A computed tomography (CT) scan is also a good diagnostic tool, as it detects calcification in the tumor.Two distinct types are recognized:
Adamantinomatous craniopharyngiomas, which resemble ameloblastomas (the most common type of odontogenic tumor), are characterized by activating CTNNB1 mutations. Papillary craniopharyngiomas are characterized by BRAFv600E mutations.In the adamantinomatous type, calcifications are visible on neuroimaging and are helpful in diagnosis. The papillary type rarely calcifies. A vast majority of craniopharyngiomas in children are adamantiomatous, whereas both subtypes are common in adults. Mixed-type tumors also occur.On macroscopic examination, craniopharyngiomas are cystic or partially cystic with solid areas. On light microscopy, the cysts are seen to be lined by stratified squamous epithelium. Keratin pearls may also be seen. The cysts are usually filled with a yellow, viscous fluid rich in cholesterol crystals. Of a long list of possible symptoms, the most common presentations include headaches, growth failure, and bitemporal hemianopsia. Malignant craniopharyngioma
Craniopharyngiomas are usually successfully managed with a combination of adjuvant chemotherapy and neurosurgery. Recent research describes the rare occurrence of malignant transformations of these normally benign tumors.
Because all three are involved in forming the hexameric glycoprotein fibrinogen, mutations in any one of the three genes can cause the deficiency. Diagnosis
Treatment
The most common type of treatment is cryoprecipitate or fibrinogen concentrate drip to increase fibrinogen levels to normal during surgical procedures or after trauma. RiaSTAP, a factor I concentrate, was approved by the U.S. FDA in 2009 for use when the fibrinogen level was below 50 mg/deciliter of plasma. Recently, antifibrinolytics have also been used to inhibit fibrinolysis (breaking down of the fibrin clot). In the case of dysfibrinogenemia that manifests by thrombosis, anticoagulants can be used. Due to the inhibited clotting ability associated with a- and hypofibrinogenemia, physicians advise against the use of Aspirin as it inhibits platelet function. See also
Congenital afibrinogenemia
Hypodysfibrinogenemia

References


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