OBJECTIVES: There is currently no consensus on whether extra-gastrointestinal stromal tumors (EGISTs) and gastrointestinal stromal tumors (GISTs) are the same type of tumor, and whether the diagnosis and treatment of EGISTs can directly replicate the current diagnostic and treatment standards for GISTs. This study aims to further elucidate the clinical and pathological characteristics, diagnosis, treatment, and prognosis of EGISTs by analyzing the research results of domestic scholars in the field of EGISTs in the past decade. METHODS: A review was conducted on original Chinese and English research articles published from 2013 to 2022 focusing on EGISTs. A descriptive approach was used to extract key information from the literature, including patient demographics, tumor location, tumor diameter, mitotic figures, risk stratification, immunohistochemical markers, cell type, and prognostic factors. The data were subjected to statistical analysis. RESULTS: A total of 12 articles containing 780 EGIST patients were included. The male-to-female incidence of EGISTs was 0.92꞉1. The most common sites of EGISTs were mesentery (30.96%), peritoneum or retroperitoneum (28.53%), omentum (20.32%), and pelvic cavity (12.52%). 52.77% of EGISTs had tumor diameters greater than 10 cm, and the proportions of EGISTs with nuclear fission patterns greater than 5/50 high power field (HPF) and greater than 10/50 HPF were 51.24% and 26.11%, respectively. The proportion of high-risk EGISTs was 79.05%. The positive rates of immune markers CD117, CD34, and DOG-1 in EGISTs were 82.3%, 69.0%, and 79.5%, respectively. The proportion of Ki-67 >5% was 49.2%, and the proportion of Ki-67 >10% was 24.8%. The proportions of EGISTs in spindle cells, epithelial cells, and mixed cells were 74.4%, 14.8%, and 13.1%, respectively. The diameter of the tumor, resection method, risk level, Ki-67 index, mitotic counts, presence of rupture/bleeding/necrosis/peripheral tissue invasion/recurrence and metastasis, as well as the use of imatinib treatment after surgery were important factors affecting the prognosis of EGISTs. CONCLUSIONS Current medical research is relatively well cognizant of GISTs with primary sites in the gastrointestinal tract. Compared with GISTs, EGISTs have large tumor diameters, high mitotic counts, a high percentage of high-risk grades, relatively unique molecular expression, and high aggressiveness. EGISTs differ from GISTs in clinicopathological characteristics. Whether EGISTs and GISTs share a common origin remains unclear. If they are distinct tumor entities, separate diagnostic and treatment guidelines for EGISTs should be established. If EGISTs are ultimately confirmed to be a special subtype of GISTs, then directly applying existing GIST-based standards to EGISTs may be inappropriate. A more scientific approach would involve subclassifying EGISTs based on anatomical location and then tailoring treatment strategies accordingly with reference to GIST guidelines.
Humans ; Gastrointestinal Stromal Tumors/epidemiology* ; Male ; Female ; Prognosis ; Proto-Oncogene Proteins c-kit/metabolism*

To explore the differences in biological characteristics for the small gastrointestinal stromal tumors and the incidence of complications and recurrence between the traditional surgical treatment and endoscopic treatment.
 Methods: We collected the relevant clinical and pathological data from patients who were diagnosed as gastrointestinal stromal tumors with the diameter less than 2 cm by the Department of Pathology of Xiangya Hospital from January 2009 to December 2015. The complications and recurrence after the surgical treatment were analyzed.
 Results: In patients with small gastrointestinal stromal tumors, the proportion of female was higher than that of male (male:female=1:1.69). The median age for patient with this disease was 49 years old and it was more common in middle-aged and elderly. Most lesions were found in the stomach, followed by the esophagus and the small intestine. The small gastrointestinal stromal tumors occurred in the colon and rectum were rare. There was 60.3% (47/78) patients with abdominal pain, 7.7% (6/78) patients with hematochezia or melena, and 98.7% (78/79) with small gastrointestinal stromal tumors' mitotic count ≤5/50 HPF. The positive rates for CD, CD34, DOG-1, actin-smooth, and S-100 were 98.7%, 86.1%, 82.3%, 31.6%, and 24.1%, respectively. Three patients occurred surgical complications, 2 suffered recurrence during the follow-up. There was no significant difference in the incidence of complications and recurrence between the traditional surgical treatment and endoscopic treatment (P>0.05).
 Conclusion: Small gastrointestinal stromal tumors' malignant potential is low, and the recurrence and metastasis rate is low. Its biological behavior tends to be benign. The traditional surgical treatment and endoscopic treatment are both safe and effective for small gastrointestinal stromal tumor. Endoscopic treatment has the advantages in lower cost, shorter hospitalization time, and small trauma. Therefore, endoscopic treatment could be the first choice for small GIST resection under the condition of mature endoscopic technology.
Aged ; Endoscopy, Gastrointestinal ; Female ; Gastrointestinal Hemorrhage ; etiology ; Gastrointestinal Neoplasms ; complications ; pathology ; surgery ; Gastrointestinal Stromal Tumors ; complications ; pathology ; surgery ; Humans ; Incidence ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Postoperative Complications ; epidemiology ; Retrospective Studies ; Tumor Burden

Primary malignant small bowel tumor as a rare kind of intestinal tumor is associated with a poor prognosis. The pathological types were various and complicated, such as adenocarcinoma, neuroendocrine tumor, malignant lymphoma, and malignant stromal tumor. The atypical early stage symptom resulted in difficult diagnosis at early stage, high misdiagnosis rate and lack of standard therapy schemes and means. In the past, X-ray, CT, MRI, and PET-CT were the main examination methods for primary small bowel tumor. However, with the development of radiology, a series of new diagnosis methods, including electronic enteroscopy, capsule endoscopy, multi-slice spiral CT enteroclysis and so on, promotes the diagnosis accurate rate. Surgery is still the most important method in the small bowel tumor treatment, and the alternative of the surgical method should depend on the tumor location, size and relationship with the adjacent organs. Application of the laparoscopic surgery for the small bowel tumor is still in the initial stage. Besides, some researches have confirmed that chemotherapy, radiotherapy, target therapy and endocrinotherapy have effects on the specific kind of small bowel tumor. Therefore this article will review the epidemiology, pathology, diagnosis and treatment of the primary malignant small bowel tumors.
Adenocarcinoma ; diagnosis ; epidemiology ; pathology ; therapy ; Capsule Endoscopy ; Gastrointestinal Stromal Tumors ; diagnosis ; epidemiology ; pathology ; therapy ; Humans ; Intestinal Neoplasms ; diagnosis ; epidemiology ; pathology ; therapy ; Intestine, Small ; diagnostic imaging ; pathology ; Laparoscopy ; methods ; Lymphoma ; diagnosis ; epidemiology ; pathology ; therapy ; Neuroendocrine Tumors ; diagnosis ; epidemiology ; pathology ; therapy ; Tomography, Spiral Computed

Gastrointestinal Stromal Tumors ; diagnosis ; epidemiology ; surgery ; Humans ; Stomach ; pathology

Gastrointestinal stromal tumor (GISTs) are a new recognized tumor entity. The incidence increases with time for the greater awareness of GISTs. Latest reports of the epidemiology of GISTs have been collected and sorted. Besides, it is still a controversy on the management of GISTs. While surgical en-bloc resection is the treatment of choice, imatinib is still optimal for patients with recurrent or metastatic GISTs. Preliminary promising effects have been showed on neo-adjuvant and post-operational adjuvant therapies. Imatinib-resistant GISTs become common and more targeted medication have been investigated and tested.
Gastrointestinal Stromal Tumors ; epidemiology ; therapy ; Humans ; Incidence

Seven hundred forty seven cases of gastrointestinal stromal tumors (GISTs) in Koreans who were diagnosed between 2001 and 2002 were analyzed to evaluate their occurrence and their clinical, pathologic and immunohistochemical findings. The most frequent location of tumor was in the stomach (63%), followed by the small intestine (30%), the colorectum (5%), and the esophagus (2%). c-kit expression was found in 93.6% of the cases, while CD34, SMA and S-100 protein was positive in 80.1%, 28.2%, and 20.2%, respectively. c-kit positivity was high in the stomach (94.2%) and small intestine (94.6%), while it was relatively low in the colorectum (85.0%), and esophagus (81.2%). The positivity for CD34 was correlated with the higher risk of GISTs (p=0.04). Follow up of the patients showed that 58 primary GISTs patients died and 20 of these patients were recurrent or metastatic at the time of diagnosis. The pathologic diagnosis to predict the risk of aggressive behavior of GISTs was correlated with the numbers of tumor, clinical stage, epithelioid histologic type, cellularity, cellular atypia, necrosis, and mucosal invasion (p= 0.00). GISTs with a poor prognosis were closely related to the clinical stage at presentation, the locations of the tumor, and the ages of the patients.
Actins/metabolism ; Adolescent ; Adult ; Aged ; Aged, 80 and over ; Antigens, CD34/metabolism ; Child ; Female ; Gastrointestinal Stromal Tumors/*epidemiology/metabolism/pathology ; Humans ; Immunohistochemistry ; Korea/epidemiology ; Male ; Middle Aged ; Prognosis ; Proto-Oncogene Proteins c-kit/metabolism ; Research Support, Non-U.S. Gov't ; S100 Proteins/metabolism