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Peak age is between 5 to 10 years of age. There are fewer than 100 pediatric cases of craniopharyngioma in the U.S. each year.
No known environmental or infectious causes predispose to the development of craniopharyngiomas. Little is also known regarding the genetic basis for craniopharyngiomas. 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. Craniopharyngioma studies have identified the beta-catenin pathway as playing a potential role in the pathogenesis of these tumors
Signs & Symptoms:
Focal neurologic dysfunction, increased intracranial pressure, hydrocephalus. The neurologic effects of the locally growing or infiltrating tumor may predict the tumor location. Headache usually worse in the morning due to intracranial pressure. Optic atrophy or blindness. Seizures, back pain, bowel or bladder dysfunction may suggest CSF metastasis.
CT, MRI, and PET scan, complete blood count, cerebral spinal fluid chemistry tests, cytology and microbiology studies. Neurologic exam includes mental condition, coordination, sensation, reflexes and motor tests
Craniophyaryngioma is a benign tumor arising from squamous cell rests derived from Rathke's pouch during embryogenesis in the region of the pituitary stalk
Lymph Node Drainage:
According to the AJCC the reason that the N in the TNM staging doesn’t work for brain and spinal cord cancers is because the brain and spinal cord do not have lymph nodes.
“An M classification is not pertinent to the majority of neoplasms that affect the central nervous system, because most patients with tumors of the central nervous system do not live long enough to develop metastatic disease (except for some pediatric tumors that tend to “seed” through the cerebrospinal fluid spaces).” This phenomenon of “seeding” is rarely seen adults and is a hallmark for some childhood tumors. The M category of the TNM staging should help “differentiate between extraneural metastasis and metastasis within CNS and CSF pathways.”
“While grossly appearing well encapsulated, craniopharyngiomas typically demonstrate invaginations into adjacent brain and may provoke a vigorous glial reaction.
Histologically benign, these tumors frequently recur after treatment. In addition, because they originate near critical intracranial structures (e.g. visual pathways, pituitary gland, hypothalamus), both the tumor and complications of curative therapy can cause significant morbidity. These characteristics have led to various treatment approaches, and disagreement continues regarding optimal treatment in children with this disease. Evidence suggests that adult craniopharyngiomas are histologically and biologically different from pediatric craniopharyngiomas.
There is no generally applied staging system for childhood craniopharyngiomas. Patients are classified as having newly diagnosed or recurrent disease.
Radiation Side Effects:
The following side effects are possible with irradiation to the brain in children. The presence of these side effects varies due to tumor location, tumor size, and treatment technique/total dose. Late radiation side effects tend to be worse the younger a child is during treatment
-Fatigue, skin irritation (on scalp) and neurologic deterioration can all occur during and shortly after the course of radiation treatment
-Hormone deficiency (growth hormone, thyroid-stimulating hormone, adrenocorticotropic hormone, and gonadotropins) can occur when the pituitary and hypothalamus receive a dose of 20 Gy or more.
-Hair loss (temporary or permanent) can occur with doses of 20-40 Gy and doses greater than 40 Gy, respectively.
-Optic neuropathy and brain necrosis can occur with doses over 60 Gy.
-Secondary malignancies in the bone, soft tissue or bone marrow can occur later in life.
-Late effects from high dose hyperfractionated regimens include: neurocognitive deficits, hearing loss, leukoencephalopathy, diffuse microhemorrhages, and dystrophic calcifications.
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%.
Treatment for craniopharyngioma is controversial.
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.
The target volume for cranipharyngioma is narrowly confined to the tumor volume, including the solid component and cyst.
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.
The following organs have the potential of being in the treatment field depending on tumor size and location.
Brain: 45Gy (whole), 50Gy (2/3), 60Gy (1/3)
Brainstem (large tumors): 50Gy (whole), 53Gy (2/3), 60Gy (1/3)
Ear (acute serous otitis): 30Gy
Ear (chronic serous otitis): 55Gy
Lens: 10 Gy
Optic Chiasm: 50 Gy
Optic Nerve: 50 Gy
Retina: 45 Gy
Craniopharyngioma treatment plan in a pediatric patient.
Chao KS, Perez CA, Brady LW.
Radiation Oncology - Management Decisions.
2nd ed. Philadelphia: Lippincott, Williams & Wilkins, 2002: 623-639.
2. National Cancer Institute. Childhood Craniopharyngioma Treatment (PDQ) Health Professional Version.
. Accessed February 4, 2010.
Washington CM, Leaver D.
Principles and Practice of Radiation Therapy
. 2nd ed. Philadelphia, PA: Mosby, Inc; 2004:736-7, 880-1.
Accessed February 10, 2010.
5. eMedicine. Craniopharyngioma.
. Accessed February 4, 2010.
6. Gunderson and Tepper. Clinical Radiation Oncology. 2nd Edition. Philadelphia, PA: Churchill Livingstone. 2007.
7. Greene et al. Cancer Staging Handbook. 6th Edition. Chicago, Illinois: Eli Lilly and Company. 2001.
Winkfield K., et al. Surveillance of Craniopharyngioma Cyst Growth in Children Treated with Proton Radiotherapy.
Int. J. Radiation Oncol.
2009; 73(3): 716-721.
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