Although gastrointestinal stromal tumor(GIST) occurs generally in the digestive tract,omental GIST is very rare.We report the first case of an adult greater omental GIST with a new platelet-derived growth factor recep...Although gastrointestinal stromal tumor(GIST) occurs generally in the digestive tract,omental GIST is very rare.We report the first case of an adult greater omental GIST with a new platelet-derived growth factor receptor α gene(PDGFRA)-mutation with hemoperitoneum.A 43-year-old man was admitted to our hospital complaining of acute abdominal pain.Abdominal contrast-enhanced computed tomography revealed a huge mass in the right abdominal cavity,and a large accumulation of fluid in the pelvic cavity,suggesting hemoperitoneum.We diagnosed the rupture as an intra-abdominal tumor,and an emergency tumorectomy was performed with resection of the greater omentum.This tumor was located in the distal right side ofthe greater omentum,and showed no continuity with the gastric wall.The tumor occurred primarily in the greater omentum.The resected tumor was about 19 cm × 12 cm × 14 cm in diameter,and weighed 1529 g.Histologically,the tumor was composed of epithelioidshaped cells with high cellularity,and was positive for CD117 and CD34,and negative for S-100,α-smooth muscle actin.The mitosis was 6/50 under high power field.This case showed exon 18 mutation of PDGFRA with 846(Asp to Glu) substitution,848(Asn to Lys) substitution.This is the first report of this PDGFRA mutation in omental GIST,and this might play an important role in the tumorigenesis of this case.Based on these findings,the tumor was diagnosed as high risk GIST primarily occurring in the greater omentum.The patient was treated with imatinib at a dose of 400 mg/d as adjuvant chemotherapy,and has been followed up for 24 mo with no evidence of recurrence.展开更多
Dear editor,Lung carcinoma is responsible for the highest fatal-ity rate among cancer-related deaths globally,with lung adenocarcinoma(LADC)emerging as the prevailing sub-type.
文摘Although gastrointestinal stromal tumor(GIST) occurs generally in the digestive tract,omental GIST is very rare.We report the first case of an adult greater omental GIST with a new platelet-derived growth factor receptor α gene(PDGFRA)-mutation with hemoperitoneum.A 43-year-old man was admitted to our hospital complaining of acute abdominal pain.Abdominal contrast-enhanced computed tomography revealed a huge mass in the right abdominal cavity,and a large accumulation of fluid in the pelvic cavity,suggesting hemoperitoneum.We diagnosed the rupture as an intra-abdominal tumor,and an emergency tumorectomy was performed with resection of the greater omentum.This tumor was located in the distal right side ofthe greater omentum,and showed no continuity with the gastric wall.The tumor occurred primarily in the greater omentum.The resected tumor was about 19 cm × 12 cm × 14 cm in diameter,and weighed 1529 g.Histologically,the tumor was composed of epithelioidshaped cells with high cellularity,and was positive for CD117 and CD34,and negative for S-100,α-smooth muscle actin.The mitosis was 6/50 under high power field.This case showed exon 18 mutation of PDGFRA with 846(Asp to Glu) substitution,848(Asn to Lys) substitution.This is the first report of this PDGFRA mutation in omental GIST,and this might play an important role in the tumorigenesis of this case.Based on these findings,the tumor was diagnosed as high risk GIST primarily occurring in the greater omentum.The patient was treated with imatinib at a dose of 400 mg/d as adjuvant chemotherapy,and has been followed up for 24 mo with no evidence of recurrence.
基金This research was supported in part by the Japan Agency for Medical Research and Development(AMED)(JP15ck0106096 to TK)Japan Science and Tech-nology Agency(JST)Core Research for Evolutionary Science and Technology(JPMJCR1689 to RH)+5 种基金Artifi-cial Intelligence,Big Data,IoT,Cyber Security Integration Project of the Public/Private R&D Investment Strategic Expansion Program(JPMJCR18Y4 to RH)the Japan Soci-ety for the Promotion of Science(JSPS)Grant-in-Aid for Scientific Research(S)(17H06162 to HN),Grant-in-Aid for Scientific Research(B)(20H03695 to KS),Grants-in-Aid for the Tailor-Made Medical Treatment Program(BioBank Japan Project)from the Japanese Ministry of Education,Culture,Sports,ScienceandTechnology(MEXT),Princess Takamatsu Cancer Research Fund,and National Cancer Center Research and Development Fund(NCC Biobank and NCC Core Facility).The J-MICC study was supported by Grants-in-Aid for Scientific Research for Priority Areas of Cancer(No.17015018 to KW)Innovative Areas(No.221S0001 to KW)from MEXTby JSPS Grant-in-Aid for Scientific Research Grant(No.16H06277[CoBiA])The JPHC Study was supported by National Cancer Center Research and Development Fund since 2011(latest grant number:2020-J4)and a Grant-in-Aid for Cancer Research from the Ministry of Health,Labor and Welfare of Japan(1989-2010).ToMMoissupportedinpartbyMEXT-JSTand AMED(most recent grant numbers:JP20km0105001 and JP20km0105002)Iwate Tohoku Medical Megabank Orga-nization(Iwate Medical University)is supported in part by MEXT-JST and AMED(most recent grant numbers:JP20km0105003 and JP20km0105004).
文摘Dear editor,Lung carcinoma is responsible for the highest fatal-ity rate among cancer-related deaths globally,with lung adenocarcinoma(LADC)emerging as the prevailing sub-type.