Primary extragastrointestinal stromal tumors: A clinicopathological and immunohistochemical study-A tertiary care center experience

Patnayak, Rashmi; Jena, Amitabh; Parthasarathy, Sriram; Prasad, PallaDurga; Reddy, Munish; Chowhan, Amit Kumar; Rukamangadha, N; Phaneendra, Bobbidi Venkata

doi:10.4103/0019-509x.112298

Cited by 12 publications

(15 citation statements)

References 20 publications

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“…Based on our analyses, the order of high occurrence of eGIST was 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%) and urinary bladder (1.8%). The location order of high-occurrence was partly similar to the findings in the literature, 22 who reported the retroperitoneum (n = 4), mesentery (n = 3), omentum (n = 2) and pelvis (n = 1) as their most commonly observed location of eGIST.…”

Section: Discussionsupporting

confidence: 86%

<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: 86%

<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 histopathological appearance of EGISTs is variable, but, in general, three subtypes, including spindle, epithelioid and mixed cell types, are noted. In the present study, the EGISTs predominantly displayed the spindle cell subtype (87.5%; 21/24), which is consistent with previous studies ( 13 , 16 , 17 ). KIT is overexpressed at a high frequency (96.4–100%) when detected by an immunohistochemical method and has been shown to be a good immunomarker for diagnosing EGISTs ( 16 , 18 ).…”

Section: Discussionsupporting

confidence: 93%

“…Due to the similar histological appearance and immunophenotype compared with GISTs, EGISTs are believed to be representations of either GISTs that have separated from the GI tract wall or independent mesenchymal cell growth of the mesentery, omentum and retroperitoneum ( 5 , 14 ). The incidence of EGISTs is uncertain with regard to gender ( 3 , 4 , 13 , 15 – 17 ). The present study exhibits a slight male predominance (54.2%; 13/24).…”

Section: Discussionmentioning

confidence: 99%

Extragastrointestinal stromal tumors: Computed tomography and magnetic resonance imaging findings

et al. 2014

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Extragastrointestinal stromal tumors (EGISTs) are rare mesenchymal tumors that originate outside the gastrointestinal tract. The aim of the present study was to investigate the computed tomography (CT) and magnetic resonance imaging (MRI) features of EGISTs and analyze the correlations between radiological findings and pathological features. CT and MRI images of 24 patients with EGISTs were reviewed retrospectively. Patient demographics and tumor characteristics, including localization, size, contours, borders, cystic-necrotic components, calcification, hemorrhage, tumor vessels, attenuation and intensity, degree and pattern of enhancement, ascites, tumor invasion, lymphadenopathy and distant metastasis were recorded. Statistical analyses to compare the radiological characteristics of low- and high-grade EGISTs were performed with χ2 or Fisher’s exact tests. The mean patient age at the time of presentation was 53 years. A total of 24 EGISTs were detected, originating in the omentum (n=4), mesentery (n=19) and retroperitoneum (n=1), respectively. The EGISTs displayed a predominantly spindle cell subtype (87.5%; 21/24). The majority of the EGISTs appeared as large (>10 cm; 70.8%; 17/24), round or oval (66.7%; 16/24), cystic-solid (87.5%; 21/24) and ill-defined (66.7%; 16/24) soft-tissue masses. The EGISTs were hypodense (69.6%; 16/23) or isodense (30.4%; 7/23) on CT images, hypointense (50%; 3/6), isointense (33.3%; 2/6) or hyperintense (16.7%; 1/6) on T1-weighted imaging (T1WI), and hyperintense on T2WI (100%; 6/6) and diffusion-WI (DWI; 100%; 6/6). A total of 54.2% (13/24) of the EGISTs displayed tumor vessels. Overall, 95.8% (23/24) of the masses showed marked enhancement and 87.5% (21/24) demonstrated heterogeneous enhancement. Calcification, hemorrhage, ascites and lymphadenopathy were rare characteristics in the EGISTs. Distant metastases were present in 10 patients (41.7%). The size, borders, tumor vessels and distant metastasis correlated with high-grade EGISTs [>5 mitoses/50 high-power fields (HPFs)] (P<0.05). The results of the present study indicated that clinical and radiological features, including advanced age, a large tumor size, cystic-necrotic components, rare lymphadenopathy, a heterogeneous enhancement pattern and hepatic metastasis may aid in the diagnosis of EGISTs. Radiological characteristics, such as a large size (>10 cm), ill-defined borders, tumor vessels and distant metastasis, can provide useful information in identifying the malignant behavior of EGISTs.

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“…Literature search of MEDLINE was performed for all articles in English published from 1998 through 2015. MEDLINE search resulted in 47 case reports 8 9 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60 including 57 patients and 6 case series 61 62 63 64 65 66 including 40 cases. As a result, a total of 99 omental GISTs patients were identified.…”

Section: Methodsmentioning

confidence: 99%

Clinicopathological features and prognosis of omental gastrointestinal stromal tumor: evaluation of a pooled case series

Feng

Tian

Liu

et al. 2016

Sci Rep

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Clinicopathological features and prognosis of omental GISTs are limited due to the extremely rare incidence. Therefore, the aim of the present study was to investigate the clinicopathological features and prognosis of omental GISTs. Omental GISTs cases were obtained from our center and from case reports and clinical studies extracted from MEDLINE. Clinicopathological features and survivals were analyzed. A total of 99 cases of omental GISTs were enrolled in the present study. Omental GISTs occurred predominantly in greater omentum (78/99, 78.8%). The majority of tumors exceeded 10 cm in diameter (67/98, 68.3%) and were high risk (88/96, 91.7%). Histological type was correlated with tumor location and mutational status. The five year DFS and DSS was 86.3% and 80.6%, respectively. Mitotic index was risk factor for prognosis of omental GISTs. Prognosis of omental GISTs was worse than that of gastric GISTs by Kaplan-Meier analysis. However, multivariate analysis showed that the prognosis was comparable between the two groups. The majority of omental GISTs were large and high risk. Mitotic index was risk factor for prognosis of omental GISTs. The prognosis was comparable between omental and gastric GISTs.

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Primary extragastrointestinal stromal tumors: A clinicopathological and immunohistochemical study-A tertiary care center experience

Abstract: EGISTs are rare and should be considered in the differential diagnosis of the mesenchymal tumors and immunohistochemistry helps to confirm the diagnosis. Further study with better follow-up is desired to characterize these uncommon tumors.

Cited by 12 publications

References 20 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 tumors: Computed tomography and magnetic resonance imaging findings

Clinicopathological features and prognosis of omental gastrointestinal stromal tumor: evaluation of a pooled case series

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