scholarly journals Second Primary Tumors in Patients with Gastrointestinal Stromal Tumors: A Single-Center Experience

Medicina ◽  
2021 ◽  
Vol 57 (5) ◽  
pp. 494
Author(s):  
Murat Koçer ◽  
Sadık Muallaoğlu ◽  
Bülent Çetin ◽  
Hasan Şenol Coşkun ◽  
Nermin Karahan ◽  
...  

Background and Objectives: In this study, we investigated the frequency and type of second primary malignant tumors (SPMTs) accompanying gastrointestinal stromal tumors (GISTs), patient and tumor characteristics, and follow-up and survival data. Materials and Methods: We included 20 patients with SPMTs from a total of 103 patients with GISTs in a single center in Turkey. At the time of GIST diagnosis, patient age, sex, presentation symptoms, localization, pathological features of the tumor, stage, recurrence risk scoring for localized disease, treatments received, time of SPMT association, follow-up times, and survival analysis were recorded for each patient. Localization, histopathology, and stage of SPMT accompanying GISTs were also recorded accordingly. Results: SPMT was detected in 19.4% of patients with GISTs. Of the patients, 50% were men and 50% were women. The mean age at the time of diagnosis of GIST was 63.8 ± 10.81 years (range: 39–77 years). Of the GISTs, 60% were localized in the stomach, 25% in the small intestine, and 70% were at low risk. Of the SPMTs, 60% were in the gastrointestinal system. SPMTs were diagnosed as synchronous with GISTs in 50% of the patients. The mean follow-up period of the patients from the diagnosis of GIST was 45.6 (0.43–129.6) months. When the data were finalized, 5% died due to GIST, 35% died due to SPMT, and 15% died due to non-disease-related causes. Conclusions: SPMT was detected in 19.4% of patients with GISTs. GISTs were frequently located in the stomach, and most of them were at low risk. The most common SPMTs were gastrointestinal system tumors, and their coexistence was found to be synchronous. Most patients died due to SPMT during follow-up.

2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e23518-e23518
Author(s):  
Silvia Gasperoni ◽  
Francesca Castiglione ◽  
Margherita Vannucchi ◽  
Laura Papi ◽  
Elena Fumagalli ◽  
...  

e23518 Background: In patients with GIST, literature reports a risk of second primary tumors between 4.5% and 33% with different distribution in the worldwide. The network of 7 italian EURACAN centers has collected clinical and molecular features of GIST patients with second primary tumors. Methods: We reviewed the clinical characteristics of 201 patients with GIST and second primary tumors in order to evaluate association between risk of dead and each possible factor, using Kaplan meier curve, log-rank test and Cox model for Hazard Ratio and it’s interval Confidence 95% estimation. Furthermore, NGS analysis ( 56 gene onco panel) in 72 patients with GIST was performed. Results: On the basis of the significant correlation previously observed between the Miettinen risk criteria of GIST (low/very low) and the incidence of second primary tumors (gastrointestinal tumors),P < 0.001 (Abstr 11032 ASCO 2019), we observed in these patients a median age of diagnosis of GIST of 68, with prevalent gastric site localization ( KIT exon 11 mutation), NF1 and Lynch (kit/pdgfra WT) syndromes in the low risk subgroup. The more frequent site of second epithelial tumor in gastrointestinal tract was the colon followed by gastric, pancreatic and biliary tract. In patients with GIST with low-very low risk according Miettinen classification, after a median follow-up period of 25 years, we have observed that the gastroenteric site of second tumors occurrence is significantly related to the survival (p < .0003). In the NGS analysis of the GIST we observed the pathogenetic somatic mutations in the following genes: BRCA 2 (p.Thr2125fs), but germline test negative, TP53 (p.Arg192*,p.Gly244Ser,p.His168Leu), RET (p.Lys120Asn, p.His168Leu, p.Thr930Met ), NRAS (p.Gly134Asp), CTNNB1 (p.Ser45Phe), MSH6 (P.Ala164Val), SMARCB1 (p.Arg192), VHL (p.Gly93Val), PTEN(p.Val158Ile, p.Asn323fs), STK1I (p.Arg40Cys), SMO (p.Glu194Lys), EGFR (p.Gly721Asp), ATM (p.Asp2708Asn), ERBB4 (p.Asn181Ile). Conclusions: In our analysis patients with GIST (low-very low classes according to Miettinen) have significant risk to death because of second primary tumors in gastrointestinal tract. Specific attention to gastrointestinal screening during the follow-up of GIST (low and very low risk) is required.


2020 ◽  
Vol 6 (02) ◽  
pp. 53-60
Author(s):  
Chirag Jain ◽  
Mansi Jain ◽  
Ajay Kumar Dewan ◽  
Pavani Medisetty ◽  
Ankush Jajodia ◽  
...  

Abstract Introduction This study was performed to study the incidence and clinicopathological profile of second primary tumors (SPTs) in patients with squamous cell carcinoma of head and neck at our institute. Materials and Methods In this study, we included the data of 120 patients who developed an SPT of the upper aerodigestive tract following treatment of their index tumor (IT). Since the online data of cancer patients in our cancer registry was available from January 2005, we started the study retrospectively from that time. At our institute, Rajiv Gandhi Cancer Institute and Research Centre, the incidence was found to be 8.4%. Warren and Gates criteria were followed for defining a second tumor. Results Our study results showed an incidence of 8.4% of SPTs among patients of head and neck squamous cell carcinoma (HNSCC). The mean age of the patients was 56.47 ± 10.42 years with a male predominance. The mean period of addiction in patients was 18.48 ± 4.63 years. It was found that patients with SPT had significant history of tobacco and alcohol use. The most common location for ITs and SPT was tongue and buccal mucosa. The main modality of treatment was surgery in all patient groups. Patients were followed up at three-month intervals for the first 2 years. The SPT was diagnosed with a confirmation biopsy. Majority of patients with SPT again underwent surgery with reconstruction with either free flaps or local flaps. Recurrence after SPT treatment was seen in 16.67% cases, and primarily, it was a locoregional recurrence. Only patients with at least 6 months follow-up posttreatment of SPT were included in this study. At the end of the study, 62.5% patients were disease free, 20.83% patients were alive with disease, and 16.67% patients were dead. Some of the patients who are alive with disease developed a third primary tumor which was managed as per guidelines. Conclusion The incidence of SPTs is 8.4% in our institute. This study adds to the theory of field cancerization proposed by Slaughter et al. We found a significant history of tobacco chewing in our patients who developed SPT. The clinical significance of this study is identifying the features of SPT in patients with HNSCC and allowing for a rational follow-up schedule. The most important part of treatment although still lies with the patient by quitting use of alcohol and tobacco.


2013 ◽  
Vol 79 (7) ◽  
pp. 657-665 ◽  
Author(s):  
Sarah B. Fisher ◽  
Steven C. Kim ◽  
David A. Kooby ◽  
Kenneth Cardona ◽  
Maria C. Russell ◽  
...  

Large single-institution series of patients undergoing resection for gastrointestinal stromal tumors (GIST) are lacking. Clinicopathologic characteristics and postoperative outcomes were retrospectively collected and analyzed from patients undergoing resection for GIST from 2002 to 2011. One hundred seventy-six patients were identified; 156 underwent resection of primary nonmetastatic disease. KIT mutations were identified in 131 patients (84.0%). Of the 156 patients with primary disease, the most common site was the stomach (75.6%). Tumors were categorized as very low (24.4%), low (35.9%), intermediate (12.2%), high (24.4%), or unknown (3.2%) risk. Symptomatic patients more often had high risk (35.6 vs 9.8%; P < 0.0001) and larger tumors (7.3 vs 3.0 cm; P < 0.0001). Forty-seven patients (30.1%) underwent laparoscopic resection (LR). Compared with open surgery, LR was performed for smaller tumors (3.8 vs 6.2 cm; P = 0.002). Positive margin rates were similar (4.3% LR vs 10.2% open; P = 0.346). Median follow-up for the 156 patients with primary tumors was 32.9 months; mean overall survival was 120.9 months (median not reached). Of the 20 patients with metastatic GIST (excluded from above analysis), five patients (25.0%) died of disease with a median follow-up of 15.9 months. Most patients with resectable primary GIST have a favorable prognosis. The presence of symptoms directly related to GIST may be associated with a poor prognosis and is likely related to increased tumor size. Laparoscopic resection is well tolerated and does not appear to compromise outcomes in well-selected patients. Highly selected patients with metastatic disease may benefit from resection.


Medicina ◽  
2021 ◽  
Vol 57 (11) ◽  
pp. 1242
Author(s):  
Natalia Dowgiałło-Gornowicz ◽  
Klaudia Sztaba ◽  
Paweł Lech ◽  
Anna Botulińska ◽  
Maciej Michalik

Background and Objectives: Gastrointestinal stromal tumors (GISTs) are rare mesenchymal neoplasms located mainly in the fundus (60–70%). The incidence of GIST is approximately 10 per million population per year in Europe, with a peak incidence at the age of 63. Recent studies suggest that morbidly obese patients have a higher incidence of GIST than the general population. The aim of this study was to analyze the incidence of GIST in patients undergoing laparoscopic sleeve gastrectomy (LSG) in our department. Materials and Methods: this paper present the retrospective study of prospectively collected data of 1564 patients who underwent LSG in a single large bariatric center from October 2013 to September 2021. After surgery, each sample of the resected stomach was sent for histopathological examination. For the analysis, we included patients diagnosed with GIST intraoperatively or postoperatively. Results: GISTs were found in five patients (0.31%). There were three men and two women. The mean age was 50.2 (range 32–63 ± 11.8) and the mean preoperative body mass index was 43.3 kg/m2 (40–49.4 ± 3.2). In four cases, GISTs were found in the fundus (80%), and in one in the pylorus (20%). None of the tumors were larger than 7 mm in diameter and all were diagnosed as a very low-risk category. No adjuvant treatment was required. All patients achieved good or satisfactory bariatric and metabolic results. Conclusions: The incidence of GIST in our study was estimated at 0.31%. All patients had a very low-risk GIST and no recurrence until follow-up. Recent literature suggests that the risk of GIST is higher in the obese population, and therefore surgeons should be aware of the risk of incidental GIST during LSG.


2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 20523-20523
Author(s):  
Y. Zhou ◽  
C. Du ◽  
H. Fu ◽  
G. Zhao ◽  
Y. Shi

20523 Background: Gastrointestinal stromal tumors (GISTs) with positive kit staining, although rare, are the most common mesenchymal neoplasms affecting the gastrointestinal tract. We present our experience in the treatment of disease involving adjacent organs with multivisceral resections. Methods: The clinicopathologic records of twenty-two patients with multivisceral resection, among 170 patients suffered from GISTs treated in our hospital from 1994 to 2005, were retrospectively reviewed. Nine primary tumors originated from the stomach, 4 from the small intestine, 6 from the colorectum, 1 from abdominal cavity, and 2 from the retroperitoneal area. Meanwhile complete follow-up records were available in 15 patients with median 5-year follow-up. Results: The patients included 9 men and 13 women, from 36 to 77 year old. The largest tumor diameter ranged from 2 to 20 cm. The types of multivisceral operation comprised splenectomy (n=8), coloectomy (n=6), nephrectomy (n=5), partial hepatectomy (n=1), duodenectomy (n=1), oophorectomy (n=1), pancreatectomy (n=1), and adrenalectomy (n=1). None of lymph node metastatsis was found in 7 patients with lymph node dissection with gastric GISTs (n=5) or small intestinal GISTs (n=2). The involved organs confirmed by the pathologic diagnosis included kidney (n=2), spleen (n=2), pancreas (n=2), mesentery (n=1), and omentum (n=1). There was no perioperative mortality in this series. Among follow up patients, 9 were alive and 5 were died from the tumor recurrence or metastasis. Among them, one person with gastrectomy, pancreatectomy, adrenalectomy is still alive under 2 year follow-up by imatinib mesylate therapy postoperatively. Conclusions: Complete surgical resection with a negative gross margin by en bloc resection of the involved organs remains the standard treatment for non-metastatic GISTs. Imatinib mesylate represents a major breakthrough in the treatment of advanced GISTs and is the first effective systemic therapy for the disease. No significant financial relationships to disclose.


2018 ◽  
pp. 3-14

Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the digestive tract (1%). These tumors express the CD 117 in 95% of cases. The stomach is the preferential localization (70%). Diagnosis is difficult and sometimes late. Progress of imaging has greatly improved the management and the prognosis. Computed tomography (CT) is the gold standard for diagnosis, staging, and treatment follow-up. The increasing recognition of GIST’s histopathology and the prolonged survival revealed some suggestive imaging aspects. Key words: gastro-intestinal stromal tumors; computed tomography; diagnosis


Author(s):  
Elizabeth Vogel ◽  
Thomas Leaver ◽  
Fiona Wall ◽  
Ben Johnson ◽  
Michael Uglow ◽  
...  

Abstract Objective There are no data on the effect of X-Ray irradiation to the vulnerable pelvic organs of babies during DDH follow-up. This study aims to calculate, for the first time, the radiation exposure to infants during follow-up for DDH harness treatment, and thus quantify the lifetime risk of malignancy. Methods Patients who had completed 5 years’ follow-up following successful Pavlik harness treatment were identified from the hospital DDH database. The radiation dose was extracted from the Computerised Radiology Information System database for every radiograph of every patient. The effective dose (ED) was calculated using conversion coefficients for age, sex and body region irradiated. Cumulative ED was compared to Health Protection Agency standards to calculate lifetime risk of malignancy from the radiographs. Results All radiographs of 40 infants, successfully treated in Pavlik harness for DDH, were assessed. The mean number of AP pelvis radiographs was 7.00 (range: 6–9, mode: 7). The mean cumulative ED was 0.25 mSv (Range: 0.11–0.46, SD: 0.07). This is far lower than the annual ‘safe’ limit for healthcare workers of 20 mSv and is categorised as “Very Low Risk”. Conclusion Clinicians involved in the treatment DDH can be re-assured that the cumulative radiation exposure from pelvic radiographs following Pavlik harness treatment is “Very Low Risk”. Whilst being mindful of any radiation exposure in children, this study provides a scientific answer that help addresses parental concerns.


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