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Gastrointestinal mesenchymal neoplasm

Ke-Kang Sun, Song Xu, Jinzhen Chen, Gang Liu, Xiaojun Shen, Xiaoyang Wu
Gastrointestinal stromal tumors (GISTs) are mesenchymal neoplasms that arise in the gastrointestinal tract, accounting for ~1% of gastric malignancies. The present study reports the case of a GIST of the stomach in a 75-year-old man who presented with abdominal distension and anorexia for 1 month. Gastroscopy was unremarkable. Ultrasound and computed tomography (CT) scans showed a giant intraabdominal cystic lesion of unknown origin. The lesion was initially believed to be a duplication cyst, a pancreatic pseudocyst or a liver cyst in the pre-operative diagnosis...
October 2016: Oncology Letters
Sudhir Kumar Mohanty, Kumarmani Jena, Tanmaya Mahapatra, Jyoti Ranjan Dash, Dibyasingh Meher, Ajax John, Manjushree Nayak, Shafqat Bano
INTRODUCTION: Gastrointestinal stromal tumor (GIST) is the commonest mesenchymal tumor of GI tract and 60-70% of it seen in the stomach, whereas Gastric schwannoma is a benign, slow growing and one of the rare neoplasms of stomach. Age distribution, clinical, radiological features and gross appearance of both tumors are similar. PRESENTATION OF CASE: We report a rare case of gastric schwannoma in a 20-year-old girl, who underwent subtotal gastrectomy with the suspicion of a GIST preoperatively but later confirmed to be gastric schwannoma postoperatively after immunohistochemical study...
September 22, 2016: International Journal of Surgery Case Reports
Meng-Li Gu, Ya-Mei Wang, Xin-Xin Zhou, Hang-Ping Yao, Song Zheng, Zun Xiang, Feng Ji
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasm featured by activated mutations of KIT and PDGFRA. Although overall survival rates have greatly improved by the development of receptor tyrosine kinase inhibitors, most patients ultimately acquire resistance due to secondary mutations of KIT or PDGFRA. Inhibition of the histone acetyltransferases (HATs) CREB‑binding protein (CBP) and p300 results in antineoplastic effects in various cancers. To determine whether CBP/p300 can serve as an antineoplastic target for GISTs, specific short interfering RNA sequences and the selective HAT inhibitor C646 were administered to GIST882 cells...
September 12, 2016: Oncology Reports
Yuka Kiriyama, Tetsuya Tsukamoto, Yoshikazu Mizoguchi, Shin Ishihara, Akihiko Horiguchi, Takamasa Tokoro, Yutaro Kato, Atsushi Sugioka, Makoto Kuroda
BACKGROUND: Perivascular epithelioid-cell tumor (PEComa) is a group of rare mesenchymal neoplasms that express myomelanocytic-cell markers and exhibit a wide variety of histopathological features. Although heterotopic pancreas has been reported to occur in the gastrointestinal tract, intrahepatic heterotopic pancreas has been reported only rarely. CASE PRESENTATION: We present a case of intrahepatic PEComa that showed a strong regional correlation with the presence of heterotopic pancreas...
2016: Diagnostic Pathology
Adrián Mariño-Enríquez, Judith V M G Bovée
Sarcomas are infrequent mesenchymal neoplasms characterized by notable morphological and molecular heterogeneity. Molecular studies in sarcoma provide refinements to morphologic classification, and contribute diagnostic information (frequently), prognostic stratification (rarely) and predict therapeutic response (occasionally). Herein, we summarize the major molecular mechanisms underlying sarcoma pathogenesis and present clinically useful diagnostic, prognostic and predictive molecular markers for sarcoma...
September 2016: Surgical Pathology Clinics
Guang-Ang Tian, Chun-Chao Zhu, Xiao-Xin Zhang, Lei Zhu, Xiao-Mei Yang, Shu-Heng Jiang, Rong-Kun Li, Lin Tu, Yang Wang, Chun Zhuang, Ping He, Qing Li, Xiao-Yan Cao, Hui Cao, Zhi-Gang Zhang
Gastrointestinal stromal tumor (GIST) is the most major mesenchymal neoplasm of the digestive tract. Up to now, imatinib mesylate has been used as a standard first-line treatment for irresectable and metastasized GIST patients or adjuvant treatment for advanced GIST patients who received surgical resection. However, secondary resistance to imatinib usually happens, resulting in a major obstacle in GIST successful therapy. In this study, we first found that collagen and calcium binding EGF domains 1 (CCBE1) expression gradually elevated along with the risk degree of NIH classification, and poor prognosis emerged in the CCBE1-positive patients...
2016: Scientific Reports
R J Rieker, A Marx, A Agaimy, P Ströbel
Mesenchymal neoplasms of the thymus and mediastinum account for only 2 % of neoplasms of the mediastinum and are therefore very rare. With very few exceptions the histology, immunohistochemistry and (based on current knowledge) molecular biology of mediastinal soft tissue tumors are not different from their counterparts in other organs. Characteristic features are more concerned with clinical epidemiological and therapeutic aspects as well as the multitude of possible differential diagnoses. With the exception of organ-specific tumors, such as gastrointestinal stromal tumors (GIST), virtually all entities encountered in peripheral soft tissues can also arise in the mediastinum...
September 2016: Der Pathologe
Chi Yuen Cheung, Stanley Hok King Lo, Ching Kit Chan, Fu Keung Li, Ignatius Kum Po Cheng, Ka Foon Chau
Gastrointestinal stromal tumors (GISTs) are the most common primary mesenchymal gastrointestinal neoplasms. However, GISTs occurring in kidney transplant recipients, including their treatment and outcome, are rarely described in literature. We hereby report two kidney transplant recipients with GISTs. Our first patient was diagnosed with high-risk epithelioid gastric GIST 2 years after kidney transplant. He received everolimus after resection and remained disease-free for 2 years before liver metastasis was confirmed...
July 27, 2016: Asia-Pacific Journal of Clinical Oncology
Riccardo Ricci
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of gastrointestinal tract. They feature heterogeneous triggering mechanisms, implying relevant clinical differences. The vast majority of GISTs are sporadic tumors. Rarely, however, GIST-prone syndromes occur, mostly depending on heritable GIST predisposing molecular defects involving the entire organism. These conditions need to be properly identified in order to plan appropriate diagnostic, prognostic and therapeutic procedures...
2016: Hereditary Cancer in Clinical Practice
Ryuichi Wada, Hiroki Arai, Shoko Kure, Wei-Xia Peng, Zenya Naito
Gastrointestinal stromal tumor (GIST) is a mesenchymal tumor of the gastrointestinal tract. Mutation of KIT and PDGFRA genes is implicated in the tumorigenesis. Approximately 10% of GISTs do not harbor mutation of these genes, and they are designated as "wild type" GIST. They are classified into succinate dehydrogenase (SDH)-deficient and non-SDH-deficient groups. SDH-deficient group includes Carney triad and Carney Stratakis syndrome. The patients are young women. Tumors occur in the antrum of the stomach, and tumor cells are epithelioid...
August 2016: Pathology International
Magnus Hallin, Satvinder Mudan, Khin Thway
Gastrointestinal stromal tumors (GISTs) are potentially aggressive mesenchymal neoplasms with spindle cell, epithelioid, or mixed morphology. They typically express CD117, DOG1, and CD34 and can be diffusely and strongly positive for h-caldesmon. Leiomyomas are benign smooth muscle neoplasms that can arise in a variety of visceral and soft tissue sites, including the gastrointestinal tract. We illustrate a case of a neoplasm of the gastroesophageal junction that was clinically suspected to be a GIST. Histology showed a tumor composed of ovoid and spindle cells arranged in short intersecting fascicles, which was positive for desmin, smooth muscle actin, and h-caldesmon, with a prominent interspersed subpopulation of CD117- and DOG1-positive elongated or dendritic-like cells...
July 15, 2016: International Journal of Surgical Pathology
Mehmet Tahir Oruç, Tuğrul Çakir, Arif Aslaner, Sema Çekiç, Alkan Sakar, Erdem Can Yardimci
Gastric glomus tumors (GGTs) are unusual benign, subepithelial, mesenchymal neoplasms of modified smooth muscle cells representing a neoplastic counterpart of glomus bodies. A 38-year-old woman was admitted to our clinic presenting morbid obesity. Routine preoperative evaluations, such as laboratory analysis, abdominal ultrasonography, and upper gastrointestinal endoscopy, were performed. She underwent a classical laparoscopic sleeve gastrectomy (LSG). The postoperative course was uneventful and she was discharged for outpatient control...
January 2016: Autopsy & case reports
Federica Vernuccio, Adele Taibbi, Dario Picone, Ludovico LA Grutta, Massimo Midiri, Roberto Lagalla, Giuseppe Lo Re, Tommaso Vincenzo Bartolotta
Once considered an obscure tumor entity with poor prognosis, gastrointestinal stromal tumors (GISTs) are nowadays recognized as the most common mesenchymal tumors of the alimentary tract. GISTs differ from other mesenchymal neoplasms at pathology since 90% of them exhibit strong immunohistochemical staining for KIT, a tyrosinase kinase growth factor receptor. In the early 2000s, the ability of imatinib mesylate, a tyrosine kinase inhibitor, to inhibit KIT established a new paradigm for cancer treatment. A reduction in lesion size may not be observed or may appear many months after therapy; thus, tumor response criteria alternative to the Response Evaluation Criteria in Solid Tumors were developed...
June 2016: Anticancer Research
Cláudia Paiva, Filomena Soares, Raquel da Inez Correia, Vítor Valente
INTRODUCTION: Inflammatory myofibroblastic tumor (IMFT) is an uncommon mesenchymal solid tumor commonly documented in children and young adults. It is usually located in lungs however, extrapulmonary involvement has also been reported. Here we report a case of IMFT presenting as an ileocecal intussusception. PRESENTATION OF CASE: A 55-year-old man presented with a two months history of colicky abdominal pain, more intense at the right inferior abdominal quadrant, and unintentional weight loss of 8kg in the previous four months...
2016: International Journal of Surgery Case Reports
Ashish Jakhetiya, Pankaj Kumar Garg, Gaurav Prakash, Jyoti Sharma, Rambha Pandey, Durgatosh Pandey
Gastrointestinal stromal tumours (GISTs) are mesenchymal neoplasms originating in the gastrointestinal tract, usually in the stomach or the small intestine, and rarely elsewhere in the abdomen. The malignant potential of GISTs is variable ranging from small lesions with a benign behaviour to fatal sarcomas. The majority of the tumours stain positively for the CD-117 (KIT) and discovered on GIST-1 (DOG-1 or anoctamin 1) expression, and they are characterized by the presence of a driver kinase-activating mutation in either KIT or platelet-derived growth factor receptor α...
May 27, 2016: World Journal of Gastrointestinal Surgery
Sung Jin Oh, Byoung Jo Suh, Jong Kwon Park
A schwannoma is a kind of neurogenic tumor that rarely occurs in the gastrointestinal tract. Gastric schwannomas make up 0.2% of all gastric neoplasms. Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors and up to 60-70% of GIST occur in the stomach. Schwannoma and GIST are similar in clinical features, so they are difficult to differentiate preoperatively. Differential diagnosis of these two submucosal tumors is important because of the malignant potential of GIST and the relatively benign course of gastric schwannomas...
January 2016: Case Reports in Oncology
Enrico Fiori, Daniele Ferraro, Alessandro De Cesare, Giovanni Leone, Cecilia Barmann, Alberto Schillaci, Francesco Borrini
UNLABELLED: In this article, we reviewed the case of a patient who was object, in 1999, of a published case report of schwannoma of the jejunal wall. Recently, the patient has been referred to our institution for a mass of the stomach identified by upper gastrointestinal endoscopy. The patient underwent a wedge resection of the stomach and a histopathological diagnosis of GIST of the stomach, based on a positive immunohistochemical staining of c-kit and CD34, was made. In consideration of these findings, we performed immunohistochemistry for c-kit and for CD34 on the previous lesion of the jejunal wall, which resulted strongly positive for CD117 and negative for CD34...
2016: Annali Italiani di Chirurgia
Javier Martin-Broto, Nadia Hindi
PURPOSE OF REVIEW: Sarcoma is a heterogeneous group of malignancies historically treated with classic cytotoxic chemotherapy. This review updates the recent advances in targeted therapies in soft-tissue sarcoma, bone sarcoma and other connective diseases with local aggressiveness. RECENT FINDINGS: Platelet-derived growth factor receptor (PDGFR) inhibitors, antiangiogenics, cell cycle inhibitors and immunomodulatory agents are the main targeted therapies in development in sarcoma...
July 2016: Current Opinion in Oncology
Wojciech Błogowski, Ewa Zuba-Surma, Daria Sałata, Marta Budkowska, Barbara Dołęgowska, Teresa Starzyńska
Recently, there has been a growing interest in the importance of stem cells (SCs) in the development/progression of gastric neoplasms. In this study, we performed a comprehensive analysis of different populations of bone-marrow-derived stem cells (BMSCs) in patients with various types of gastric malignancies, including gastric cancer, gastrointestinal stromal tumors (GISTs), neuroendocrine neoplasms (NENs), and lymphomas. We found significantly lower numbers of circulating Lin-/CD45 +/ CD133 + hematopoietic stem/progenitor cells (HSPCs), and intensified peripheral trafficking of both Lin-/CD45-/CXCR4+/CD34+/CD133+ very small embryonic/epiblast-like stem cells (VSELs) and CD105 + /STRO-1 +/ CD45- mesenchymal SCs (MSCs) in patients with gastric cancer, but not in those with other types of gastric neoplasms...
April 2016: Oncoimmunology
Lei Liu, Yingqiao Zhu, Dongxuan Wang, Changbin Yang, Q I Zhang, Xiukun Li, Yang Bai
Gastrointestinal stromal tumors (GIST) are mesenchymal neoplasms of the gastrointestinal tract (GI) that are defined, in part, by the expression of CD117, a c-Kit proto-oncogene protein. GISTs emerge outside of the GI at a very low frequency, typically in a single organ or location. GISTs that occasionally emerge outside of the GI are classified as extra-gastrointestinal stromal tumors (EGIST). The present study reports an extremely rare case of EGIST detected in the pancreas and the liver. The pancreatic and liver tumors were 4...
May 2016: Oncology Letters
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