Nesting squamous cells arising from remenent of rathke’s pouchThis 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
Garrè ML, Cama A (2007). "Craniopharyngioma: modern concepts in pathogenesis and treatment". Curr. Opin. Pediatr. 19 (4): 471–9.
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 kinase–3b (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 receptor–related 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.
Pediatric rotation-Al Maqased Hospital
This presentation was prepared by:
Craniopharyngiomas are dysontogenic tumors with benign histology and malignant
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
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.
History and Physical Examination
The most common presenting symptoms are
endocrine dysfunction (66-90%)
visual disturbances (37-68%).
-Puffiness and non-pitting edema
-Slow return phase of deep tendon reflexes
-Hypoventilation and decrease in cardiac output
-Pericardial and pleural effusions
Adrenal insufficiency 25%
-Decreased cardiac output.
-Cardiac arrhythmias due to
The characteristic imaging finding of
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
The tumor usually demonstrates T1 high
intensity on MR, reflecting the protein or
cholesterol content of the
“motor oil-like” fluid found in the tumor cysts .
on plain skull film, craniopharyngioma may be
suspected by the presence of sellar or
Essentially, 2 main management options are
available for craniopharyngiomas:
(1) attempt at gross total resection
(2) planned limited surgery followed by
Inflammatory cytokines and biomodulation