Isolated abdominal wound metastasis from a gastrointestinal stromal tumor

Background. Gastrointestinal stromal tumors (GIST) frequently recur even after complete resection. The typical pattern of failure from GISTs is both local and distant with hepatic and peritoneal metastases being most common. Isolated abdominal-wall recurrence from GISTs has not been previously described. Aim of the Study. To report an isolated abdominal-wound recurrence in the absence of widespread disease in a patient with GIST. Methods. Case report of a GIST and isolated abdominal-wound recurrence after laparoscopic-assisted en bloc resection. Results. Elderly male patient presented with an isolated abdominal wall incisional recurrence 18 mo after gastric resection and adjuvant imatinib mesylate therapy for a high-grade GIST. Conclusions. Complete resection of gastrointestinal stromal tumors followed by imatinib therapy may alter the extent of recurrence.     

Resection of GIST in the duodenum and proximal jejunum: A retrospective analysis of outcomes

IntroductionGastrointestinal stromal tumors (GISTs), with a primary occurrence in the duodenum and proximal jejunum, are rare and treatment is poorly understood. This study aimed to evaluate the main factors influencing the prognosis of GIST resection in this complex anatomical structure.Materials and methodsThis retrospective study included 47 patients who underwent surgery for primary GIST of the duodenum (20) and proximal jejunum (27) between 2012 and 2017. Perioperative clinical data as well as relapse and survival information were collected.ResultsAll patients underwent negative margin resection (R0) of duodenal and proximal jejunum GISTs. Complications occurred more frequently in treatment of duodenal GISTs than proximal jejunum GISTs (p = 0.003). GISTs in D3 (the 3rd portion of duodenum) were related to larger tumor size (p = 0.001), higher probability of severe complication rate (p = 0.042), longer hospital stays (p = 0.023) and fasting time (p = 0.020). More complications were found for patients with digestive reconstruction than limited resection (p = 0.010). Additionally, patients with a tumor mass larger than 5 cm or a mitotic index greater than 5 mitoses/50 HPFs showed poorer therapeutic outcomes. The 1- and 3-year overall survival was 97.9% and 86.1%, respectively and were not influenced by operation type (p = 0.061) or GIST position (p = 0.447).ConclusionWith negative operational margins, limited resection is a safe and feasible procedure for duodenal and proximal jejunum GIST patients and unnecessary digestive reconstruction should be avoided. Considering the severe complication rate, resection for GISTs in D3 should be performed with care.     

Robotic duodenal (D3) resection with Roux-en-Y duodenojejunostomy reconstruction for large GIST tumor: Step by step with video

Background: Duodenal gastrointestinal stromal tumors (GISTs) are uncommon, making up only 3–5% of all GISTs. [1,2] Historically, the treatment of choice for duodenal GIST tumors was pancreaticoduodenectomy. [3]Currently, newer surgical intervention methods including local resection via laparotomy, endoscopic resection, and robotic resection are feasible. When doing a local resection, the defect can be closed either primarily or via a Roux-en-Y duodenojejunostomy. [3]Case presentation: Our patient is a 64-year- old female who presented initially with shortness of breath and was found to have a pulmonary embolism. She then developed upper GI bleeding from anticoagulation and was found to have an ulcerated GIST tumor in the anti-mesenteric border of the third portion of the duodenum (D3). Initial surgery was postponed due to high pulmonary artery pressure from the pulmonary embolism. The patient underwent argon beam coagulation of the bleeding mass to control the bleeding, followed by localized radiotherapy plus Gleevec. Unfortunately, the tumor grew in size during follow-up. The patient was then taken to the OR for a robot-assisted partial duodenal resection (D3) with Roux-en-Y duodenojejunostomy to reconstruct the large defect. She did well post operatively and her final pathology showed a GIST tumor, c-kit and DOG1 positive, 3.5 cm in size, with negative margins.Conclusion: Robotic duodenal resection is a new technique currently being used to resect duodenal GIST tumors. Our video demonstrates the feasibility of D3 partial resection with Roux-en-Y duodenojejunostomy. Duodenal GIST tumor robotic resection offers both decreased morbidity and adequate oncologic outcomes.     

Extragastrointestinal stromal tumor originating from the vulva

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. They have gain-of-function mutations of the c-kit receptor tyrosine kinase gene and have been suggested to originate from the interstitial cells of Cajal. A small percentage of GISTs form extragastrointestinal masses. We report a rare case of a GIST originating from the vulva. A 55-year-old woman presented with a vulvar tumor. The tumor was initially diagnosed as a leiomyosarcoma following the first resection. Following a second recurrence the patient was administered chemotherapy. A third recurrence occurred and the patient underwent a third resection. Histology revealed that a bundle of fibrous tumor cells had invaded the connected tissue and muscular coat, and some spindle-shaped and blunt-ended nuclei were detected. Furthermore, immunohistochemical evaluation revealed that the tumor cells exhibited strong and diffuse staining for c-kit and CD34. The recurrent tumor was diagnosed as a GIST and a reevaluation of the original specimens also revealed a GIST. The patient was treated with imatinib, and is currently healthy with no evidence of recurrence at 20 months after the last surgery.     

Intragastric single-port surgery (IGS) for gastric endophytic gastrointestinal stromal tumor (GIST): A novel surgical treatment

BackgroundIntragastric surgery with a single incision has been performed for several diseases, such as gastric tumors[1] and pancreatic pseudocyst[2], safety, feasibility and potential benefits of which have been reported in previous relevant studies[3].MethodsThe video shows a 65-year-old man with upper gastrointestinal hemorrhage, preoperative abdominal CT scan and endoscopy suggested an endophytic tumor located in gastric corpus, suggesting gastrointestinal stromal tumor (GIST). Intragastric single-port surgery (IGS) was indicated.ResultsUnder general anesthesia, patient was placed in supine position. Surgeons stood on the right side of the patient. After a 2.5cm transverse incision was made on left upper abdominal wall, gastric anterior wall was exteriorized and fixed to the skin incision. Single-port device was inserted inside the stomach after anterior gastric wall was opened. Next, laparoscope was introduced into gastric cavity and identified the location of tumor. Full thickness resection of the tumor was performed by using linear stapler, then stapler line was embedded with continuous sutures. Finally, after specimen and single-port device removal, the stomach incision was closed extracorporeally. The operation time was 112 minutes. Final pathology confirmed GIST (4.5cm) with negative margins and patient discharged after 4 days, without postoperative complications. In 1-year follow-up time, without recurrence or death.ConclusionIGS is safe and effective for gastric endophytic GIST resection, which not only preserves all advantages of conventional laparoscopic operation, but also includes other benefits like obviating the need of abdominal cavity exploration, potential reduction of risk of neoplasm seeding, and offering good postoperative cosmetic result.     

Management of Gastrointestinal Stromal Tumors: Looking Beyond the Knife. An Update on the Role of Adjuvant and Neoadjuvant Imatinib Therapy

Background

Gastrointestinal stromal tumors (GISTs) have traditionally been treated with surgical resection alone resulting in high rates of recurrence. However, the discovery of imatinib efficacy in GIST has revolutionized its management.

Discussion

Imatinib may be used as neoadjuvant therapy with the goal of reducing tumor size, minimizing surgical morbidity and, in some cases, rendering inoperable cases operable. In addition, imatinib use in the adjuvant setting to eradicate micrometastases and prevent recurrence has shown promising results in reducing relapse rates. Appropriate patient selection and optimal dose and duration of imatinib therapy remain undecided and require further investigation. We present a literature review and a case report of our patient with a symptomatic gastric GIST managed successfully utilizing neoadjuvant imatinib therapy, laparoscopic limited resection, and adjuvant imatinib therapy.     

31例胃小间质瘤临床特点及预后分析

  目的   探讨原发于胃的小间质瘤的临床病理特点和预后影响因素。   方法  回顾性分析1999年3月~2012年3月期间在中国医学科学院肿瘤医院接受手术治疗并经病理证实的31例胃小间质瘤的临床资料, 统计临床特点并分析影响其预后的因素。   结果  31例患者中29例获得随访(随访率93.5%)。其中男性10例, 女性21例, 中位年龄58(37~81)岁。腹部不适和腹痛是最主要的首发症状(各10例, 32.3%)。全组患者均接受手术治疗, 胃大部切除和胃壁局部切除各11例, 腹腔镜下胃壁局部切除5例, 胃镜下肿瘤剥离4例, 术后无重大并发症。截止到随访终点, 肿瘤复发2例, 肝转移1例; 因肿瘤进展死亡1例, 因其他恶性肿瘤死亡1例。中位总生存时间(OS)预测值为130.4个月, 中位无疾病进展时间(PFS)预测值为120.3个月。   结论  胃小间质瘤总体预后较好; 手术切除是主要治疗方式, 可根据情况选取微创治疗方式; 肿瘤坏死、出血和胃黏膜受累可能是预后不良因素。      

Five cases of early gastric cancer in the reconstructed gastric tube after radical resection for esophageal cancer

We report seven early gastric cancers in five patients, which arose in the reconstructed gastric tube after radical resection for esophageal cancer. Four of them occurred in the middle gastric tube and three in the distal gastric tube. Three of 5 cases were reconstructed via the retromediastinal route and two via the presternal route. They all were diagnosed by follow-up endoscopy from 8 months to 5 years after esophagectomy. All of them were treated surgically with partial resection of the gastric tube because they were suspected to have invaded the submucosal layer or large enough to be treated with endoscopic mucosal resection (EMR). Histologically, six of seven were diagnosed as well differentiated adenocarcinoma and one as signet ring cell carcinoma. Although one of them died for reasons other than cancer itself, the others are alive and well without any recurrence. Recently, gastric tube cancer after esophagectomy has been increasingly reported to be accompanied with prolongation of survival of esophageal cancer patients. Total or partial gastrectomy is proposed for surgical treatment of gastric tube cancer, but the operating procedure is complicated and invasive, especially in the case of gastric tube reconstructed via the retromediastinal route. Total gastrectomy is much more invasive because it needs re-reconstruction with other organs. Therefore, it is important to detect the lesion in early stages so as to treat it with minimally invasive surgery such as EMR or partial resection. Hence intensive follow up with endoscopy is necessary after resection of esophageal cancer.     

High-risk CD117-positive gastrointestinal stromal tumor of the colon in a 12-year-old girl: adjuvant treatment with imatinib mesylate

Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors of the alimentary tract rarely observed in children. The treatment of choice for GIST is surgical resection. Although the prognosis of GISTs with low malignant potential is excellent, high-malignant-potential GISTs have a high rate of recurrence. Prognostic factors such as tumor size, mitotic rate and presence of metastases may provide an indication for adjuvant imatinib mesylate (IM) treatment. Here we present a young patient with a large GIST with high-risk features who is in complete remission after surgical excision and adjuvant IM treatment. This patient is the only colon-located CD117-positive case where IM was administered. The exact indications as well as the optimal dose and duration of IM need to be clarified with the contribution of new cases and the growing experience of this rare disease.     

16例巨大型胃肠间质瘤手术治疗分析

目的：通过对巨大型胃肠间质瘤（GIST）的诊断和手术方式进行探索,为临床治疗巨大型 GIST 提供新思路。方法对16例巨大型 GIST 患者一般资料、影像学资料、手术方式、随访指标进行回顾性调查,并进行统计学分析。结果11例服用甲磺酸伊马替尼患者无复发生存期1 a 以上,而5例未服用伊马替尼治疗患者1 a 内复发,中位无复发生存期为33个月。COX 多因素分析显示,肿瘤部位、术中肿瘤有无破裂、是否使用伊马替尼治疗与患者预后有关（P <0.05）,可作为患者预后的独立因素。结论巨大型 GIST 在选择手术时,应遵循术前影像学评估、术前服用甲磺酸伊马替尼治疗缩小肿瘤和手术切除时边缘切净为3大原则,这对于改善患者预后和延长生存期有益。     

Soft Tissue Sarcoma, Version 2.2012: Featured Updates to the NCCN Guidelines

The major changes to the 2012 and 2011 NCCN Guidelines for Soft Tissue Sarcoma pertain to the management of patients with gastrointestinal stromal tumors (GISTs) and desmoid tumors (aggressive fibromatosis). Postoperative imatinib following complete resection for primary GIST with no preoperative imatinib is now included as a category 1 recommendation for patients with intermediate or high risk of recurrence. The panel also reaffirmed the recommendation for preoperative use of imatinib in patients with GISTs that are resectable with negative margins but associated with significant surgical morbidity. Observation was included as an option for patients with resectable desmoid tumors that are small and asymptomatic, not causing morbidity, pain, or functional limitation. Sorafenib is included as an option for systemic therapy for patients with desmoid tumors.     

Pedunculated gastric tube interposition in an esophageal cancer patient with prepyloric adenocarcinoma

Gastric carcinoma is one of the malignancies that are most frequently associated with esophageal carcinoma.We describe herein our device for advanced esophageal cancer associated with early gastric cancer in the antrum.A 57-year-old man presenting with dysphagia and upper abdominal pain was admitted to our hospital.Preoperative examinations revealed locally advanced squamous cell carcinoma (SCC) of the middle thoracic esophagus (T3N0M0 Stage ⅡA) and mucosal signetring cell carcinoma of the gastric antrum (T1N0M0 Stage ⅠA).Although the gastric tumor appeared to be an intramucosal carcinoma,its margin was obscure,so endoscopic en-bloc resection was considered inadequate.We chose surgical resection of the gastric tumor as well as the esophageal SCC after neoadjuvant chemotherapy with 5-fluorouracil and cisplatin for advanced esophageal cancer.Following transthoracic esophagectomy with three-field lymph node dissection,the gastric carcinoma was removed by gastric antrectomy,which preserved the right gastroepiploic vessels,and a pedunculated short gastric tube was used as the esophageal substitute.Twenty-eight months after the surgery,the patient is well with no evidence of cancer recurrence.Because it minimizes surgical stress and organ sacrifice,gastric tube interposition is a potentially useful technique for esophageal cancer associated with localized early gastric cancer.     

HER4 is a novel prognostic biomarker in gastrointestinal stromal tumor specifically originated from stomach

HER family has been implicated in a number of malignant tumors for predicting prognosis and potential targeted therapy purposes, however, the prognostic roles of HER family in GISTs have not been elaborated yet. Our study aims to fully evaluate the prognostic value of HER family in GIST patients and efficacy of imatinib adjuvant therapy. For HER family expression detection, qPCR were used in 33 flesh GIST specimens, and then, 453 GIST samples (405 GISTs with operation only and 48 with imatinib adjuvant therapy after radical surgery) were collected for tissue microarrays construction and immunohistochemistry (IHC). Clinicopathological data were confirmed by pathological diagnosis and clinical recorders, recurrence-free survivals (RFS) were evaluated in 453 GIST patients. With qPCR and IHC performed, EGFR, HER2 and HER4 are focused on examining prognostic value in remainder of our study by high positive expression rates in GISTs. In high-risk GISTs with or without imatinib adjuvant therapy, EGFR negative expression are associated with decreased RFS when compared to positive cases. HER2 present no relationship with GIST patients’ prognosis. HER4 positive expression significantly associated with disease recurrence in GISTs. Further subgroup studies revealed HER4 was an independent prognostic indicator especially for gastric GISTs, and also for gastric high-risk GISTs. In our study, detection of EGFR expression helps to precisely subdivide high-risk GISTs for different prognosis and probably predict outcomes for imatinib treatment. HER4 is a novel independent prognostic biomarker for gastric GISTs specifically, which could be potential therapeutic target in GISTs originated from stomach.     

Pediatric gastrointestinal stromal tumors and leiomyosarcoma

BACKGROUND: With the introduction of molecularly targeted therapy for gastrointestinal stromal tumors (GISTs), it became important to distinguish GISTs from leiomyosarcomas (LMSs). The authors sought to characterize the clinicopathologic features of these tumors in pediatric patients. METHODS: The authors reviewed the medical records of 11 patients for whom GIST or LMS was diagnosed between March 1962 and July 2002 at St. Jude Children's Research Hospital and reclassified the tumors according to current histologic and immunophenotypic criteria. The authors also reviewed the literature pertaining to pediatric GISTs and LMSs. RESULTS: Seven patients had GISTs, and four had LMS. The median age of the patients at diagnosis was 11.5 years. At diagnosis, metastases were present in one patient with GISTs and in another with LMS. Unlike the focal distribution of CD117 (KIT) in LMS, diffuse and strong immunostaining was observed in GISTs. Only GISTs expressed CD34. Six patients underwent complete resection (four with GISTs and two with LMS), four patients underwent incomplete resection (three with GISTs and one with LMS), and one patient (with LMS) underwent a biopsy only. Radiotherapy or chemotherapy was used to treat one patient with GISTs and three patients with LMS. One patient with a high-risk GIST (largest dimension of 32 cm and high mitotic count) was treated with adjuvant imatinib mesylate outside the preferred setting of a clinical trial, due to concerns regarding the high risk of tumor recurrence. Four patients with GISTs and two with LMS survived median disease-free a median of 10.4 years and 4.3 years after diagnosis, respectively. Tumors in all but one survivor were completely resected. CONCLUSIONS: KIT staining helped to distinguish GISTs from LMSs. Surgery was the treatment of choice for both entities, and tumor resectability was a key prognostic factor.     

The role of surgery in the management of gastrointestinal stromal tumors (GISTs) in the era of imatinib mesylate effectiveness

Surgical resection is the treatment of choice for the gastrointestinal stromal tumors (GISTs). In the literature, the 5-year patient survival after surgical resection, ranged from 48 to 80%, before the era of imatinib mesylate and the exploration of the prognostication criteria. Imatinib mesylate targets an intracellular signaling molecule of the natural history and malignant development of GISTs, and increased the 5-year survival rate, after the resection of primary low-risk GISTs, to similar values to the normal population. For high-risk GISTs, current knowledge which is still under expansion, show major improvement at the 1-year survival rate of more than 90% versus less than 50% before imatinib era. After surgical resection, for both low and high malignant potential GISTs, a closed control directed to the early identification of confined resectable recurrences, is required. This paper assesses the current knowledge of GIST management, motivated by a case of patient with intermediate risk GIST.     

A case of recurrent esophageal cancer treated by local injection of OK-432 for 8 months and the patient survived for 2 years and 3 months

It is sometimes difficult to decide a treatment strategy for postoperative recurrence of esophageal cancer. Such recurrent esophageal cancer cases often present with extremely poor prognosis. We report a case of an 85-year-old man with a massive recurrent tumor of mediastinum 3 years after esophagectomy for squamous cell carcinoma (T3N2M0, Stage III) of the intrathoracic esophagus. The operative procedures were transhiatal esophagectomy and gastric reconstruction via posterior mediastinal route. Endoscopic local injection of OK-432 and balloon dilation was given to this patient after mediastinal recurrence. This patient lived for two years and three months after recurrence without severe side effects. Local injection of OK-432 is effective as a palliative therapy for recurrent case.     

Prognostic Factors of Gastrointestinal Stromal Tumors: A Single Institutional Retrospective Experience with Surgical Management over 20 Years

OBJECTIVE To analyze the pathological features and prognosis factors of gastrointestinal stromal tumor (GIST) after primary resection. METHODS Medical records of the diagnosis, surgery, and follow-up of 327 patients with GISTs who underwent surgery between 1988 and 2007 were retrospectively reviewed. The predic-tive factors for the survival of these patients were identi. ed using multivariate analysis. RESULTS In the 327 tumors, 152 (46.5%) were located in the stomach, 89 (27.2%) in the small intestine, 33 (10.1%) in the colon and rectum, and 43 (13.1%) in other sites including the omentum and mesentery. The 3-year and 5-year overall survival rates of the 327 GIST patients were 74.4% and 62.7%, respectively, and univariate survival analysis demonstrated that factors, such as tumor size, mitotic index, NIH categories, Ki-67 index, tumor location, surgical margins, tumor bleeding, and tumor necrosis have significant effect on survival of the patients (P < 0.05). Multivariate analysis demonstrated that the NIH categories, surgical margins, and Ki-67 index were independent prognostic factors for the survival rate. In the group of patients with postoperative recurrence or metastasis, the median survival time of patients who did not receive imatinib treatment was 30 months and that of patients who received imatinib treatment was 59 months. Their 5-year survival rates were 16.4% and 39.4%, respectively, and the difference was statistically significant (P = 0.017). CONCLUSION Complete resection is the .rst choice of treat-ment for GISTs. It is reasonable to evaluate the prognosis of resect-able GISTs and guide the adjunctive therapy with NIH categories and Ki-67 index. Imatinib treatment can signi.cantly increase the survival rate of patients with recurrent and metastatic GISTs.     

A KIT-negative, DOG1-positive epithelioid GIST of the stomach harboring a novel PDGFRA exon 14 single nucleotide deletion

Gastrointestinal stromal tumors (GISTs) are the most common primary mesenchymal tumors of the gastrointestinal tract, and most of them harbor KIT or platelet-derived growth factor receptor alpha (PDGFRA) gain-of-function mutations. Proper diagnostic assessment of GISTs has become very important since the availability of the molecular-targeted therapy with imatinib mesylate. Histopathology remains the gold standard in GIST diagnosis, and immunohistochemistry plays the major confirmatory role. Moreover, genetic sequencing not only further confirms the diagnosis of GIST, but also provides information for the optimal treatment of patients. Herein, we describe a gastric GIST harboring a novel PDGFRA exon 14 frameshift mutation caused by a single-nucleotide deletion. The case reported here represents further evidence regarding the existence of a distinct subset of GISTs characterized by the PDGFRA mutation, the gastric localisation, the epithelioid morphology, and the weak or negative immunohistochemical expression of KIT. DOG1 is emerging as a promising biomarker for this subgroup of GISTs.     

Behavior of advanced gastrointestinal stromal tumor in a patient with von-Recklinghausen disease: Case report

Gastrointestinal stromal tumors (GISTs) represent a malignant gastrointestinal tumor of neurofibromatosis type 1 (NF1) Von Recklinghausen disease. In the current case, we report a 27-year-old woman with NF1, who presented with a lower abdominal mass, symptomatic anaemia, and significant weight loss. We employed multiple approaches to assess the tumor behavior, including computed tomography (CT) scan, surgical tumor resection, histological and immunohistochemical analysis and gene sequencing. Additionally, the patient was given Imatinib mesylate (Gleevec) as adjuvant therapy. CT scan delineated a large thick wall cavity lesion connecting to the small bowel segment. Resection of the tumor yielded a mass of 17 cm × 13 cm with achievement of safety margins. The diagnosis was GIST, confirmed by immunohistochemical expression of CD117, CD34, and Bcl-2. Sequencing revealed no mutations in either KIT or platelet-derived growth factor receptor-alpha, genes which are mutated in over 85% of sporadic GIST cases. Further, there was no evidence of recurrence, metastasis or metachronous GIST for over three years in our patient. From our analyses, we believe selective genotyping is advisable for high risk patients to predict potential tumor behavior.