Image showing<unk>  after surgical removal For localized, resectable adult<unk> s, if anatomically and physiologically feasible, surgery is the primary treatment of choice. Surgery can be potentially curative, but watchful waiting may be considered in small<unk> s in carefully selected situations. Post-surgical adjuvant treatment may be recommended. Lymph node metastases are rare, and routine removal of lymph nodes is typically not necessary. Laparoscopic surgery, a minimally invasive abdominal surgery using telescopes and specialized instruments, has been shown to be effective for removal of these<unk> s without needing large incisions. The clinical issues of exact surgical indications for<unk>  size are controversial. The decision of appropriate laparoscopic surgery is affected by<unk>  size, location, and growth pattern. Radiotherapy has not historically been effective for<unk> s and<unk> s do not respond to most chemotherapy medications, with responses in less than 5%. However, three medications have been identified for clinical benefit in<unk> : imatinib, sunitinib, and regorafenib. Imatinib (Glivec/Gleevec), an orally administered drug initially marketed for chronic myelogenous leukemia based on bcr-abl inhibition, also inhibits both c-kit tyrosine kinase mutations and PDGFRA mutations other than D842V, is useful in treating<unk> s in several situations. Imatinib has been used in selected neoadjuvant settings. In the adjuvant treatment setting, the majority of<unk><unk> s are cured by surgery, and do not need adjuvant therapy. An exception to this is where the anatomical position of the<unk> r means that surgery is technically difficult or complex. For example, rectal<unk>  often requires radical surgery to achieve complete resection, involving abdominoperineal resection and permanent stoma. In these situations, the use of neoadjuvant imatinib can significantly decrease both<unk> r size and mitotic activity, and permit less radical sphincter-preserving surgery. A substantial proportion of<unk><unk> s have a high risk of recurrence as estimated by a number of validated risk stratification schemes, and can be considered for adjuvant therapy. The selection criteria underpinning the decision for possible use of imatinib in these settings include a risk assessment based on pathological factors such as<unk>  size, mitotic rate, and location can be used to predict the risk of recurrence in<unk>  patients.<unk> s <2 cm with a mitotic rate of <5/50 HPF have been shown to have lower risk of recurrence than larger or more aggressive<unk> s. Following surgical resection of<unk> s, adjuvant treatment with imatinib reduces the risk of disease recurrence in higher risk groups. In selected higher risk adjuvant situations, imatinib is recommended for 3 years. Imatinib was approved for metastatic and unresectable<unk>  by the US FDA, February 1, 2002. The two-year survival of patients with advanced disease has risen to 75–80% following imatinib treatment. If resistance to imatinib is encountered, the multiple tyrosine kinase inhibitor sunitinib (marketed as Sutent) can be considered. The effectiveness of imatinib and sunitinib depend on the genotype. cKIT- and PDGFRA-mutation negative<unk><unk> s are usually resistant to treatment with imatinib as is neurofibromatosis-1-associated wild-type<unk> . A specific subtype of PDGFRA-mutation, D842V, is also insensitive to imatinib. Recently, in PDGFRA mutated<unk> , Avapritinib has been approved by FDA. Now there is real world data coming for avapritinib as well (Dr Sameer Rastogi et al) Regorafenib (Stivarga) was FDA approved in 2013 for advanced<unk> s that cannot be surgically removed and that no longer respond to imatinib (Gleevec) and sunitinib (Sutent).
Gastrointestinal stromal tumor
Ectopic pancreas
Krukenberg tumor
Signet ring cell carcinoma
Lipoblastoma
Nesidioblastoma
Angiomyofibroblastoma
Myxoid cyst