endocrine function and may compromise the optic nerve.
Often impinges the third ventricle, which may hinder CSF flow and lead to increased ICP.
Spinal Cord Tumors - CORRECT ANSWER May be Extramedullary or Intramedullary
Extramedullary Tumors - situated outside of the spinal cord; may be extradural or intradural; Most extradural-extramedullary primary tumors are chordomas or sarcomas.
Intradural-extramedullary primary tumors include meningiomas and neurofibromas. This category comprises the highest percentage of spinal cord tumors and they can be treated by surgical incision alone. [Show Less]