Gene Mutations and Prognostic Factors Analysis in Extragastrointestinal Stromal Tumor of a Chinese Three-Center Study

Zheng, Shu; Huang, Keer; Tao, De-you; Pan, Yi

doi:10.1007/s11605-010-1292-x

Cited by 18 publications

(24 citation statements)

References 14 publications

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“…In contrast with our study cohort, male (56.4% vs 43.6%) cases were predominant, and the mean age of tumor occurrence was 58 years old. We hypothesize that this may be related to population ethnicity, as in the analysis of previous study, 23 among the eight studies investigated, only one 24 was from China, and in that study, male cases were also predominant (60% vs 40%) at a mean age of 60 years old, resonating to that of our findings. However, more studies are required to validate this hypothesis.…”

Section: Discussionsupporting

confidence: 88%

<p>Retroperitoneal Extragastrointestinal Stromal Tumors Have a Poor Survival Outcome: A Multicenter Observational Study</p>

Hu¹,

Zheng²,

Li³

et al. 2020

CMAR

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Purpose Gastrointestinal stromal tumors (GISTs) are commonly known to be derived from the gastrointestinal (GI) tract, but recently there have been more and more literature describing lesions with similar pathological and immunohistochemical resembling GISTs but located outside the GI tract, and they have been termed as extra-GISTs (eGISTs). However, due to the rare incidence of eGISTs, its association with survival outcomes is poorly understood, especially in the Chinese population. Here, we aimed to identify the risk factors of eGISTs and to assess their association with overall survival (OS) and disease-free survival (DFS). Patients and Methods Data of pathologically confirmed eGISTs cases, without radiological and perioperative evidence of other primary lesions, and with no microscopically identified adhesion between the tumor and the gastrointestinal serosa, which were surgically treated between January 2006 and September 2017 were retrieved from the database of four high-volume hospitals. Immunohistochemical and genetic testing were performed on the postoperative lesions and were staged using the National Institutes of Health (NIH) criteria. Results A total of 55 cases were retrieved. eGISTs were identified from the retroperitoneum (36.4%), mesocolon (25.5%), small bowel mesentery (12.7%), abdominopelvic cavity (12.7%), lesser omental sac (5.5%), ovary (3.6%), pancreatic capsule (1.8%), or urinary bladder (1.8%). Based on the NIH risk classification, majority of the lesion were classified as high risk (85.5%). KIT 11 was the most common mutation site (76.5%) and 25.0% of the cases were wild-type eGISTs. Multivariate analyses showed that tumor location and size were independent factors affecting prognoses. Patients with tumors in the retroperitoneum had significantly poorer OS and DFS as compared to those in the non-retroperitoneum (HR [95% CI] for OS and DFS: 2.546 [1.023–6.337] [ P = 0.037] and 2.475 [0.975–6.273] [ P = 0.049], respectively). Similar findings were found for tumors of size >15 cm, compared to ≤15 cm (HR [95% CI] for OS and DFS: 5.350 [2.022–14.156] [ P < 0.001] and 3.861 [1.493–9.988] [ P = 0.003], respectively). Conclusion eGISTs were predominantly found from the retroperitoneum and mostly classified as high risk. Those located in the retroperitoneum and of size >15 cm had the poorer OS and DFS as compared to those in the non-retroperitoneum and of size <15 cm.

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Section: Discussionsupporting

confidence: 88%

<p>Retroperitoneal Extragastrointestinal Stromal Tumors Have a Poor Survival Outcome: A Multicenter Observational Study</p>

Hu¹,

Zheng²,

Li³

et al. 2020

CMAR

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“…The findings of previously published studies on extragastrointestinal stromal tumors (EGIST) have led authors to believe that these tumors are histologically and immunohistochemically similar tumors, with features that are also similar to those found in gastrointestinal stromal tumors (GIST), including the expression of c-KIT and PDGFR- α gene mutations [ 4 , 14 , 15 ] (Song Zheng, Huang, Tao, & Pan, 2011). The similar findings from previous studies have supported the assumption that GIST and EGIST arise from similar cells.…”

Section: Discussionmentioning

confidence: 99%

Extragastrointestinal Stromal Tumor (EGIST): A 16-Year Experience of 13 Cases Diagnosed at a Single Center

Hatipoğlu

2018

Med Sci Monit

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BackgroundGastrointestinal stromal tumor (GISTs) rarely occurs outside the GI tract as extragastrointestinal stromal tumor (EGIST). The aim of this study was to review the clinical presentation, diagnosis, and outcome of EGIST at a single center.Material/MethodsThe study was a retrospective study performed at Istanbul University Hospital in a 16-year period and included patients with a histopathological diagnosis of EGIST confirmed to arise outside the GI tract. The patients’ available medical records included patient demographics, imaging and surgical data, and diagnostic histopathology reports. Cases of EGIST underwent follow-up for several years and the medical files of patients were well maintained.ResultsThirteen cases of EGIST included six women and seven men, with a mean age of 59.6 years (range, 33–83 years). Eleven patients had EGISTs located in the intra-abdominal cavity, one patient’s tumor was in the retroperitoneum, and in the jejunal mesentery in one patient. The mean diameter of the EGISTs was 15.6 cm (range, 4–30 cm). Immunohistochemistry showed that all cases were negative for desmin, with positive immunostaining for CD34 (n=6), smooth muscle actin (SMA) (n=3), and Ki67 (n=6), without specific diagnostic markers. Following surgical resection, tumor recurrence occurred in three patients, and metastasis in two patients. The mean overall survival (OS) was 45.66 months (56.44 months for women; 32.57 months for men); the 5-year survival rate of our patients was 38%.ConclusionsEGIST presented with a large tumor size at diagnosis, was mainly intra-abdominal, and had a low mean patient survival time with no specific diagnostic tissue immunomarkers.

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“…Zheng et al demonstrated that the c-kit and PDGFRA mutation in EGISTs was similar to that of GISTs. Therefore, from a molecular genetics perspective, EGISTs may be a unique subtype of GISTs (3). …”

Section: Discussionmentioning

confidence: 99%

“…A tumor size >10 cm, mitotic activity >2 per 50 high-power fields, tumor necrosis, marked nuclear atypia, >10% Ki-67 protein expression and epithelioid-/mixed cell-type, have acted as significant predictors of survival (2,3). Survival analysis indicated that mitotic count and Ki-67 protein expression levels were significant predictors of disease-specific survival; however, tumor size, primary location, c-kit and PDGFRA gene mutations were not.…”

Section: Discussionmentioning

confidence: 99%

“…Extragastrointestinal stromal tumors (EGISTs) have no contact with the stomach and intestine, by definition, and typically occur in the omentum, mesentery or retroperitoneum (1,2). However, EGISTs are not connected to the digestive tract (3). GISTs in the omentum or mesentery are typically metastases from primary sites within the gastrointestinal tract.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Giant extragastrointestinal stromal tumor in the transverse mesocolon concomitant with gastric cancer in an elderly patient: Case report

Xue

Chen

2012

Oncology Letters

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Extragastrointestinal stromal tumors (EGISTs) are neoplasms located outside the gastrointestinal tract in sites including the omentum, mesentery and retroperitoneum. EGISTs of the transverse mesocolon are rarely noted in the literature. Herein, we describe a rare case of giant EGIST concomitant with gastric cancer in a 78-year-old male who presented with upper abdominal pain and a palpable mass. The patient underwent en bloc resection of the tumor with a distal gastrectomy, with a D2 lymphadenectomy for the gastric cancer, accompanied with resection of a segment of the transverse colon. The patient received targeted therapy (imatinib 400 mg, daily) and adjuvant chemotherapy with FOLFOX (six cycles). Neither recurrence nor metastasis was observed after 24 months of follow-up.

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Gene Mutations and Prognostic Factors Analysis in Extragastrointestinal Stromal Tumor of a Chinese Three-Center Study

Cited by 18 publications

References 14 publications

<p>Retroperitoneal Extragastrointestinal Stromal Tumors Have a Poor Survival Outcome: A Multicenter Observational Study</p>

<p>Retroperitoneal Extragastrointestinal Stromal Tumors Have a Poor Survival Outcome: A Multicenter Observational Study</p>

Extragastrointestinal Stromal Tumor (EGIST): A 16-Year Experience of 13 Cases Diagnosed at a Single Center

Giant extragastrointestinal stromal tumor in the transverse mesocolon concomitant with gastric cancer in an elderly patient: Case report

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