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There is evolving literature on similarities and differences between GIST and EGIST. Despite their behavioural similarities, results cannot be generalised to larger population due to lack of evidence. Pancreatic GIST is a rare entity. There are many documented case reports, however long term data is unavailable. Our case series is by far the largest single centre series with long follow up data but with limited number of cases. Survival values cannot be generalised due to limited data. Large case series are required to further understand the disease biology and long term outcomes of pancreatic GIST.The median tumor size was 6 cm (2-18 cm). Mitotic index of 5 patients were < 5/50 hpf (high power eld) whereas data was not available for 4 patients and 4/8 patients were categorised as high risk as per NIH (National institute of health) risk category. Six patients displayed solid mass on cross-sectional imaging.All patients had spindle morphology on histopathology. Three patients had exon 11 kit mutation on further analysis. Two patients had recurrence with site being liver in both of them. Review of literatureA total of 57 patients were identi ed from 51 published articles. Clinicopathological characteristics of all patients have been summarised in table 1b. All patients belonged to a median age group of 55 years (30-84 years). There were 47% females (n=27) and 45% males (n=26). The most common location was pancreatic head/uncinated process (n=35) followed by body/tail (n=18) with one patient having involvement of entire pancreatic tissue. The majority of patients underwent Whipple procedure or distal pancreaticosplenectomy (DPS) as per tumor location. The median tumor size was 7 cm (0.8 to 35 cm). 18 patients had mitotic count >5/50 hpf and 23 patients =5/50 hpf. While 28 patients were categorised as high risk as per NIH risk strati cation, 11 patients belonged to intermediate and 6 patients to low risk category. 50 biopsy samples were positive for CD 117 while 1 was negative and data was not available for 6 patients. CD 34 was positive for 35 patients and negative for 9 patients while data was missing for 13 patients. 26 patients received adjuvant imatinib while 10 patients didnot receive any adjuvant therapy and adjuvant therapy details were not available for 21 patients. 8 patients had recurrence postoperatively and most common site of recurrence was liver (n=5). Spindle histology was found in 39 patients while 6 had mixed and only 1 patient had epitheloid histology and data was missing for 11 patients. Median follow up time was 24 months.Prognostic factors for DFS according to univariate analysis are summarised in table 2.The calculated median DFS was 74 months (28.6-119.3, 95% CI) with 5 year DFS being 71.9%. [Figure 2] DiscussionThe most frequent sites in GI tract are stomach (40%-70%) followed by small intestine (20-40%), colon and rectum (5%) and esophagus (< 1%) [1, 2] . The term extragastrointestinal stromal tumors (EGIST) was rst used by Reith et al to describe histologically similar stromal tumors ari...
There is evolving literature on similarities and differences between GIST and EGIST. Despite their behavioural similarities, results cannot be generalised to larger population due to lack of evidence. Pancreatic GIST is a rare entity. There are many documented case reports, however long term data is unavailable. Our case series is by far the largest single centre series with long follow up data but with limited number of cases. Survival values cannot be generalised due to limited data. Large case series are required to further understand the disease biology and long term outcomes of pancreatic GIST.The median tumor size was 6 cm (2-18 cm). Mitotic index of 5 patients were < 5/50 hpf (high power eld) whereas data was not available for 4 patients and 4/8 patients were categorised as high risk as per NIH (National institute of health) risk category. Six patients displayed solid mass on cross-sectional imaging.All patients had spindle morphology on histopathology. Three patients had exon 11 kit mutation on further analysis. Two patients had recurrence with site being liver in both of them. Review of literatureA total of 57 patients were identi ed from 51 published articles. Clinicopathological characteristics of all patients have been summarised in table 1b. All patients belonged to a median age group of 55 years (30-84 years). There were 47% females (n=27) and 45% males (n=26). The most common location was pancreatic head/uncinated process (n=35) followed by body/tail (n=18) with one patient having involvement of entire pancreatic tissue. The majority of patients underwent Whipple procedure or distal pancreaticosplenectomy (DPS) as per tumor location. The median tumor size was 7 cm (0.8 to 35 cm). 18 patients had mitotic count >5/50 hpf and 23 patients =5/50 hpf. While 28 patients were categorised as high risk as per NIH risk strati cation, 11 patients belonged to intermediate and 6 patients to low risk category. 50 biopsy samples were positive for CD 117 while 1 was negative and data was not available for 6 patients. CD 34 was positive for 35 patients and negative for 9 patients while data was missing for 13 patients. 26 patients received adjuvant imatinib while 10 patients didnot receive any adjuvant therapy and adjuvant therapy details were not available for 21 patients. 8 patients had recurrence postoperatively and most common site of recurrence was liver (n=5). Spindle histology was found in 39 patients while 6 had mixed and only 1 patient had epitheloid histology and data was missing for 11 patients. Median follow up time was 24 months.Prognostic factors for DFS according to univariate analysis are summarised in table 2.The calculated median DFS was 74 months (28.6-119.3, 95% CI) with 5 year DFS being 71.9%. [Figure 2] DiscussionThe most frequent sites in GI tract are stomach (40%-70%) followed by small intestine (20-40%), colon and rectum (5%) and esophagus (< 1%) [1, 2] . The term extragastrointestinal stromal tumors (EGIST) was rst used by Reith et al to describe histologically similar stromal tumors ari...
BACKGROUND Extragastrointestinal stromal tumors (EGIST) and gastrointestinal stromal tumors are of similar pathological type and form. Here we report a rare case of EGIST diffusely distributed in membranous tissue in abdominal cavity, the feature of which included diffuse tumors at membranous tissue in entire abdominal cavity and spontaneous bleeding of the tumors. CASE SUMMARY The patient was a 71-year man and hospitalized due to continuous pain at lower abdomen for more than 10 days. Upon physical examination, the patient had flat and tough abdomen with mild pressing pain at lower abdomen, no obvious abdominal mass was touchable, and shifting dullness was positive. Positron emission tomography-computed tomography (CT) showed that in his peritoneal cavity, there were multiple nodules of various sizes, seroperitoneum, multiple enlarged lymph nodes in abdominal/pelvic cavity and right external ilium as well as pulmonary nodules. Plain CT scanning at epigastrium/hypogastrium/pelvic cavity + enhanced three-dimensional reconstruction revealed multiple soft tissue nodules in abdominal/pelvic cavity, peritoneum and right groin. Tumor marker of carbohydrate antigen 125 was 808 U/mL, diffuse tuberous tumor was seen in abdominal/pelvic cavity during operation with hematocelia, and postoperative pathological examination confirmed EGIST. Imatinib was administered with better therapeutic effect. CONCLUSION Gene testing showed breast cancer susceptibility gene 1 interacting protein C-terminal helicase 1 and KIT genovariation, and the patient was treated with imatinib follow-up visit found that his clinical symptoms disappeared and the tumor load alleviated obviously via imageological examination.
There is evolving literature on similarities and differences between GIST and EGIST. Despite their behavioural similarities, results cannot be generalised to larger population due to lack of evidence. Pancreatic GIST is a rare entity. There are many documented case reports, however long term data is unavailable. Our case series is by far the largest single centre series with long follow up data but with limited number of cases. Survival values cannot be generalised due to limited data. Large case series are required to further understand the disease biology and long term outcomes of pancreatic GIST.
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