Concomitant gastric carcinoid and gastrointestinal stromal tumors： A case report

A gastric carcinoid tumor concomitant with gastrointestinal stromal tumor （GIST） is rarely encountered in clinical practice. We report a 65-year-old female who had a 0.8 cm gastric carcinoid tumor on the posterior wall of the upper gastric corpus detected during an esophagogastroduodenoscopy at a routine physical examination, and a concomitant 1.1 cm GIST on the anterior wall of the upper gastric corpus incidentally found during surgery of the gastric carcinoid tumor. Normal serum gastrin level and histological findings suggested that she had a type 111 gastric carcinoid tumor and a GIST which were categorized a very low risk of malignancy, based on their small size and lack of mitosis. Both tumors were treated successfully by surgical excision. The patient had an uneventful recovery. Neither recurrence nor metastasis was found after a 28-mo follow-up.     

Multimodality treatment for gastric carcinoid tumor with liver metastases

Carcinoid tumors are the most common neuroendocrine tumors in the gastrointestinal tract, and between 10% and 30% of these tumors are gastric in origin. Three types of gastric carcinoid tumors are recognized: type I, associated with chronic atrophic gastritis type A; type II, associated with multiple endocrine neoplasia; and type III, sporadic and the most malignant. We present a patient with an aggressive, sporadic-type gastric carcinoid that metastasized to the liver. Her symptomatic treatment included the somatostatin analog octreotide. Octreotide scintigraphy demonstrated that this tumor avidly bound the peptide. The patient's gastric carcinoid (assessed by endoscopy and endoscopic ultrasound) regressed and she underwent hepatic artery embolization for her liver metastases. After initial partial CT resolution the tumor grew, compressing the inferior vena cava. The patient underwent orthotopic liver transplant with excellent recovery, although she was subsequently found to have two small lung metastases. She has responded well to adjuvant Indium-111 octreotide receptor targeted therapy. This case highlights the therapeutic options for metastatic neuroendocrine tumors, including liver transplantation and adjuvant receptor targeted therapy.     

胃肠间质瘤个体化治疗策略的探讨

胃肠间质瘤（GIST）是最常见的胃肠道间叶源性肿瘤，多见于胃和小肠，GIST多具有恶性潜能，生物学行为可以从良性到高度恶性不等。GIST的预后与肿瘤的发病部位、瘤体大小、核分裂数以及肿瘤是否破裂密切相关，伊马替尼辅助治疗明显延长了GIST患者的生存时间。近年对于GIST患者实施个体化治疗的理念也得到了越来越多的重视，本文就GIST个体化治疗的策略研究进行阐述。     

Gastrointestinal carcinoid tumor]

Carcinoid tumors originate from the neuroendocrine cells throughout the body and occur most frequently (74%) in the gastrointestinal tract. The clinical course is often indolent but can also be aggressive and resistant to therapy. Clinical manifestations are often vague or absent. Nevertheless, in approximately 10% of patients, the tumors secrete bioactive mediators which may engender various elements of characteristic carcinoid syndrome. In many instances, the neoplasms are detected incidentally at the time of surgery for other gastrointestinal disorders. The tendency for metastatic spread correlates with tumor size, and is substantially higher in lesions larger than 2.0 cm. Management of patients with carcinoid tumors requires an understanding of the disease process and a multimodality approach. Treatment consists of radical surgical excision of the tumor, although gastric (type I and II) and rectal carcinoids may be managed with local excision. However, advanced carcinoid tumor remains incurable.     

Laparoscopic-endoscopic cooperative surgery for gastric submucosal tumors

AIM:To assess the feasibility,safety,and advantages of minimally invasive laparoscopic-endoscopic cooperative surgery(LECS)for gastric submucosal tumors(SMT).METHODS:We retrospectively analyzed 101 consecutive patients,who had undergone partial,proximal,or distal gastrectomy using LECS for gastric SMT at Peking Union Medical College Hospital from June 2006to April 2013.All patients were followed up by visit or telephone.Clinical data,surgical approach,pathological features such as the size,location,and pathological type of each tumor;and follow-up results were analyzed.The feasibility,safety and effectiveness of LECS for gastric SMT were evaluated,especially for patients with tumors located near the cardia or pylorus.RESULTS:The 101 patients included 43(42.6%)menand 58(57.4%)women,with mean age of 51.2±13.1 years(range,14-76 years).The most common symptom was belching.Almost all(n=97)patients underwent surgery with preservation of the cardia and pylorus,with the other four patients undergoing proximal or distal gastrectomy.The mean distance from the lesion to the cardia or pylorus was 3.4±1.3 cm,and the minimum distance from the tumor edge to the cardia was 1.5 cm.Tumor pathology included gastrointestinal stromal tumor in 78 patients,leiomyoma in 13,carcinoid tumors in three,ectopic pancreas in three,lipoma in two,glomus tumor in one,and inflammatory pseudotumor in one.Tumor size ranged from 1 to8.2 cm,with 65(64.4%)lesions<2 cm,32(31.7%)>2 cm,and four>5 cm.Sixty-six lesions(65.3%)were located in the fundus,21(20.8%)in the body,10(9.9%)in the antrum,three(3.0%)in the cardia,and one(1.0%)in the pylorus.During a median follow-up of 28 mo(range,1-69 mo),none of these patients experienced recurrence or metastasis.The three patients who underwent proximal gastrectomy experienced symptoms of regurgitation and belching.CONCLUSION:Laparoscopic-endoscopic cooperative surgery is feasible and safe for patients with gastric submucosal tumor.Endoscopic intraoperative localization and support can help preserve the     

Perivascular epithelioid cell tumor of the liver coexisting with a gastrointestinal stromal tumor

Approximately 10% of patients with gastrointestinal stromal tumors （GIST）develop other neoplasms, either synchronously or metachronously. In this report we describe coexistence of a gastrointestinal stromal tumor and a hepatic perivascular epithelioid cell tumor （PEComa） in a 51-year-old woman with no evidence of tuberous sclerosis. A subcapsular hepatic nodule （0.8 cm in diameter） was found during surgery for symptomatic gastric neoplasm （15 cm in diameter） arising from the lesser curvature. Both tumors revealed histomorphological and immunohistochemical features confirming a diagnosis of a small incidental hepatic PEComa and a high risky extramural gastric GIST, respectively. The patient remained disease-free 25 mo after surgery with no evidence of tumor recurrence or new neoplasms. To our knowledge, this is the first report of PEComa in a patient with GIST. Hepatic lesions detected synchronously or metachronously in patients with GISTs may represent histogenetically distinct lesions and should be sampled to confirm or exclude metastatic GISTs.     

Synchronous Adenocarcinoma and Gastrointestinal Stromal Tumor in the Stomach

In recent years, the synchronous occurrence of tumors of different histotypes arising in the same organ has been reported more frequently in the literature. In the stomach, adenocarcinoma has been described with coexisting primary rhabdomyosarcoma, carcinoid, and low-grade B-cell lymphoma of mucosa-associated lymphoid tissue. The simultaneous development of adenocarcinoma and gastric mesenchymal tumor has been documented rarely. We report one such case. A 65-year-old male was diagnosed with a proximal gastric adenocarcinoma and underwent subtotal gastrectomy. Subsequent histopathological examination revealed the presence of another tumor at the gastric antrum. This was a gastrointestinal stromal tumor of low risk category (GIST). The literature has only a few previous reports of this very rare association. It is not known whether this synchronicity is incidental or there is a causative factor inducing the development of tumors of different histotypes in the same organ. Pathologists, oncologists and surgeons should be aware of this interesting condition.     

Synchronous occurrence of a primary colon adenocarcinoma and a gastric stromal tumor. A case report

Gastrointestinal stromal tumors are currently the object of a great clinical and experimental interest. We are presenting the case of a 69-year-old patient, who was presented with lower gastrointestinal bleeding and dyspeptic symptoms over the last six months. The colonoscopy showed a large tumor of the sigmoid and the gastroscopy a large gastric tumor of the antrum, which were histologically diagnosed as colonic adenocarcinoma and gastric stromal tumor respectively. The patient underwent a sigmoidectomy and a partial gastrectomy. Six months after surgery were the clinical condition, abdominal CT, gastroscopy and colonoscopy without pathological findings. To our best knowledge, this is the second report of a synchronous gastric stromal tumor and a colonic adenocarcinoma in medical literature.     

不同类型胃肠类癌的临床分析与比较

目的　分析不同病理类型胃肠类癌的临床特点 ,以提高对该类疾病的认识与诊治水平。方法　回顾性分析北京协和医院 1982～ 2 0 0 3年的病理分类胃肠类癌 36例 ,并根据世界卫生组织肿瘤国际组织学新分类标准 (2 0 0 0 )分为恶性类癌、类癌 2组 ;比较 2组类癌的发病年龄特点、临床表现差异、转移情况、在消化系统内分泌肿瘤和肿瘤中的发病比例以及确诊的主要手段等。结果 (1)患病比 :胃肠类癌占同期消化系统肿瘤的 0 .35 % ,占同期消化系统内分泌肿瘤的 10 .2 0 %。 (2 )年龄 :恶性类癌发病年龄为 (5 5 .8± 12 .8)岁 ,类癌发病年龄为 (45 .1± 12 .0 )岁 ,前者明显高于后者。 (3)部位 :类癌多发于直肠 (6 3% ) ,而发生于胃 (35 % )与结肠 (2 5 % )应考虑恶性类癌。(4)转移 :淋巴结与肝脏是恶性类癌常见的转移部位 (6 0 % ) ;胃与结肠部位的恶性类癌转移率明显高于直肠与其他部位。(5 )检查手段 :胃肠类癌的发现多依赖内镜检查 ,但直肠指检不应偏废。 (6 )类癌综合征的发生率低 ,恶性类癌多见。结论　胃肠类癌在消化系统肿瘤中的发病率较低 ,但并非罕见 ;直肠是类癌的好发部位 ,胃与结肠是恶性类癌的好发部位 ;恶性类癌发病年龄较大且易伴肝与淋巴结转移 ,类癌综合征多见于恶性类癌。     

Gastrointestinal stromal tumor of the stomach with a giant abscess penetrating the gastric lumen

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. In large GISTs, cystic degeneration, necrosis and focal hemorrhage that occur inside the tumor can result in gastrointestinal bleeding. We describe a case of a 74-year old male with GIST of the stomach accompanied with a giant abscess that penetrated the gastric lumen. The patient experienced undiagnosed fever for two months prior to hospitalization. Gastrointestinal endoscopy, X-ray series and computed tomography of the patient’s abdomen revealed a gastric submucosal tumor in the fornix, with a fistula to the gastric lumen that was inundated with a great deal of pus. The mass was diagnosed as a GIST from biopsy specimens. The patient was treated by endoscopic drainage of the abscess and intravenous administration of antibiotics. Eventually, a partial gastrectomy was performed. He was also administered Imanitib mesylate as adjuvant therapy. He was followed up for 2 years and no metastasis or recurrence was recognized at the follow- up examinations. This is the first report of a patient with clearly diagnosed GIST with endoscopic evidence of an abscess penetrating into the gastric lumen.     

消化道黏膜下肿物的内镜及超声内镜下特点分析

目的探讨消化道黏膜下肿物的内镜及超声内镜下特点。方法对153例消化道黏膜下肿物进行内镜及超声内镜检查,最终经病理学及免疫组化明确诊断。结果确诊消化道平滑肌瘤51例、胃肠道间质瘤37例、脂肪瘤30例、类癌21例、异位胰腺7例、神经鞘瘤3例、错构瘤3例和血管球瘤1例。超声内镜对平滑肌瘤、间质瘤、脂肪瘤、类癌、异位胰腺、神经鞘瘤、错构瘤和血管球瘤的诊断符合率分别为92%、92%、100%、71%、71%、0、0和100%,其总诊断符合率为86%,明显高于内镜总诊断符合率(70%)。结论超声内镜可明确消化道黏膜下肿物的大小、起源、回声、生长方式,对消化道黏膜下肿物的诊断及鉴别诊断有重要作用。     

内镜超声在消化道黏膜下肿瘤诊断与治疗中的价值

目的 评价内镜超声在消化道黏膜下肿瘤的诊断及治疗中的价值.方法 对内镜检查中怀疑黏膜下肿瘤者进行内镜超声检查,根据黏膜下肿瘤的起源层次及性质决定治疗方案,内镜治疗包括内镜下黏膜切除术、黏膜剥离-肿瘤摘除术、高频电切术及硬化治疗.结果 73例良性间质瘤起源于黏膜肌层,7例直肠类癌位于黏膜固有层;脂肪瘤13例、异位胰腺17例、胃底静脉曲张5例、囊肿6例起源于黏膜下层;95例良性间质瘤及21例恶性间质瘤起源于固有肌层,1例类癌侵及固有肌层.61例源于黏膜肌层及8例源于固有肌层的良性间质瘤、8例脂肪瘤、8例异位胰腺及7例类癌经内镜切除,4例囊肿行内镜下穿刺治疗;33例源于固有肌层的良性间质瘤、18例恶性间质瘤、2例脂肪瘤、2例异位胰腺及1例类癌经手术切除.病理符合率为97.97%.结论 超声内镜能够对消化道黏膜下肿瘤进行起源和定性诊断,对黏膜下肿瘤治疗方案的选择具有重要的指导意义.     

Treatment of solitary gastric carcinoid tumor by endoscopic polypectomy in a patient with pernicious anemia

Type I gastric carcinoid tumors result from hypergastrinemia in 1%-7% of patients with pernicious anemia. We diagnosed pernicious anemia in a 48-year-old female patient with complaint of fatigue for three months. She had no gastrointestinal symptoms. Endoscopic examination ot the upper gastrointestinal tract revealed atrophic gastritis and a polypoid lesion in the corpus of 3-4 mm in size. Endoscopic polypectomy was performed. Histopathological examination of the specimen revealed positive chromogranin A and synaptophysin stainings compatible with the diagnosis of a carcinoid tumor. Serum gastrin level was increased, urinary 5-hydroxyindoleacetic acid was within the normal range. There was no other symptom, sign, or laboratory finding of a carcinoid syndrome in the patient. No metastasis was found with indium-111 octreotide scan, computed tomographies of abdomen and thorax. Type I gastric carcinoid tumors are only rarely solitary and patients with tumors < 1 cm in size may benefit from endoscopic polypectomy.     

内镜下胃间质瘤检出情况探讨

目的探讨首都医科大学附属北京友谊医院消化内镜中心胃间质瘤内镜下的检出率。方法对2004年5月～2010年5月于首都医科大学附属北京友谊医院消化内镜中心行胃镜检查发现胃黏膜下隆起性病变而进行超声检查的病例进行回顾性分析,计算胃间质瘤检出的阳性率。结果6年间胃镜检查共计52368例,胃黏膜下隆起病变461例。其中240例行超声内镜检查,怀疑胃间质瘤161例,其中切除治疗36例,术后病理证实33例。估计胃间质瘤的内镜下检出率约为0．5％。结论消化内镜及超声内镜是胃间质瘤重要的诊断方法,对选择合适的治疗方案具有指导意义。     

Minute carcinoid tumor of the stomach: report of two cases and review of the Japanese literature

Two cases of minute carcinoid tumor of the stomach were reported and 167 cases of gastric carcinoid in Japan up to December 1984 were reviewed. In Case 1, two carcinoid tumors of the body were found as sessile polyps of 4.2 and 4.8 mm in diameter, respectively, the latter having been detected two years previously. Case 2 had 60 polypoid lesions on the body and the fundus, ranging from 1.0 to 4.5 mm in diameter. In both cases the tumor invasion was limited to the mucosa and submucosa, although many micronests were found around the polypoid lesions. Among 167 cases of gastric carcinoid in Japan, 12 were 5 mm or less in size and 12 were 6 to 10 mm in size. In the former cases, there was no metastasis while in the latter, metastasis to the lymph nodes was found in 25.0%. We propose that the minute gastric carcinoid should be defined as a tumor not exceeding 5 mm in size with the invasion limited to the mucosa or submucosa. In addition, we emphasized that radical gastrectomy should be performed even for the minute carcinoid tumor because of its multicentricity and invasiveness.     

Small gastrointestinal stromal tumor concomitant with early gastric cancer： A case report

The term gastrointestinal stromal tumors (GISTs)is defined diagnostically as the main group of mesenchymal tumors with spindle or epithelioid cells arising from the wall of the gastrointestinal tract with immunohistochemical reactivity for CD117 antibody.Previous studies revealed that cells in GISTs express a growth factor receptor with tyrosine kinase activity (termed c-kit), which is the product of the c-kit protooncogene. The most specific and practical diagnostic criteria for GISTs are: immunohistochemically determined c-kit (CD117) expression; mitotic score; and tumor size.A small GIST concomitant with early gastric cancer is rarely encountered clinically. Herein we have reported a case of a 1.1-cm GIST detected by esophagogastroduo denoscopy concomitant with a Ⅱc type of early gastric cancer (signet ring cell type). It was detected during a routine physical health examination. To our knowledge,this is the first report of a small GIST concomitant with a signet ring cell type of early gastric cancer.     

Clinicopathological features of gastric glomus tumor

AIM:To study the clinicopathological features of gastric glomus tumor and review the related Chinese literature published in 1990-2010.METHODS:A case of gastric glomus tumor was re-ported.Clinicopathological findings in 56 cases of gastric glomus tumor were analyzed.RESULTS:Gastric glomus tumor was far more common in women than in men with a female to male ratio of 1.6:1.The median age of the patients was 45 years(range 28-79 years).The patients often complained of epigastric pain and bloody stool.The tumor was located in antrum of the stomach.The greatest diameter of the tumor was 0.8-11cm.Histologically,the tumor was comprised of nests of glomus cells surrounding the capillaries.Glomus cells were small,uniform and round.Vimentin,smooth muscle actin and actin were expressed in the tumor.Other markers,including S-100 protein,CD34,CD117,desmin,CD56,synaptophysin,chromo-granin A,neuron specific enolase and cytokeratin were all negative.CONCLUSION:Gastric glomus tumor is a rare benign mesenchymal neoplasm.Its diagnosis depends on pathologic examination.Differential diagnosis includes gastrointestinal stromal tumor,paraganglioma and carcinoid tumor.     

Unusual Gastrointestinal Hemorrhaging Mimicking a Rupture of Solitary Gastric Varices Due to a Gastric Gastrointestinal Stromal Tumor with Exogenous Growth

Gastric gastrointestinal stromal tumors can lead to upper gastrointestinal hemorrhaging, which is usually caused by dimpling or ulceration on the tumor surface. While rare, pedunculated gastric gastrointestinal stromal tumors outside the stomach can present as a huge mass with delayed complaints. We herein report an unusual hemorrhaging mimicking a rupture of solitary gastric varices due to a pedunculated gastric gastrointestinal stromal tumor. In this case, contrast-enhanced computed tomography (CECT) was essential for tumor detection. An endoscopic investigation revealed dilated, aberrant veins and arteries in the submucosa of this tumor, recognized as solitary gastric varices.     

罕见病理类型胃间质瘤——附文献复习

胃肠道间质瘤为最常见的胃肠道非上皮性肿瘤。大多数GIST病理免疫染色KIT（C-kit原癌蛋白）阳性。产生编码第三型受体型酪胺酸激酶的突变基因，包括c-kit或PDGFRA。多数胃肠道间质瘤临床上无症状，好发于老年。本文报道一例PDGFRA外显子18突变的胃肠道间质瘤。并对胃肠道间质瘤的临床、病理特点及预后加以综述。     

Stromal tumor of the pancreas with expression of c-kit protein: report of a case

We report on a case of a stromal tumor, similar to a gastrointestinal stromal tumor, originating from the pancreas. The patient was a 54-year-old woman, who was seen at the Kofu Municipal Hospital because of an abdominal tumor. On abdominal computed tomography and splenic arteriography, the tumor was detected in the pancreatic tail. The patient underwent distal pancreatectomy with splenectomy. Macroscopically, the cut surface of the tumor showed almost completely surrounded by the normal pancreatic tissue. Microscopically, the tumor composed of spindle-shaped cells that were immunoreactive for vimentin, CD34, and c-kit protein. Therefore, the tumor was diagnosed as a stromal tumor of the pancreas. The expression of c-kit protein suggests that this pancreatic stromal tumor may originate from primitive mesenchymal cells which can be a logical candidate for the origin of gastrointestinal stromal tumors and extra-gastrointestinal stromal tumors.