Objective: To analyze the clinicopathological characteristics and prognosis of patients with small bowel tumors. Methods: This was a retrospective, observational study. We collected clinicopathological data of patients with primary jejunal or ileal tumors who had undergone small bowel resection in the Department of Gastrointestinal Surgery, West China Hospital, Sichuan University between January 2012 and September 2017. The inclusion criteria included: (1) older than 18 years; (2) had undergone small bowel resection; (3) primary location at jejunum or ileum; (4) postoperative pathological examination confirmed malignancy or malignant potential; and (5) complete clinicopathological and follow-up data. Patients with a history of previous or other concomitant malignancies and those who had undergone exploratory laparotomy with biopsy but no resection were excluded. The clinicopathological characteristics and prognoses of included patients were analyzed. Results: The study cohort comprised 220 patients with small bowel tumors, 136 of which were classified as gastrointestinal stromal tumors (GISTs), 47 as adenocarcinomas, and 35 as lymphomas. The median follow-up for all patient was 81.0 months (75.9-86.1). GISTs frequently manifested as gastrointestinal bleeding (61.0%, 83/136) and abdominal pain (38.2%, 52/136). In the patients with GISTs, the rates of lymph node and distant metastasis were 0.7% (1/136) and 11.8% (16/136), respectively. The median follow-up time was 81.0 (75.9-86.1) months. The 3-year overall survival (OS) rate was 96.3%. Multivariate Cox regression-analysis results showed that distant metastasis was the only factor associated with OS of patients with GISTs (HR=23.639, 95% CI: 4.564-122.430, P<0.001). The main clinical manifestations of small bowel adenocarcinoma were abdominal pain (85.1%, 40/47), constipation/diarrhea (61.7%, 29/47), and weight loss (61.7%, 29/47). Rates of lymph node and distant metastasis in patients with small bowel adenocarcinoma were 53.2% (25/47) and 23.4% (11/47), respectively. The 3-year OS rate of patients with small bowel adenocarcinoma was 44.7%. Multivariate Cox regression-analysis results showed that distant metastasis (HR=4.018, 95%CI: 2.108-10.331, P<0.001) and adjuvant chemotherapy (HR=0.291, 95% CI: 0.140-0.609, P=0.001) were independently associated with OS of patients with small bowel adenocarcinoma. Small bowel lymphoma frequently manifested as abdominal pain (68.6%, 24/35) and constipation/diarrhea (31.4%, 11/35); 77.1% (27/35) of small bowel lymphomas were of B-cell origin. The 3-year OS rate of patients with small bowel lymphomas was 60.0%. T/NK cell lymphomas (HR= 6.598, 95% CI: 2.172-20.041, P<0.001) and adjuvant chemotherapy (HR=0.119, 95% CI: 0.015-0.925, P=0.042) were independently associated with OS of patients with small bowel lymphoma. Small bowel GISTs have a better prognosis than small intestinal adenocarcinomas (P<0.001) or lymphomas (P<0.001), and small bowel lymphomas have a better prognosis than small bowel adenocarcinomas (P=0.035). Conclusions: The clinical manifestations of small intestinal tumor are non-specific. Small bowel GISTs are relatively indolent and have a good prognosis, whereas adenocarcinomas and lymphomas (especially T/NK-cell lymphomas) are highly malignant and have a poor prognosis. Adjuvant chemotherapy would likely improve the prognosis of patients with small bowel adenocarcinomas or lymphomas.
Humans ; Prognosis ; Intestinal Neoplasms/diagnosis* ; Duodenal Neoplasms ; Gastrointestinal Stromal Tumors ; Lymphoma ; Adenocarcinoma/surgery* ; Constipation ; Abdominal Pain ; Retrospective Studies

Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor in the gastrointestinal tract. At present, many professional academic groups and associations at home and abroad have released guidelines for the diagnosis and treatment of GIST. In 2020, Gastrointestinal Stromal Tumor Expert Committee of China Society of Clinical Oncology (CSCO) issued the first "CSCO gastrointestinal stromal tumor diagnosis and treatment guidelines" in China. In the same year, NCCN also released the first "NCCN guideline for the diagnosis and treatment of gastrointestinal stromal tumors (first edition in 2021)" by separating GIST-related content from the guideline for soft tissue sarcoma. However, there are many inconsistencies between the guidelines' recommendation and clinical practice in China. For example, in the aspect of diagnosis, the emphasis on and implementation of molecular detection are variable, the format of molecular detection report is not unified or standardized, the selection of detection methods is inappropriate, the diagnosis of wild-type GIST is not standardized, the risk assessment is improper, and the use of EUS-FNA is relatively seldom; in the aspect of drug therapy, there are some problems, such as the lack of standardization in dose and course of targeted drugs, and off-label medication such as cross line therapy; in terms of surgical treatment, there are also some problems, such as the clinical application scenarios of laparoscopic surgery exceed the recommendations of the guidelines, and the general description of surgical intervention of advanced GIST in the guidelines cannot well guide specific clinical practice. In this context, on the one hand, clinicians need to understand the academic frontier and standardize their medical behavior with the latest guidelines; on the other hand, clinicians need to consider the scientific rationale and accessibility of treatment on some clinical problems that are not clear in the guidelines, and take whether patients can benefit the most as the criteria.
China ; Gastrointestinal Stromal Tumors/diagnosis* ; Humans

Due to the lack of typical symptoms and imaging findings, gastric gastrointestinal stromal tumor (GIST) is easy to be misdiagnosed as other gastric tumors clinically. In clinical practice, clinicians should adopt the multidisciplinary team model, fully understand the characteristics of gastric GIST, grasp the key points of its differential diagnosis from other gastric tumors to achieve precise diagnosis and treatment. This paper analyzes the causes of misdiagnosis and elucidates the differential diagnosis of gastric GIST, aiming to improve the accuracy of preoperative diagnosis, reduce the misdiagnosis, and improve treatment outcomes.
Diagnosis, Differential ; Diagnostic Errors ; Gastrointestinal Stromal Tumors/diagnosis* ; Humans ; Stomach Neoplasms/diagnosis* ; Treatment Outcome

Objective: Contrast-enhanced CT is an important method of preoperative diagnosis and evaluation for the malignant potential of gastric submucosal tumor (SMT). It has a high diagnostic accuracy rate in differentiating gastric gastrointestinal stromal tumor (GIST) with a diameter greater than 5 cm from gastric benign SMT. This study aimed to use deep learning algorithms to establish a diagnosis model (GISTNet) based on contrast-enhanced CT and evaluate its diagnostic value in distinguishing gastric GIST with a diameter ≤ 5 cm and other gastric SMT before surgery. Methods: A diagnostic test study was carried out. Clinicopathological data of 181 patients undergoing resection with postoperative pathological diagnosis of gastric SMT with a diameter ≤ 5 cm at Department of Gastrointestinal Surgery of Renji Hospital from September 2016 to April 2021 were retrospectively collected. After excluding 13 patients without preoperative CT or with poor CT imaging quality, a total of 168 patients were enrolled in this study, of whom, 107 were GIST while 61 were benign SMT (non-GIST), including 27 leiomyomas, 24 schwannomas, 6 heterotopic pancreas and 4 lipomas. Inclusion criteria were as follows: (1) gastric SMT was diagnosed by contrast-enhanced CT before surgery; (2) preoperative gastroscopic examination and biopsy showed no abnormal cells; (3) complete clinical and pathological data. Exclusion criteria were as follows: (1) patients received anti-tumor therapy before surgery; (2) without preoperative CT or with poor CT imaging quality due to any reason; (3) except GIST, other gastric malignant tumors were pathologically diagnosed after surgery. Based on the hold-out method, 148 patients were randomly selected as the training set and 20 patients as the test set of the GISTNet diagnosis model. After the GISTNet model was established, 5 indicators were used for evaluation in the test set, including sensitivity, specificity, positive predictive value, negative predictive value and the area under the receiver operating curve (AUC). Then GISTNet diagnosis model was compared with the GIST-risk scoring model based on traditional CT features. Besides, in order to compare the accuracy of the GISTNet diagnosis model and the imaging doctors in the diagnosis of gastric SMT imaging, 3 radiologists with 3, 9 and 19 years of work experience, respectively, blinded to clinical and pathological information, tested and judged the samples. The accuracy rate between the three doctors and the GISTNet model was compared. Results: The GISTNet model yielded an AUC of 0.900 (95% CI: 0.827-0.973) in the test set. When the threshold value was 0.345, the sensitivity specificity, positive and negative predictive values of the GISTNet diagnosis model was 100%, 67%, 75% and 100%, respectively. The accuracy rate of the GISTNet diagnosis model was better than that of the GIST-risk model and the manual readings from two radiologists with 3 years and 9 years of work experience (83% vs. 75%, 60%, 65%), and was close to the manual reading of the radiologist with 19 years of work experience (83% vs. 80%). Conclusion: The deep learning algorithm based on contrast-enhanced CT has favorable and reliable diagnostic accuracy in distinguishing gastric GIST with a diameter ≤ 5 cm and other gastric SMT before operation.
Deep Learning ; Diagnosis, Differential ; Gastrointestinal Stromal Tumors/diagnostic imaging* ; Humans ; Retrospective Studies ; Stomach Neoplasms/diagnostic imaging* ; Tomography, X-Ray Computed

Intra-abdominal desmoid tumor (IADT) and gastrointestinal stromal tumor (GIST) are both mesenchymal tumors mostly found in gastrointestinal tracts and easily misdiagnosed, which would directly damage the survival prognosis and quality of life of patients. With the advent of the era of precision medicine, the understanding of the above two diseases is more in-depth, and the requirements for accurate diagnosis and individualized precision treatment are more stringent. Moreover, there seems to be some internal relationship between IADT and GIST, and the lack of systematic research and discussion makes clinical decision-making and patient management easy to fall into traps and misunderstandings. Therefore, this paper reviews the clinical characteristics, pathogenesis and treatments of the two, and explore their differences and internal relations, so as to provide research and practical reference for promoting more precise and individualized diagnosis and treatment regimens.
Clinical Decision-Making ; Fibromatosis, Aggressive/diagnosis* ; Gastrointestinal Stromal Tumors/diagnosis* ; Humans ; Prognosis ; Quality of Life

BACKGROUND/AIMS: Endoscopic ultrasonography (EUS) is the most efficient imaging modality for gastric subepithelial tumors (SETs). However, abdominopelvic computed tomography (APCT) has other advantages in evaluating the characteristics, local extension, or invasion of SETs to adjacent organs. This study aimed to compare the diagnostic ability of EUS and APCT based on surgical histopathology results.METHODS: We retrospectively reviewed data from 53 patients who underwent both EUS and APCT before laparoscopic wedge resection for gastric SETs from January 2010 to December 2017 at a single institution. On the basis of histopathology results, we assessed the diagnostic ability of the 2 tests.RESULTS: The overall accuracy of EUS and APCT was 64.2% and 50.9%, respectively. In particular, the accuracy of EUS vs. APCT for the diagnosis of gastrointestinal stromal tumors (GISTs), leiomyomas, and ectopic pancreas was 83.9% vs. 74.2%, 37.5% vs. 0.0%, and 57.1% vs. 14.3%, respectively. Most of the incorrect diagnoses with EUS involved hypoechoic lesions originating in the fourth echolayer, with the most common misdiagnosed lesions being GISTs mistaken for leiomyomas and vice versa.CONCLUSIONS: APCT showed a lower overall accuracy than EUS; however, APCT remains a useful modality for malignant/potentially malignant gastric SETs.
Diagnosis ; Endosonography ; Gastrointestinal Stromal Tumors ; Humans ; Leiomyoma ; Pancreas ; Retrospective Studies

Gastrointestinal subepithelial tumors (GSTs) are usually detected incidentally on endoscopic or radiologic examinations. In conventional endoscopy, a GST usually presents as a protuberant lesion with an intact mucosal surface. As the lesion is located beneath the mucosal layer of the gastrointestinal tract, conventional biopsy typically does not reveal the pathologic diagnosis. First, a GST should be differentiated from an extrinsic compression through the positional change of the patient during conventional endoscopic examination. In cases of GSTs originating from the gastrointestinal wall, endoscopic ultrasonography (EUS) can be beneficial for narrowing the differential diagnosis through delineation of echo findings and by determining the layer of origin. EUS findings can also help determine the management strategies for GSTs by making a differential diagnosis according to malignant potential.
Biopsy ; Diagnosis ; Diagnosis, Differential ; Endoscopy ; Endosonography ; Gastrointestinal Stromal Tumors ; Gastrointestinal Tract ; Humans

BACKGROUND/AIMS: Gastrointestinal stromal tumor (GIST) is one of the most common types of submucosal tumors (SMTs). Because of GIST's malignant potential, it is crucial to differentiate it from other SMTs. The present study aimed to identify characteristic endoscopic findings of GISTs in the small intestine. METHODS: We reviewed the clinicopathological and endoscopic findings of 38 patients with endoscopically or surgically resected SMTs in the small intestine. SMTs were classified into GIST and non-GIST groups, and clinicopathological and endoscopic findings were compared between the 2 groups. RESULTS: Fifteen patients had GIST and 23 patients had other types of SMTs in the small intestine. Comparison of the endoscopic findings between the 2 groups revealed that dilated vessels in the surrounding mucosa were significantly more in number in the GIST group than in the non-GIST group (P<0.05). However, there were no other differences in endoscopic findings between the 2 groups. Among patients with GISTs, the presence of dilated vessels in the surrounding mucosa was not associated with bleeding risk, tumor size, or metastasis rate at diagnosis. CONCLUSIONS: Dilated vessels in the surrounding mucosa, identified during balloon-assisted endoscopy, may be a diagnostic indicator for GIST in the small intestine. However, its clinical significance should be further analyzed.
Diagnosis ; Endoscopy ; Gastrointestinal Stromal Tumors ; Hemorrhage ; Humans ; Intestine, Small ; Mucous Membrane ; Neoplasm Metastasis

The Chinese Consensus on Endoscopic Diagnosis and Management of Gastrointestinal Submucosal Tumors is the first guideline in the area of gastrointestinal submucosal tumors(SMT) in China. SMTs of the Gastrointestinal tract are bulge lesions that originate from muscularis mucosa, submucosa, or muscularis propria. Endoscopic treatment of SMT is an effective way to improve the quality of life for patients, to reduce the burden on patients' families and the society, and to save national medical resources. For these reasons, this consensus has proposed the indications for endoscopic resection, on the basis of current status of diagnosis and treatment for SMT in China, and in combination with domestic and foreign literature and experts' experience:(1)For tumors with malignant potential suspected by preoperative examination or pathologically confirmed through biopsy, endoscopic resection should be considered when technically possible; (2) Endoscopic resection is indicated for SMT with symptoms (e.g. hemorrhage and obstruction); (3) For benign tumors suspected by preoperative examinations or confirmed by pathological examination, endoscopic resection could be considered when patients cannot attend regular follow-up, tumors grow rapidly in a short period or patients have a strong willing for endoscopic treatment. After endoscopic resection for SMT, different treatment algorithms should be recommended according to pathological types:(1)For benign lesions, such as lipoma and leiomyoma, postoperative routine treatment and follow-up are recommended;(2)For SMT without malignant potential, such as well-differentiated rectal neruoendocrine tumors (NET) that are < 1 cm, survival rate after complete resection is approximately 98.9%-100% and the recurrence rate is extremely low. Therefore, routine follow-up is recommended when the margin were confirmed negative pathologically; (3)Low-malignant-potential SMT, such as low-risk GIST, should be assessed by endoscopic ultrasonography or imaging every 6-12 months, and then managed according to clinical instructions; (4)Medium/high-malignant-potential SMT, such as type 3 and type 4 gastric NET, colorectal NET that are >2 cm, and medium/high-risk GISTs, additional treatment is required according to the guidelines for each specific disease. This expert consensus aims to provide an endoscopic SMT diagnosis and treatment standard,which fits our current national status, to domestic hospitals at all levels.
China ; Consensus ; Gastric Mucosa ; Gastrointestinal Stromal Tumors ; diagnosis ; surgery ; Humans ; Neoplasm Recurrence, Local ; Quality of Life ; Stomach Neoplasms ; diagnosis ; surgery ; Treatment Outcome

Management of large-size retrorectal gastrointestinal stromal tumors (GISTs) is complex and challenging from diagnosis to treatment. This may create technical difficulties in surgical access and complete resection of the tumor. The abdominosacral resection has the benefit of improved visualization via the anterior incision, with enhanced exposure of the midrectal area, which makes resecting the tumor completely via the posterior approach easier. We report 2 cases of patients with a retrorectal GIST and neurofibromatosis type 1, one in a 27-year-old woman with a defecation complaint and the other in a 58-year-old woman with a defecation and urination complaint. Based on the anatomical pathology, both patients were diagnosed with a GIST. The tumors were excised via an abdominosacral resection. Retrorectal GISTs are rare, and abdominosacral resection allows complete resection of a large-size retrorectal GIST with low morbidity and an absence of functional impairment. The abdominosacral resection should be considered in certain situations.
Adult ; Defecation ; Diagnosis ; Disease Management ; Female ; Gastrointestinal Stromal Tumors ; Humans ; Middle Aged ; Neurofibromatosis 1 ; Pathology ; Rectal Neoplasms ; Urination