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A.A. refer a case report on a patient observed in emergency condition for acute bleeding from digiunal bowel by a GIST. Starting from clinical case, describe a brief profile of clinical, histogenetic, diagnostic and prognostic characteristics of the GIST also delineated by recent reports from literature; agree with other A.A. about treatment procedure represented always by resection of affected intestinal tract and never by enucleation for inconstant and unforeseeable biologic comportment by this tumours.  相似文献   

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IntroductionA gastrointestinal stromal tumor (GIST) often arises in the stomach and small intestine, while esophageal GIST is rare. The first-choice treatment is surgical resection, but there is no standard technique. Herein, we describe our experience in the treatment of esophageal GIST and discuss the usefulness of robotic esophagectomy.Presentation of caseThe patient was a 60-year-old woman, who was diagnosed with a 30 mm GIST in the middle thoracic esophagus. We underwent robot-assisted thoracoscopic esophagectomy in the prone position. The duration of the thoracoscopic part was 69 min and the total operation time was 319 min. Total blood loss was 135 ml. The patient's postoperative course was uneventful after surgery and the patient was discharged home in good condition on the 18th postoperative day.DiscussionThe prognosis of esophageal GIST was less favorable compared with gastric GIST, and due to the anatomical peculiarities of the esophagus, which surgical procedure should be performed is still under debate. Robotic surgery has several technological advantages as it provides a three-dimensional view, ten times magnification, tremor control, and ambidexterity. Therefore, Robotic-assisted minimally invasive esophagectomy (RAMIE) allows achieving for safe R0 resection of esophageal GIST.ConclusionRAMIE may be useful for esophageal GIST because it facilitates safe and minimally invasive surgery in a limited space of the thoracic cavity.  相似文献   

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INTRODUCTIONGastrointestinal stromal tumors (GISTs) of the alimentary canal are malignant tumors with <1% of cases diagnosed in esophagus. These cases require special consideration given their close proximity to vital structures and propensity to be highly aggressive. Management of patients with GISTs has been transformed since the introduction of tyrosine kinase inhibitors. In this report, we present an unusual case of GIST with spontaneous esophageal perforation.PRESENTATION OF CASEA 39-year-old Caucasian male presented to our hospital with complaints of severe chest and abdominal pain. A diagnostic chest radiograph revealed a moderate right-sided pleural effusion. Subsequently, an esophagram demonstrated a perforation proximal to an elongated stricture in the distal esophagus. A left thoracotomy was performed whereby a large mediastinal mass firmly attached to the esophagus and gastroesophageal junction was encountered. The neoplasm involved proximal one-third of the stomach and perforated into the right hemithorax. Histopathological evaluation of the tumor led to a diagnosis of GIST.DISCUSSIONGISTs of the gastroesophageal junction are uncommon and may rarely present with esophageal perforation. The standard of care for treating GIST at present includes tyrosine kinase inhibitors. This pharmacologic agent, along with improved surgical techniques and understanding of molecular markers for accurate diagnosis, will assuredly continue to improve overall survival of patients with GISTs.CONCLUSIONWhen stricture or achalasia is detected on imaging, GIST should be considered in the differential diagnosis for individual patients. Additionally, chest and abdomen CT scans of may be performed to confirm presence of a tumor mass, thereby ruling out achalasia.  相似文献   

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INTRODUCTIONThe peritoneum is one of the most common sites of distant gastrointestinal stromal tumor (GIST) metastases. In particular, GIST arising from the small intestine with resected minimal synchronous macroscopic peritoneal carcinomatosis or with primary tumor rupture has a higher risk of developing peritoneal recurrence. Current clinical practice does not envisage second-look surgery in GIST patients at high risk of developing peritoneal recurrence, and no literature data are available.PRESENTATION OF CASEWe describe a 45-year-old woman who underwent emergency surgical resection of jejunal GIST presenting with spontaneous tumor rupture, synchronous ovarian and minimal macroscopic peritoneal involvement, and subsequent second-look surgery after 13 months of imatinib treatment.DISCUSSIONSecond-look surgery confirmed a 2.6 cm lesion close to the mesenteric border of the fourth jejunal loop, and 11 peritoneal lesions with a macroscopic necrotic aspect related to treatment response. After conversion to an open procedure, a segmental jejunal resection was performed with removal of all peritoneal lesions and macroscopic radical cytoreduction.CONCLUSIONSecond-look surgery in selected GIST patients may be performed after at least 12 months of medical treatment with tyrosine-kinase inhibitors to identify those patients with limited peritoneal disease not disclosed by instrumental imaging who could undergo radical cytoreduction of peritoneal lesions.  相似文献   

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Introduction and importanceCecal gastrointestinal stromal tumors (GIST) constitute a rarer subtype of all GISTs. Rarely, it can present with ileocolic intussusception in adults making it a challenging diagnosis due to non-specific clinical features.Case presentationA 30-year previously healthy woman presented with lower abdominal pain and a distended abdomen who was subsequently diagnosed with ileocolic intussusception on a CT scan. Intraoperatively, a pedunculated polypoid hard mass was identified in the cecum and thus, a standard right hemicolectomy was performed with the suspicion of malignancy. Histopathology of the resected mass confirmed CD117 negative, spindle type GIST.DiscussionCecal GIST presenting in the form of ileocolic intussusception is rare. Contrast-enhanced CT scan is the preferred imaging modality for the evaluation of patients with suspected GIST to determine the extent of the tumor, the presence or absence of metastatic disease alongside evaluation of the possibility of complete resection. Adjuvant imatinib therapy following complete resection decreases the disease recurrence.ConclusionIntussusception in an adult can be the first manifestation of underlying malignancy like GIST. Complete surgical resection of the tumor with a negative margin offers long-term survival.  相似文献   

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十二指肠间质瘤是胃肠道间质瘤(gastrointestinal stromal tumor,GIST)中罕见部位的肿瘤,因其发生于十二指肠,毗邻肝脏、胰腺等重要器官,故在诊断和治疗上有特殊性.我院2006年收治1例,报告如下.  相似文献   

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正1型神经纤维瘤病(NF1)是一种较常见的遗传性疾病,发病率估计在1/2500~3000,由神经纤维瘤病中的多种突变引起~([1])。NF1患者发生神经、间质性和神经内分泌肿瘤的风险增加并时有报道~([2,3])。胃肠道间质瘤(GIST)为特定的KIT-或PDFGRA-信号传导的间充质肿瘤,NF1伴发GIST患者中,绝大多数肿瘤发生在空肠或回肠,极少发KIT PDGFRA  相似文献   

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Introduction and importanceGastrointestinal stromal tumors are the most prevalent mesenchymal tumors of the gastrointestinal tract. Distant metastases are most often found in the liver or peritoneum with surgery being the preferred treatment option. In our center, fluorescence-guided surgery with indocyanine green is used as standard-of-care for hepatic metastases in colorectal cancer. This case report describes fluorescence-guided metastasectomy for a hepatic gastrointestinal stromal tumor in two patients undergoing open liver resection and radiofrequency ablation.Case presentationA 69-year old women was seen during follow-up after laparoscopic resection of a GIST in the lesser curvature of the stomach. Contrast-enhanced computed tomography imaging showed two suspicious lesions in liver segment VI and VIII. Intraoperative near-infrared fluorescence imaging of the liver clearly revealed the lesion in segment VIII, and an additional lesion in segment V – which was not seen on preoperative CT-imaging, neither on intraoperative ultrasonography. The lesion in segment VI was not seen with NIRF imaging due to its deeper location in the liver parenchyma. The second case is an 82-year old man who was also diagnosed with liver metastases from a GIST in the stomach and was scheduled for near-infrared fluorescence-guided liver resection and radio frequency ablation.Clinical discussionIn this case report we demonstrated the feasibility of fluorescence-guided surgery in detection of liver metastases and treatment planning of two patients with hepatic GIST metastases using indocyanine green.ConclusionNIRF-imaging with ICG is useful for identification of preoperatively discovered lesions, surgical resection planning and margin evaluation, and for detection of additional hepatic GIST metastases.  相似文献   

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INTRODUCTION

Pancreatic neuroendocrine tumors (PNET) are rare, often indolent malignancies. PNET are classified as functional or nonfunctional based on the secretion of hormones without a negative feedback loop; the latter account for up to 60% of PNET. Although PNET are associated with a better prognosis compared to pancreatic adenocarcinomas, they are often diagnosed in advanced stages, making them a significant source of morbidity for patients. Here we present a rare case of venous tumor thrombus arising from a nonfunctional PNET.

PRESENTATION OF CASE

A 44-year-old woman was referred for evaluation and treatment of a possible tail of pancreas PNET discovered during work-up for a 9 year history of intermittent subcostal pain. Previous endoscopic ultrasound with fine needle aspiration revealed a 3.5 cm × 3 cm mass, with cytological diagnosis of neuroendocrine tumor. Patient was scheduled for laparoscopic distal pancreatectomy. During surgery the mass was found to encase the splenic vein leading the surgeon to perform an en bloc distal pancreatectomy and splenectomy. Pathologic analysis revealed a 1.8 cm × 5 cm tumor thrombus lodged in the splenic vein.

DISCUSSION

Nonfunctional PNET usually present in advanced stages and can be associated with venous tumor thrombi. Preoperative imaging may not accurately predict the presence of venous tumor thrombi.

CONCLUSION

En bloc resection of primary tumor, involved organs and thrombus is the recommended treatment option and often results in long term survival. New multi-modality strategies are needed for detection of venous involvement in nonfunctional PNET to better assist with preoperative planning and counseling.  相似文献   

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Gastrointestinal stromal tumors (GISTs) are characterized by remarkable variability in their differentiation potential, but most of these lesions do not display convincing smooth muscle or neuronal differentiation. Here we report the case of a 65-year-old woman who underwent a perfect resection of a large submucosal tumor that displayed extragastric growth. The specimen was revealed to be an elastic soft tumor, 18 x 25 x 11 cm in size. Histologically, the tumor consisted of spindle-shaped cells, with a mitotic rate of 12 per 10 high-power fields. Immunohistochemically, the tumor showed positive staining for CD34 and c-kit but negative staining for alpha-smooth muscle actin, Desmin, and s-100 protein. From these findings, the tumor was diagnosed as an uncommitted type of GIST with high-grade malignancy. This case needs careful and long-term follow-up to monitor for signs of local recurrence or distant metastasis.  相似文献   

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IntroductionGastrointestinal stromal tumors (GISTs) are clinically asymptomatic until they reach a significant size; therefore, GISTs that are 2 cm or less are typically asymptomatic. Patients with symptomatic GISTs typically present with abdominal pain, gastrointestinal bleeding, or a palpable mass but rarely present with hemoperitoneum.Presentation of caseA 72-year-old Japanese man presented to us with acute onset abdominal pain. Physical examination showed peritoneal irritation in the lower abdomen. Findings of abdominal computed tomography were suggestive of hemoperitoneum; therefore, urgent surgery was performed. Approximately 1500 ml of blood in the abdominal cavity was removed. A small, ruptured mass was found in the middle of the small intestine, and partial resection of the small intestine, including the mass, was performed. The resected tumor was 2 cm in size and exhibited an exophytic growth pattern. Immunohistochemical staining revealed that the tumor was positive for KIT and CD34; therefore, a final diagnosis of GIST was made. Treatment with imatinib at 400 mg per day was started from postoperative month 1. The patient is doing well without recurrence 5 months after surgery.DiscussionEven small GISTs in the small intestine can spontaneously rupture and cause hemoperitoneum. Moreover, when a patient presents with sudden abdominal pain and hemoperitoneum without an evident mass on imaging, clinicians should be aware of the possibility of bleeding from a small GIST in the small intestine.ConclusionWe present an extremely rare case of a patient with a small, spontaneously ruptured GIST in the small intestine, resulting in hemoperitoneum.  相似文献   

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Huang YC  Wang JY  Lin PY  Chin CC  Chen CS 《Urology》2006,68(3):672.e11-672.e13
We report a case of prostate stromal sarcoma with initial presentation of obstructive voiding symptoms. The synchronous rectal gastrointestinal stromal tumor was also finally discovered. The patient underwent pelvic exenteration as the choice of treatment. Synchronous prostate stromal sarcoma and rectal gastrointestinal stromal tumor is an extremely rare disease in the literature view.  相似文献   

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