胃肠道基质瘤

自 2 0世纪 4 0年代Stout等描述由胃肠道起源的基质瘤以来 ,通常认为都是源于平滑肌的肿瘤 ,并分别称之为平滑肌瘤、平滑肌肉瘤、平滑肌母细胞瘤、奇异平滑肌瘤。 60年代末 70年代初电镜研究发现 ,此类肿瘤中很少见到向平滑肌分化的超微结构。 80年代初期免疫组化研究也缺乏向平滑肌分化的免疫表型证据。因此 ,有人主张统称之为胃肠道基质瘤 (gastrointestinalstromaltumor ,GIST )。但并未得到广泛的认同 ,至今文献上还经常称为胃肠道平滑肌 (肉 )瘤 (本刊在 1 996和 1 998年曾刊载相关报道 )。此外 ,某些间质肿瘤电镜下显示向神经轴分化…     

Gastrointestinal stromal tumor of the posterior mediastinum

Gastrointestinal stromal tumor (GIST) is a rare, but potentially aggressive tumor. We present an asymptomatic 64-year-old man with an incidental 9-cm GIST that arose in the posterior mediastinum. Wide surgical excision was performed with rotation of an intercostal muscle flap to buttress a surgically created esophageal wall defect. The patient is now free of disease 26 months postoperative. This tumor is defined by the carcinogenic over-expression of KIT-protein, a tyrosine kinase receptor. Accurate diagnosis of gastrointestinal stromal tumor is imperative, as specific medical therapy is now available for potential control of recurrent or metastatic disease.     

Gastrointestinal stromal tumor in ileal neobladder

Abstract  Gastrointestinal stromal tumor (GIST) is a recently described mesenchymal tumor that can develop in any portion of the gastrointestinal tract. The occurrence of a GIST in the urinary tract is rare, but GIST can present as tumor of the urinary tract or invade the urinary tract. This is the first reported case of GIST in the ileal neobladder, which presented as a submucosal tumor. The patient underwent an open exploration and partial resection of the neobladder pouch.     

Gastrointestinal stromal tumor of small intestine--case report

Gastrointestinal stromal tumor is a form of mesenchimal neoplasm that may be present in all parts of gastrointestinal system. We are reviewing diagnostic and therapeutic algorithms for patient with diagnosed gastrointestinal neoplasm of small intestine, experiencing repeated episodes of painful bleeding from gastrointestinal trackt.     

Gastrointestinal stromal tumor (gist) of the duodenum

This is a report of a rare gastrointestinal stromal tumor of the duodenum in a 75 years old man who presented with recurrent episodes of intestinal obstruction and melena. The patient underwent successful Whipple's procedure.     

Gastrointestinal stromal tumor with diffuse mesenteric metastases

Although malignant gastrointestinal stromal tumors (GISTs) sometimes show peritoneal dissemination, diffuse metastasis to only the mesentery is rare. We describe the unusual case of GIST in a 69-year-old woman who showed multiple nodules restricted only to the mesentery except the surface of the small and large bowel, omentum, and abdominal wall. These small nodules were similar to those seen in leiomyomatosis peritonealis disseminata. Histological and immunohistochemical findings were consistent with GIST. This case of GIST shows an apparently unique and rare spreading pattern.     

Gastrointestinal stromal tumor surgery and adjuvant therapy

Gastrointestinal stromal tumors (GIST) are a unique class of mesenchymal tumors identified within the past decade. Intense molecular and genetic study has been used to characterize these tumors and develop treatment strategies. Although the mainstay of treatment remains surgical resection, therapy targeted at inhibiting tyrosine kinases has had dramatic results. Because of the rapid accumulation of information about the diagnosis and treatment of these tumors, the National Comprehensive Cancer Network convened a GIST task force to provide updated recommendations in 2010. As understanding of these tumors advances, rapid changes in recommendations will continue and should warrant regular updates in tumor management.     

胃肠恶性间质瘤17例

胃肠恶性间质瘤是一组独立起源于胃肠道的间叶性肿瘤,与平滑肌肿瘤和间皮瘤不同,其基本肿瘤细胞是一种非定向分化的多潜能的梭形或上皮间叶样细胞,其中胃间质瘤最为常见,诊断困难,手术后易复发,预后不良。现将我院1996年1月至2004年9月收治的恶性间质瘤17例报告如下。1资料和方法1·1一般资料17例中男8例,女9例,年龄36~65岁。肿瘤部位:胃恶性间质瘤8例(胃底6例,胃体2例),空肠5例,回肠2例,横结肠1例,降结肠1例。就诊原因:上腹不适6例、腹痛10例、腹胀3例、黑便3例、贫血7例、腹部包块8例、血尿及肾积水1例,无任何不适于查体时才发现者1例。肿…     

Extragastrointestinal stromal tumor possibly originating from the prostate

We report a case of extragastrointestinal stromal tumor possibly originating from the prostate. The patient underwent radical prostatectomy because of no metastatic evidence. No recurrence and metastasis have been found during 14 months of follow up. To our knowledge, this may be the third such case published in a report in terms of pathological type.     

Gastrointestinal stromal tumor presenting as a scrotal mass

We describe a huge abdominal gastrointestinal stromal tumor (GIST) extending through the right inguinal canal. The reported case illustrates that the scrotum might be a place of initial presentation of a GIST growing along preformed anatomical structures. Urologists should be aware of this condition that might be mistaken for a primarily testicular malignancy.     

Gastrointestinal stromal tumor of the stomach mimicking adrenal tumor: a case report

A gastrointestinal stromal tumor of the stomach mimicking an adrenal tumor in a 67-year-old woman is reported. The patient sought medical attention for left flank pain in December 2001. A spherical calcification was evident in the left hypochondrium in an abdominal radiography, and computed tomography revealed a mass 8 cm in diameter at the upper pole of the left kidney. She then was admitted to our hospital. Physical examination and laboratory screening showed hypertension, diabetes mellitus and slight hemoconcentration. Endocrine examination showed normal serum adrenal hormone concentrations. Magnetic resonance imaging again demonstrated the mass, which showed enhancement along its margins after intravenous contrast administration. With a preoperative diagnosis of adrenal tumor, we performed total resection. The pediculated tumor, arising from the stomach, showed c-kit immunohistochemical staining permitting a histopathological diagnosis of gastrointestinal stromal tumor.     

Gastrointestinal stromal tumor with primary hepatic unique location--clinical case

The gastrointestinal stromal tumors are mesenchymal tumors whose primary extradigestive location is very rare (less than 10% primary liver localization). We present a clinical case of primary hepatic location of GIST in a 28 year-old patient. The discovery of this tumor is a chance, the patient presenting for non-specific dyspeptic syndrome and epigastralgia. During the presentation an abdominal ultrasound is performed which identifies an whell-delineated hepatic mass - 5/4 cm. Clinical and paraclinical investigations (CT, EDS, EDI, examination of the intestinal lumen with the videocapsula), confirm the diagnosis of unique hepatic mass of segments III-IV. The diagnosis is confirmed intraoperatory and we perform an atypical liver resection of segments III-IV (with 1 cm safety-margin). The histopatologic exam: GIST.     

Gastrointestinal stromal tumor of the duodenal ampulla: report of a case

We report herein the case of a 77-year-old woman with a gastrointestinal stromal tumor (GIST) of the duodenal ampulla. The tumor, which was removed by pancreatoduodenectomy due to its malignant potential, showed expansive growth in the muscular coat of the duodenal ampulla. Histologically, the tumor cells were spindle-shaped or round, and exhibited fascicular or storiform growth pattern with frequent mitotic figures. The immunohistochemical and ultrastructural results suggested that the tumor may have originated from the interstitial cells of Cajal in the muscular coat of the duodenal ampulla. To our knowledge, this is the first case of malignant GIST of the duodenal ampulla to be reported in Japan. Received: September 4, 2000 / Accepted: March 6, 2001     

Gastrointestinal stromal tumor of the lesser omentum: report of a case

We describe herein an extremely unusual case of a gastrointestinal stromal tumor (GIST) of the lesser omentum. A 45-year-old man was admitted to our hospital with an intra-abdominal mass that was subsequently misdiagnosed as a submucosal tumor of the stomach. The tumor arose from the lesser omentum and was removed without difficulty. Histologically, the tumor was composed of spindle–shaped cells with an interlacing bundle pattern, and immunohistochemical examination showed that it was positive for myeloid stem cell antigen (CD34), but negative for HHF35 and S-100 protein. These findings were consistent with a GIST lacking myogenic features and neural attributes. The patient had an uneventful postoperative course, and was free of recurrence when last seen 11 months after his operation. Received: April 3, 2000 / Accepted: March 6, 2001     

Gastrointestinal autonomic nerve tumor: immunohistochemical and molecular identity with gastrointestinal stromal tumor

Gastrointestinal autonomic nerve tumor (GANT) is a gastrointestinal neoplasm that ultrastructurally recapitulates the enteric neural plexus. This study identifies and defines the features of 10 cases of this rare mesenchymal tumor and compares its clinicopathologic and molecular genetic features with the data on gastrointestinal stromal tumor (GIST). The majority of patients in this series presented at an older age (mean 64 years). Tumors arose from the stomach (6), small intestine (2), and retroperitoneum (2). Mean tumor size was 14 cm; however, four neoplasms were <6 cm. Histologically, tumors were spindled or epithelioid; one epithelioid tumor demonstrated a previously undescribed rhabdoid histologic phenotype. All tumors were positive for CD117 (KIT), while eight of 10 were positive for CD34. In contrast, only two were positive for S-100, and all were negative for actin and desmin. Five GANTs demonstrated GIST-specific gain-of-function mutations in the juxtamembrane domain of the c-kit gene (50%). Three of 10 patients died of disease in 22-30 months, one patient died in the postoperative period, and one patient died of complications of CML. The clinicopathologic, histologic, immunohistologic, and molecular features of GANT are similar to GIST, indicating that GANT merely represents a phenotypic variant of GIST.     

Extraprostatic spindle cell stromal tumor of the prostate: case report

Several benign and malignant nonepithelial and stromal-like lesions arise in the prostate. Because such lesions are rare, their recognition is essential, because treatment and prognosis depend on an adequate pathohistologic classification. We report a case of an 83-year-old man with a stromal tumor of the prostate of uncertain malignant potential (STUMP). He presented with urinary retention and rectal constipation. On bimanual examination, a rectally and suprapubically palpable mass was found. Imaging studies revealed a 12 x 8 cm pararectal inhomogeneous mass of uncertain origin compressing rectum and urethra. The tumor was resected by a retropubic approach and examined immunohistochemically.     

Gastrointestinal stromal tumors

Gastrointestinal stromal tumors (GIST) are rare neoplasias, but they represent the most common non-epithelial (mesenchymal) tumor of the digestive tract. GISTs originate in an intestinal pacemaker cell called the intestinal cell of Cajal and express the cell-surface receptor KIT (CD117). They were first defined as a separate category in 1983, and since then important data regarding their clinical behavior, histopathological particularities, immunohistopathology, molecular biology and therapeutic possibilities were reported. In this article we aim to present a review of the information concerning this subject available in the medical literature at the moment.     

Gastrointestinal stromal tumors

The Authors report two cases of GIST and underline cytogenetic, clinic and prognostic aspects of this disease; they describe as well new histo-cyto-genetic data that identify as origin tissue interstitial cells of Cajal, by immunohistochemical and genetic tests. They also consider new diagnostic and therapeutic options based on surgical treatment. Finally controversial prognostic problems are discussed.