gastrointestinal stromal tumors ( gists ) are the most common mesenchymal tumors of the gastrointestinal tract .
gists originate from the interstitial cells of cajal ( icc ) or their stem cell - like precursors [ 1 , 2 ] . most gists express a receptor tyrosine kinase , kit .
kit is encoded by the c - kit gene and its ligand is stem cell factor ( scf ) .
gists frequently have a mutation of the c - kit gene which results in constitutive activation of kit without stimulation by scf .
the median ages in the largest series of gists of different locations have ranged between 55 and 65 years .
the malignant potential of gists varies from virtually benign to aggressive tumors . of all gists ,
the stomach and small intestine are the most commonly affected anatomic sites , accounting for about two thirds and one third of cases , respectively .
gist is considered to be a solitary tumor and the occurrence of multiple primary lesions is very rare , restricted to familial gists , pediatric forms , or district syndromes such as type 1 neurofibromatosis ( nf1 ) or von recklinghausen disease   or carney 's syndrome .
all these are well - defined entities that can be easily distinguished from common sporadic gists based on their peculiar clinicopathological features .
multiple sporadic gists in different organs are rare [ 8 , 9 , 10 ] .
this report presents a surgical case of sporadic synchronous gists in the stomach and jejunum .
a 71-year - old japanese male presented with a 2-year history of occasional melena and general fatigue . computed tomography of the abdomen demonstrated an enhanced extramural tumor of the stomach , 4 cm in diameter ( fig .
an upper gastrointestinal series revealed a submucosal jejunal tumor near the treitz ligament , 2.5 cm in diameter ( data not shown ) .
the patient was referred to the surgical outpatient clinic for surgical treatment of an extramural tumor of the stomach and a submucosal tumor of the jejunum .
physical examination revealed no conjunctival pallor , and there was no muscular rigidity or rebound tenderness in response to abdominal palpation .
laboratory investigations revealed a white blood cell count of 3,200/mm , a hemoglobin of 8.3 g / dl with a hematocrit of 25.8% , 321,000/mm platelets , normal electrolytes , as well as normal blood urea nitrogen levels and normal liver function .
cea was 1.0 ng / ml and ca19 - 9 was 6.0 u / ml . laparotomy revealed a nut - sized extramural tumor at the lesser curvature of the middle stomach ( fig .
2a ) that was white - yellow , elastic soft and had dilated vessels on the surface .
a thumb 's head - sized tumor was also identified on the jejunum about 15 cm from the anal side of the treitz ligament ( fig .
histopathological examination with hematoxylin and eosin staining revealed that the gastric tumor was composed of a proliferation of spindle cells with spindle nuclei and eosinophilic fibrillar cytoplasm , arranged in fascicles with a hypercellular area ( fig .
3a ) , and the jejunal tumor contained a proliferation of spindle cells with oval or spindle nuclei and occasional pleomorphic cells , arranged in a fascicular or haphazard fashion , alternating with areas of epithelioid features ( fig .
both gists were classified as low - risk , according to the nih risk classification of gist .
the patient had an uneventful recovery and was discharged from the hospital on the 15th day after the operation and followed up in the outpatient clinic .
the occurrence of multiple gists is an extraordinarily rare event restricted to familial gists , pediatric forms , or district syndromes such as nf1   or carney 's syndrome .
the presentation of multiple synchronous lesions is commonly viewed as the result of the metastatic spreading of a single primary gist .
the tumors of metastatic gist are generally located on the peritoneum , but not in the other portions of the gastrointestinal tract . moreover , the patterns of immunohistochemical and molecular analysis in metastatic gist are consistent with paired tumors . on the other hand ,
some reports indicate that the patterns of immunohistochemical and molecular analysis in multiple sporadic gists are often different [ 8 , 9 , 10 , 12 ] .
the immunohistochemical analysis in the present case revealed different patterns in the gastric and jejunal gists , though no molecular analysis was performed .
therefore , this case was diagnosed as synchronous sporadic gists in the stomach and jejunum .
familial gists patients have a germline mutation in the kit gene and diffuse hyperplasia of icc with direct continuity between the gist mass and the adjacent intestinal wall .
multiple gists in nf1 patients are caused by a mutation of the nf1 gene and have focal or mild hyperplasia of icc .
on the other hand , no hyperplasia of icc is noted in patients with multiple sporadic gists . moreover , the normal control mucosa of multiple sporadic gists retains the wild - type kit gene .
this finding supports the finding that the present case was synchronous sporadic gists in the stomach and jejunum .
complete gross resection of gists with negative margins is the standard primary treatment [ 10 , 11 , 12 , 13 ] .
the japanese clinical practice guidelines for gist recommend resection if the submucosal tumor is larger than 2 cm with clinically malignant signs , whereas observation may be chosen for a tumor smaller than 2 cm without symptoms or clinically malignant signs .
nevertheless , tumors diagnosed as gists should be resected regardless of size because of their malignant potential .
recently , the laparoscopic approach is considered effective and technically feasible when performed by skilled surgeons [ 10 , 11 , 12 , 13 , 15 ] .
laparoscopic wedge resection enables most small ( < 5 cm ) tumors to be removed with clear margins .
however , the laparoscopic approach was not performed in the present case because he was treated 12 years ago . in conclusion
, this report presents the surgical case of a patient with synchronous sporadic gastric and jejunal gists .
 this report describes a patient with synchronous sporadic gastrointestinal stromal tumors ( gists ) in the stomach and jejunum . a 71-year - old japanese male presented with a 2-year history of occasional melena and general fatigue . 
 computed tomography of the abdomen demonstrated an enhanced extramural gastric tumor , 4 cm in diameter . 
 endoscopic examination revealed a jejunal submucosal tumor . 
 he was referred to the surgical outpatient clinic for surgical treatment of an extramural gastric tumor and a jejunal submucosal tumor . 
 laparotomy allowed the identification of a nut - sized extramural tumor at the stomach and a thumb 's head - sized tumor on the jejunum . 
 partial resections of the stomach and jejunum were performed . 
 histopathological and immunohistochemical examination revealed that these tumors were gists . 
 although no molecular analysis was performed , the immunohistochemical staining patterns of these two tumors were different from each other . 
 therefore , the final diagnosis was synchronous sporadic gists in the stomach and jejunum 
 . this patient has survived without recurrence for approximately 12 years since complete resection . 
Introduction
Case Report
Discussion
Disclosure Statement