Metastatic tumors: Metastatic GISTs are treated with targeted therapy, and mutation testing is very important to determine the best options.After a period of time, however, these tumors may shrink to a degree that surgery is then possible. Unresectable tumors: Tumors that cannot be removed with surgery for some reason are treated as metastatic GIST.For tumors that have a high risk of recurrence, targeted therapy is usually recommended following surgery.
This is primarily an option for people who have GISTs that are less than 1 to 2 centimeters in diameter, and when the tumor is found incidentally when surgery is performed for another reason. Most tumors, however, have larger than this at the time of diagnosis. Very small, Incidental tumors: Watchful waiting or active surveillance (carefully monitoring a tumor but holding off on treatment) may be considered with some very small GISTs.Primary familial GIST syndrome: While most GISTs harboring a KIT mutation are sporadic, there are some families who carry a hereditary mutation (autosomal dominant), frequently developing GISTs in late childhood.(A syndrome called Carney triad syndrome is similar, but instead of hereditary mutations in the genes is related to epigenetic changes in the genes.) Carney-Stratakis syndrome: Mutations in succinate dehydrogenase genes (SDHA, SDHB, SDHC, and SDHD) are associated with GIST as well as paragangliomas or pheochromocytoma.
#Grapefruit size tumor on small intestine skin#
People with this mutation often develop multiple GISTs along with tumors around nerves (neurofibromas) and multiple brown patches of skin (cafe au last spots). Tumors associated with NF1 are found primarily in the stomach (60%) and small intestine (25%), and it's estimated that 3.9% to 25% of people with neurofibromatosis type 1 will develop a GIST at some point in their lifetime.
Type-1 neurofibromatosis: This condition is associated with a mutation in the NF1 gene.
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