Craniopharyngioma+(child)

These tumors can cause symptoms in three major ways. 2 Large Craniopharyngiomas may present with little to no time for diagnostic workup as surgery is sometimes rushed along to decrease the amount of pressure the tumor is creating within the brain. 2 If the tumor is present without the need for emergent surgery. A more standard Computed Tomography (CT) scan is performed to determined size and location. 2 Another diagnostic tool that may be used are endocrinologic and ophthalmologic evaluations. 2 || Craniopharyngioma is a type of benign neoplasm that can be found in the pituitary gland of both children and adults. However, the adult version of this disease is very different from the cancer seen in children. Most tumors that occur in adults are squamous epithelial cells while tumors occurring in children are microscopically adamantinomata tissue. 3 They can contain both cystic and solid components and can have calcium deposits as well. 3 The image below is a typical representation of the adamantinomata tissue found in children.
 * **Epidemiolgy:** || Rachel
 * Craniopharyngioma is a benign tumor, arising from squamous cells rests derived from Rathke's pouch during embryogenesis, in the region of the pituitary stalk. 1
 * Craniopharyngioma presents as a suprasellar tumor, frequently partially calcified and usually including an intrasellar component.
 * Cystic or solid tumor extension may occur laterally into the middle cranial fossa or posteriorly into the posterior fossa. ||
 * **Etiology:** || Rachel
 * Endocrine deficits are apparent in 50% to 90% of children at diagnosis, most often related to growth hormone, thyroid -stimulating hormone, and adrenocorticotropic hormone; diabetes insipidus is present in 10%-15%. 1 ||
 * **Signs & Symptoms:** || Brandon
 * Impaired Hormonal Function
 * This is due to the fact that most of these tumors grow in close proximity to the pituitary gland. Since the pituitary gland is responsible for all kind of hormonal function, deficiencies may be noticeable such as slow grow rate.
 * Increased Brain Pressure
 * The location of the tumor may affect the flow of fluid in the brain and as a result increased pressure is noted
 * Loss of vision
 * Tumor growth may also impinge on the optic nerves cause sudden vision loss in one or both eyes. ||
 * **Diagnostic Procedures:** || Brandon
 * **Histology:** || Ashley

|| Because the brain doesn't contain lymph node channels, metastasis do not occur through lymphatics. 5 || Craniopharyngiomas rarely metastasis outside central nervous system (CNS). 6 || According to world health organization (WHO), craniopharyngiomas are classified as Grade I central nervous system (CNS) tumors. 7 || Craniopharyngiomas rarely spread to parts of the body far from where it started. Therefore, there is no standard staging system for this type of tumor. 8 || Radiation therapy can cause age-related permanent learning and memory problems, as well as slow the metabolism and decrease levels of hormones needed for the body to function normally. 8 || Relatively good outcome. 10 yr survival rate for patient's with a gross total resection is 86-100%. 10 yr survival rate for patient's with a subtotal resection or with a recurrence treated with surgery and radiation therapy is 57-86%. 9 || General Management * Treatment for craniopharyngioma is controversial. Radiation Therapy * The target volume for cranipharyngioma is narrowly confined to the tumor volume, including the solid component and cyst.
 * **Lymph node drainage:** || Ashley
 * **Metastatic spread:** || Amanuel
 * **Grading:** || Amanuel
 * **Staging:** || Lindsey
 * **Radiation side effects:** || Lindsey
 * **Prognosis:** || Kevin
 * **Treatments:** || Kevin
 * Total resection as the primary approach is attempted in most cases.
 * For incompletely resected rumors, it is generally preferable to administer postoperative irradiation rather than await tumor progression.
 * They cystic nature of craniopharyngiomas has led to trials of intracystic application of beta-emitting radionuclides such as yttrium 90 or phosphorus 32.
 * There are no data regarding systemic chemotherapy for craniopharyngioma and only limited reports of intracystic bleomycin sulfate.
 * In cases with cyst aspiration or limited resection, it is important to cover the cyst wall.
 * It is appropriate to limit the target volume to postoperative residual tumor if large cystic components are remove surgically.
 * High-energy photons are used with two or three stationary fields or the classic coronal arc configuration.
 * Stereotactic irradiation or 3-D conformal therapy limiting the high-dose volume to the well-cirumscribed neoplasm is preferred.
 * Improved disease control has been reported with doses of 50 to 60 Gy using conventional fractionation (1.8 Gy per day)
 * Toxicity is associated with doses higher than 60 Gy. 10

Craniopharyngioma treatment plan in a pediatric patient. 11

||
 * **TD 5/5:** || Jenn
 * Tissue dose associated with 5% injury rate within 5 years ** . **(TD 5/5)** 12

2. Craniopharyngioma. Boston Children's Hospital. []. Accessed July 3, 2013. 3. Craniopharyngioma. Wikipedia Web site. []. Updated June 14, 2013. Accessed June 28, 2013. 4. Chang D, Lee S. (2012, November). Suprasellar tumor with rapid decline in vision. Endocrine Today, (20)6, 3. Retrieved from []- 47B398E00F53%7D/Suprasellar-tumor-with-rapid-decline-in-vision. 5. Chao KS, Perez CA, Brady LW. //Radiation Oncology Management Decisions//. 3rd ed. Philadelphia, PA: Lippincott, Williams, and Wilkins; 2011. 6. Brain tumor FQA. National brain society. [|http://www.braintumor.org/patients-family-friends/about-brain-tumors/brain-tumor-faq.html#q2425]. Accessed July, 2013. 7. Craniopharyngioma. Radiopaedia. http://radiopaedia.org/articles/craniopharyngioma. Accessed July 1, 2013. 8. Craniopharyngioma-Childhood. http://www.cancer.net/cancer-types/craniopharyngioma-childhood/staging. Accessed July 3, 2013. 9. eMedicine. Craniopharyngioma. []. Accessed June 27, 2013. 10. Chao KS, Perez CA, Brady LW. //Radiation Oncology - Management Decisions//. 3rd ed. Philadelphia, PA; Lippincott, Williams and Wilkins, 2011: 730-731. 1 1. Winkfield K., et al. Surveillance of Craniopharyngioma Cyst Growth in Children Treated with Proton Radiotherapy. Int. J. Radiation Oncol. 2009; 73(3): 716-721. 1 2. Washington C, Leaver D. Principles and Practice of Radiation Oncology. 3rd ed. St. Louis, MO. Mosby Elsevier; 2010: 80-82 ||
 * Ear (Middle): Serous otitis 5000cGy
 * Ear (Vestibular): Meniere’s syndrome 6000cGy
 * Retina: Blindness 5500cGy
 * Cornea: Blindness 5000cGy
 * Lens: Blindness 500cGy
 * Salivary Glands: Xerostomia 5000cGy
 * Oral cavity and pharynx: Ulceration 6000cGy
 * Pituitary: Reduced hormone production 4500cGy ||
 * **References:** || 1. Chao KS, Perez CA, Brady LW. Radiation Oncology - Management Decisions. 3rd ed. Philadelphia, PA; Lippincott, Williams and Wilkins, 2011: 730.

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