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- 1. Pediatric rotation-Al Maqased Hospital This presentation was prepared by: LAYTH HELWA Craniopharyngioma AUTUMN-2014
- 2. Craniopharyngiomas are dysontogenic tumors with benign histology and malignant behavior.
- 4. Epidemiology 1- The estimated incidence is 1.4 cases per million children per year. 2- In children, craniopharyngioma represents 5-10% of all intracranial tumors . 3- A slight male predominance exists in all age groups (55%). 4- Craniopharyngiomas have a bimodal age distribution pattern, with a peak between ages 5 and 14 years and in adults older than 65 years, although there reports involving all age groups.
- 5. History and Physical Examination The most common presenting symptoms are headache (55-86%). endocrine dysfunction (66-90%) visual disturbances (37-68%). Neurologic examination General examination Hypothyroidsm-40 % -Puffiness and non-pitting edema -Slow return phase of deep tendon reflexes -Hypoventilation and decrease in cardiac output -Pericardial and pleural effusions -Constipation -Psychiatric change Adrenal insufficiency 25% -Hypovolemia. -Decreased cardiac output. -Fatigue. -Cardiac arrhythmias due to hyperkalemia
- 6. Imaging The characteristic imaging finding of craniopharyngioma in a child is an enhancing suprasellar mass that is calcified and cystic on CT. When two out of these three features are present, craniopharyngioma is still the most likely diagnosis. The tumor usually demonstrates T1 high intensity on MR, reflecting the protein or cholesterol content of the âmotor oil-likeâ fluid found in the tumor cysts . Rarely on plain skull film, craniopharyngioma may be suspected by the presence of sellar or suprasellar calcifications.
- 9. Treatment Essentially, 2 main management options are available for craniopharyngiomas: (1) attempt at gross total resection or (2) planned limited surgery followed by radiotherapy. Inflammatory cytokines and biomodulation
- 10. Neurosurg 119:106â112, 2013 106 J Neurosurg / Volume 119 / July 2013 ©AANS, 2013 Molecular oncogenesis of craniopharyngioma: current and future strategies for the development of targeted therapies.
Hinweis der Redaktion
- Nesting squamous cells arising from remenent of rathkeâs pouch This theory relates to development of the adenohypophysis and transformation of the remnant ectoblastic cells of the craniopharyngeal duct and the involuted Rathke pouch. The Rathke pouch and the infundibulum develop during the fourth week of gestation and together form the hypophysis. Both elongate and come in contact during the second month. The infundibulum is a downward invagination of diencephalon; the Rathke pouch is an upward invagination of the primitive oral cavity (ie, stomodeum). The craniopharyngeal duct is the neck of the pouch, connecting to the stomodeum, which narrows, closes, and separates the pouch from the primitive oral cavity by the end of the second month. Thus, the pouch becomes a vesicle, which flattens and surrounds the anterior and lateral surfaces of the infundibulum. Walls of this vesicle form different structures of the hypophysis. Finally, this vesicle involutes into a mere cleft and may disappear completely. The Rathke cleft, together with remnants of the craniopharyngeal duct, can be the site of origin of craniopharyngiomas Craniopharyngiomas are typically very slow growing tumors. They arise from the cells along the pituitary stalk, specifically from nests of odontogenic (tooth-forming) epithelium within the suprasellar/diencephalic region and, therefore, contain deposits of calcium, which are evident on an x-ray. They are classified by histology as benign GarrĂš ML, Cama A (2007). "Craniopharyngioma: modern concepts in pathogenesis and treatment". Curr. Opin. Pediatr. 19 (4): 471â9.
- Craniopharyngioma typically is a slow-growing tumor. Symptoms frequently develop insidiously and usually become obvious only after the tumor attains a diameter of about 3cm. The time interval between the onset of symptoms and diagnosis usually ranges from 1-2 years. On presentation, 40% of patients have symptoms related to hypothyroidism (eg, weight gain, fatigue, cold intolerance, constipation). Almost 25% have associated signs and symptoms of adrenal failure (eg, orthostatic hypotension, hypoglycemia, hyperkalemia, cardiac arrhythmias, lethargy, confusion, anorexia, nausea and vomiting), and 20% have diabetes insipidus (eg, excessive fluid intake and
- Recurrences usually occur at the primary site. Ectopic and metastatic recurrences are extremely rare but have been reported after surgical removal. The 2 possible mechanisms of seeding are dissemination of tumor cells along the surgical paths during the procedure and migration of tumor cells through the subarachnoid space or Virchow-Robin spaces, which explains ectopic recurrences distant from the surgical bed and within brain parenchyma). In one metastatic case, after removal of a suprasellar (adamantinomatous) craniopharyngioma, 2 peripheral lesions were identified 7 years later, adjacent to the dura and contralateral to the initial craniotomy site. They proved to be composed of adamantinomatous tissue, raising the possibility of meningeal seeding. In another reported case, an adamantinomatous craniopharyngioma recurred at different intervals and at different sites, along the operative track of the initial surgical procedure as well as a distant site within the brain parenchyma, suggesting that both seeding mechanisms were involved in these recurrences.
- Axial Non enhanced CT (NECT) scan of brain (KV: 120, MAS: 60) of a 22-year-old woman male with intrinsic third ventricular craniopharyngioma At the level of thalamus revealed midline relatively large homogenous, hyperdense mass in third ventricle with subtle periventricular hypodensity (arrow) due to interstitial edema. Dilatation of frontal horns is due to obstructive hydrocephaly
- Bleomycin acts by induction of DNA strand breaks
- The Wnt/b-catenin signaling pathway in craniopharyngioma cells. Left: In the absence of Wnt, adenomatous polyposis coli (APC), axin, and glycogen synthase kinaseâ3b (GSK-3b) form a complex that functions to phosphorylate intracytoplasmic b-catenin. Phosphorylation of b-catenin subsequently leads to its proteasome-mediated degradation. Right: When Wnt binds to a receptor complex composed of Frizzled (Fz) and low-density lipoprotein receptorârelated protein (LRP), Dishevelled (DSH) is recruited to this complex, where it binds with axin, preventing the APC/axin/GSK-3b phosphorylation complex from forming. The subsequent accumulation of b-catenin in the cytoplasm leads to an increase in the translocation of b-catenin to the nucleus, where it binds with transcription factors that regulate cell mobility, angiogenesis, and proliferation. A mutated, degradation-resistant form of b-catenin is implicated as the primary driver of oncogenesis in craniopharyngioma cells.