Epidemiology:
"Craniopharyngioma are the most common nonglial brain tumors in children, occurring primarily in the late first and second decades, although they can present at any age."⁷
Etiology:
Although there are no proven etiologic factors for brain tumors there are some risk factors that can be found. Environmental factors have been implicated in occupations such as farmers or petrochemical workers. No increase of risk has been noticed with any viral association with the exception the Epstein-Barr virus with CNS Lymphoma. Some hereditary syndromes that are associated are Neurofibromatosis type 1, Li-Fraumeni Syndrome, Von Hippel-Lindau Syndrome, Turcot's syndrome, and nevoid basal cell carcinoma syndrome.⁷
Signs & Symptoms:
Headache, mental changes, hemiplegia, seizure, vomiting (and ocular changes), cranial nerve defects (2-7).⁸
Diagnostic Procedures:
Workup as detailed by Chao, et. al. includes: complete history and physical, complete neurologic evaluation, CT or MRI head scan (or both) with contrast, positron emission tomography (PET) scan, complete blood count, cerebral spinal fluid chemistry tests, cytology and microbiology studies. Neurologic exam includes mental condition, coordination, sensation, reflexes and motor + cranial nerves. Ophthalmoscopy to check for papilledema, which indicates intracranial pressure. MRI contrast scan of the complete neuraxis for staging.¹
Histology:
Craniopharyngiomas are usually located in the suprasellar region.² There are two types: adamantinomatous and papillary, which are normally benign.³ Adamantinaomatous closely resemble adamantinoma of the manible and papillary usually consist of squamous epithelium.⁴Although both lesions can be either solid or cystic, they usually have cystic qualities with adamantinomatous containing calcifications whereas papillary does not.⁵
Lymph Node Drainage:
There is no lymphatic vasculature in the CNS and tumors of the CNS do not metastsize through lymphatics.⁶
Metastatic Spread:
Craniopharyngioma is a benign tumor that is usually slow-growing and not as likely to spread.¹³
Grading:
Craniopharyngioma is a grade ll. The grade of a tumor refers to how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread. the pathologist determines the grade of the tumor using tissue removed for biopsy. The following grading system may be used for adult brain tumors. Grade l, The tumor grows slowly, has cells that liil similar to normal cells, and rarely spreads into nearby tissues. it may be possible to remove the entire tumor by surgery. Grade ll, the tumor grows slowly, but may spread into nearby tissue and may become a higher grade tumor. Grade lll, the tumor grows quickly, is likely to spread into nearby tissue, and the tumor cells look very different from normal cells. Grade lV, the tumor grows very aggressively, has cells that look very different from normal cells, and is difficult to treat successfully.¹⁴
Staging:
Craniopharyngioma rarely, if ever, spreads. There is no standard staging system for this type of tumor.
Staging is a way of describing a tumor, such as where it is located, if or where it has spread, and if it is affecting the functions of other organs in the body. After a brain tumor has been diagnosed, additional tests will be done to learn more about the tumor.
There is no formal staging system for adult brain tumors. Instead, once a brain tumor has been diagnosed, the pathologist will perform several tests on a tissue sample of the tumor to learn as much as possible about the tumor. If the tumor is a glial brain tumor, the pathologist will assign a “grade” using a number from I to IV (one to four). The grade number indicates the degree of abnormality of the tumor cells. The degree of malignancy is often determined by characteristics of the tumor, as seen under the microscope. Generally, the lower the grade, the better the prognosis (chance of recovery or long-term control of the tumor).¹⁴

Radiation Side Effects:
General side effects include nausea and vomiting, radiation dermatitis, alopecia, fatigue, and decreased blood counts. If the ear is included, otitis externis, and high tone hearing loss can result. If the eye is treated then cataract formation can result as well as changes in visual acuity, visual field, or blindness in doses above 54 Gy.
Cranial treatment can result in decreased learning ability, deficits in short term memory, and difficulties with problem solving
6-12 weeks after radiation, neurologic deterioration may occur and usually responds to steroid treatment.¹²

Prognosis:
If there is a complete resection, long term control and cure is a possibility. However, if there is tumor recurrence prognosis is worse. Other important prognostic indicators are age, performance status, and tumor grade.¹²
Treatments:
Treatment is surgery alone or combination of surgery and radiation. Typical radiation doses are 50-54 Gy in 1.8 Gy fractions. They are given to a margin of 1.5 cm around the tumor volume. Techniques include 3-field treatments, with opposing lateral fields and a vertex field. Also 3D conformal treatment may be used.¹¹

If the patient presents with any type of compressive symptom, the compression must be relieved surgically prior to radiation as the radiation can induce edema and cause the symptom to get worse.¹¹

TD5/5:
Cranio_and_Meningio.jpg
Table 1 TD5/5,TD50/510
Paired_wedge_field.JPG
Figure 1. Paired Wedge Fields9
Vertex_field.JPG
Figure 2. Vertex Angulation9

References
1. Chao KS, Perez CA., Brady LW. Radiation Oncology - Management Decisions. 2nd ed. Philadelphia, PA: Lippincott, Williams & Wilkins; 2002:132-133.
2. Chao KS, Perez CA., Brady LW. Radiation Oncology - Management Decisions. 2nd ed. Philadelphia, PA: Lippincott, Williams & Wilkins; 2002:153.
3. Elmaci L, Kurtkaya-Yapicier O, Ekinci G, et. al. Metastatic papillary craniopharyngioma: case study and study of tumor angiogenesis. Neuro-Oncology. 2002;4(2):123-128.
4. Elmaci L, Kurtkaya-Yapicier O, Ekinci G, et. al. Metastatic papillary craniopharyngioma: case study and study of tumor angiogenesis. Neuro-Oncology. 2002;4(2):123-128.
5. Elmaci L, Kurtkaya-Yapicier O, Ekinci G, et. al. Metastatic papillary craniopharyngioma: case study and study of tumor angiogenesis. Neuro-Oncology. 2002;4(2):123-128.
6. Adams RD, Langlin L, Leaver D. Central nervous system. In: Washington CM, Leaver D, eds. Principles and Practice of Radiation Therapy. 2nd ed. St. Louis, MO: Mosby; 2004:726-727.

7. Devita, Hellman, Rosenberg's. Cancer-Principles and Practices of Oncology. 8th ed. Philadelphia, PA: Lipincott, Williams & Wilkins; 2008.
8. Washington CM, Leaver D, eds. Principles and Practice of Radiation Therapy. 2nd ed. St. Louis, MO: Mosby; 2004.



9. Chao KS, Perez CA., Brady LW. Radiation Oncology - Management Decisions. 2nd ed. Philadelphia, PA: Lippincott, Williams & Wilkins; 2002: Figure 1 and 2
10. Wikibooks en.wikibooks.org TD5/5 adapted from Emami 1991

11. Chao KS, Perez CA. Brady LW. Radiation Oncology - Management Decisions. 2nd ed. Philadelphia, PA: Lippincott, Williams & Wilkins. 2002
12. Washington CM, Leaver D. Principles and Practice of Radiation Therapy. 2nd ed. St. Louis, MO: Mosby. 2004

13. Chao KS, Perez CA. Brady LW. Radiation Oncology- Management Decisions. 2nd ed. Philadelphia, PA: Lippincott, Williams & Wilkins. 2002
14. www.cancer.net/craniopharyngioma Accessed January 12, 2010

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