Craniopharyngioma+(child)

Symptoms caused by craniopharyngioma can be general (caused by pressure building up in the brain), or specific (affecting the normal functioning of the part of the brain where the tumor is pressing on nerves or blood vessels). Generally, craniopharyngioma is not diagnosed until symptoms appear[7].
 * **Epidemiolgy:** || Peak age is between 5 to 10 years of age predominantly but also seen in the 5-14 years of age. [3] This type of brain tumor is rare with 300 cases diagnosed per year and 100 of those are children under the age of 14. There is a higher incidence of this tumor type in Asia and Africa. Aside from the change in distribution of cases race does not have an impact and this tumor type. Sex does not play a significant role but there is a slight dominance towards males. [4] ||
 * **Etiology:** || There is no clear link either biologically or environmental to the cause of this type of tumor. It is been proposed that craniophyngioma tumors are Transformation of normal cells into neoplastic ones likely involves multiple genomic changes, including loss of tumor-suppressor genes, activation of oncogenes, and alterations in DNA repair and methylation mechanisms.[4[3]]there have been studies that show the beta-catenin pathway plays a role in this tumor due to its downstream signal pathway. [4] ||
 * **Signs & Symptoms:** || Children with craniopharyngioma may experience any of the symptoms or signs listed below[7]. Sometimes, children with craniopharyngioma do not show any of these symptoms, or these symptoms may be caused by another medical condition that is not a tumor[7].

General symptoms include[7]:

Headaches, which may be severe and may be worse in the early morning

Nausea and/or vomiting

Difficulty with balance

Increased sleepiness or fatigue

Mood or behavior changes

Location-specific symptoms include:

Vision changes, blurriness, or loss of peripheral vision

Excessive thirst

Increased urination

Slow or halted growth

Excessive weight gain

Early or delayed puberty

The doctor will ask you and your child questions about the symptoms your child is experiencing to help find out the cause of the problem, called a diagnosis. This may include how long you’ve been experiencing the symptom(s) and how often[7].

If a tumor is diagnosed, relieving symptoms and side effects remains an important part of your child’s care and treatment[7]. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with the health care team about symptoms your child experiences, including any new symptoms or a change in symptoms[7]. || The following tests may be used to diagnose craniopharyngioma: Physical examination. The doctor will examine the patient’s head and body and ask questions about the symptoms the patient is experiencing and his/her medical history. This may also include tests to check the patient’s vision and CNS functioning. Blood tests[7]. The doctor may recommend different blood tests, including checking the levels of certain hormones, to help determine whether there is reason to look for a brain tumor. Results of the physical examination and blood tests may suggest that imaging tests are needed to look for a craniopharyngioma. There are two main types of imaging tests used to find craniopharyngioma[7]. Magnetic resonance imaging (MRI)[7]. An MRI uses magnetic fields, not x-rays, to produce detailed images of the brain and spinal column. A contrast medium may be injected into a patient’s vein to create a clearer picture. Treatment)[7].
 * **Diagnostic Procedures:** || In diagnosing craniopharyngioma, the child’s doctor may consider different factors when choosing tests, including[7]:
 * Child’s age and medical condition
 * Type of tumor suspected
 * Severity of symptoms
 * Previous test results

|| The American Joint Committee on Cancer recommends the following guidelines for grading tumors: [8] __**GX:**__ Grade cannot be assessed (Undetermined grade) __**G1:**__ Well-differentiated (Low grade) __**G2:**__ Moderately differentiated (Intermediate grade) __**G3:**__ Poorly differentiated (High grade) __**G4:**__ Undifferentiated (High grade) || __** Neurocognitive problems: **__ __** Depression: **__ __** Seizures: **__ __** Visual problems: **__ __** Pituitary and hypothalamic dysfunction: **__ __** During infection, surgery, and illness, optic glioma survivors are very likely to need support with extra steroid medication: **__ __** Increased risk for cerebrovascular events (strokes): **__ __** Increased risk of second neoplasms: **__ __** Cranial RT more than 10 years old can be associated with scattered radiation to the thyroid. There is an increased risk of: **__ Radiation therapy offers a role for incompletely resected tumors. ||
 * **Histology:** || forming during embryogenesis as a benign, squamous cell tumor in the region of the pituitary stalk and considered a tumor of the sellar region by the World Health Organization. [2] ||
 * **Lymph node drainage:** || There are no true lymphatic pathways from the brain.[5] ||
 * **Metastatic spread:** || Primary brain tumors very rarely spread outside of the cranial cavity and are more likely to invade other areas of the brain. Brain metastases are spread from other areas of the body to the brain and are not associated with a primary brain lesion. [6] ||
 * **Grading:** || Tumor grade is a system used to classify cancer cells by how abnormal they look under a microscope, and how quick the tumor can grow and spread. The specific factors that determine tumor grade vary with each type of cancer. [8]
 * **Staging:** || Staging refers to the extent and severity of the cancer. There is no formal staging for pediatric Craniopharyngioma. It is usually described as “newly diagnosed” or “recurrent disease”. [9] ||
 * **Radiation side effects:** || Some of the late effects of Radiation Therapy (RT) on a Craniopharyngioma are: [10]
 * Poor short term memory
 * Difficulty with executive function
 * Usually related to multiple long-term health problems and neurocognitive dysfunction
 * Side effect of scarring related to tumor and previous RT
 * Responds to anticonvulsant therapy
 * Can lead to optic atrophy, decreased vision and field defects
 * RT may damage the optic nerves and chiasm
 * RT can cause cataracts (lens of the eye is very sensitive to low dose RT)
 * Often children with Craniopharyngiomas present with pituitary insufficiency. RT can make the situation worse.
 * Increased risk of metabolic syndrome (with hypertension)
 * Blood pressure should be checked in any patient who has a history of cranial RT
 * GH deficiency (common with failure to grow)
 * Risk of TSH deficiency
 * Risk of LH/FSH deficiency
 * Risk of ACTH deficiency
 * They should be seen by an endocrinologist yearly to supervise replacement therapy
 * RT effect on cerebral vasculature
 * Significantly increased risk of TIA’s and cerebrovascular disease in these patients
 * RT induced Meningioma is common
 * RT induced malignancy is less common
 * Hypothyroidism
 * Thyroid cancer and multinodular goitre ||
 * **Prognosis:** || The chance of recovery depends on the size of the tumor, where the tumor is located in the brain, whether the tumor is solid or a fluid-filled cyst, whether there are tumor cells left surgery, the child’s age, and whether the tumor has just been diagnosed or has recurred. In general, the prognosis is favorable, with an 80-90% chance of cure if the tumor can be completely removed. [1] ||
 * **Treatments:** || Treatment is controversial; however, total resection is the primary approach. Chemotherapy does not play a role.
 * Post-op SRS has been reported for residual or recurrent disease.
 * Treatment volume is the target volume, including solid components and cysts.
 * High-energy photons are used with 2 or 3 static fields or coronal arcs.
 * 50-60 Gy given at 180 cGy/fraction. [2]

Brain: Whole 4500 cGy, 2/3 5000 cGy, 1/3 6000 cGy Ear (acute serous otitis: Whole 3000 cGy, 2/3 3000 cGy, 1/3 3000 cGy Ear (chronic serous otitis: Whole 5500 cGy, 2/3 5800 cGy, 1/3 6000 cGy Lens: Whole 1000 cGy Optic chiasm: Whole 5000 cGy Optic nerve: Whole 5000 cGy Retina: Whole 4500 cGy [3] || 2. Chao KS, Perez CA, Brady LW. //Radiation Oncology: Management Decisions.// Philadelphia, PA: Lippincott Williams & Wilkins; 2002. 3. RadiationOncology/Toxicity/Emami. []. Accessed June 25, 2012. 4. Wloters Kluwer health. Uptodate, craniopharyngioma. Available at: [|http://www.uptodate.com/contents/craniopharyngioma#H1]. Accessed 06/26/2012 5. Washington CM, Leaver D. Principles and Practice of Radiation Therapy. St. Louis, MO: Mosby; 2010 6. Brain Cancer Overview. Available at: __ ** [] ** __, Accessed June 28 2012. 7. Craniopharyngioma-Childhood. April 23, 2012. Available at:http://www.cancer.net/patient/Cancer+Types/Craniopharyngioma+-+Childhood/ci.Craniopharyngioma+-+Childhood.printer. Accessed on: June 28, 2012. 8. Tumor grade. National Cancer Institute. Available at: []. Accessed on June 25, 2012. 9. Childhood Craniopharyngioma treatment. National Cancer Institute. Available at: []. Accessed on June 25, 2012. 10. Craniopharyngioma. Pediatric Oncology Education Materials. Available at: []. Accessed on June 25, 2012. ||
 * **TD 5/5:** || Brainstem: Whole 5000 cGy, 2/3 5300 cGy, 1/3 6000 cGy
 * **References:** || 1. National Cancer Institute. []. Accessed June 25, 2012.

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