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Gastrointestinal mesenchymal tumor

Shin Saito, Chao Yan, Hisashi Fukuda, Yoshinori Hosoya, Shiro Matsumoto, Daisuke Matsubara, Joji Kitayama, Alan Kawarai Lefor, Naohiro Sata
INTRODUCTION: Gastric leiomyomas are benign mesenchymal tumors, comprising about 2.5% of gastric neoplasms, which can be difficult to differentiate from gastrointestinal stromal tumors which have malignant potential. Granular cell tumors in the abdominal wall are also rare. Since mesenchymal tumors are difficult to diagnose by imaging, further studies are needed to establish the diagnosis. PRESENTATION OF CASE: A 60-year-old asymptomatic woman underwent routine upper endoscopy and was found to have a gastric submucosal lesion...
March 6, 2018: International Journal of Surgery Case Reports
Tatsuya Usui, Masashi Sakurai, Koji Umata, Hideyuki Yamawaki, Takashi Ohama, Koichi Sato
In vitro analysis of intestinal epithelium has been hindered by a lack of suitable culture systems useful for gastrointestinal research. To overcome the problem, an air liquid interface (ALI) method using a collagen gel was established to culture three-dimensional primary cells containing both primary epithelial and mesenchymal components from mouse gastrointestinal tissues. ALI organoids accurately recapitulate organ structures, multilineage differentiation, and physiology. Since ALI organoids from human tissues have not been produced, we modified the previous protocol for mouse ALI organoid culture to establish the culture system of ALI organoids from normal and tumor colorectal tissues of human patients...
February 21, 2018: Current Protocols in Toxicology
Michela Libertini, Khin Thway, Jonathan Noujaim, Florian Puls, Christina Messiou, Cyril Fisher, Robin L Jones
BACKGROUND/AIM: Clear cell sarcoma-like tumor of the gastrointestinal tract (CCSLTGT) is a very rare and relatively recently characterized mesenchymal neoplasm arising within the wall of the small bowel, stomach, or large bowel, predominantly in adolescents and young adults. Only few anecdotal reports or small series have been published and a consensus on treatment has not been formulated. Complete resection remains the only curative option for localized disease, but despite optimal surgery, CCSLTGT typically shows highly aggressive behavior with a high rate of local recurrence, metastases, and death from disease...
March 2018: Anticancer Research
Xiuju Shi, Shuxia Yu, Fenyan Wang, Qi Zhao, Hongwei Xu, Bin Li
RATIONALE: Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors involving the gastrointestinal tract. A small percentage of GISTs may cause acute gastrointestinal bleeding, which requires urgent surgical intervention. PATIENT CONCERNS: In this case report, we present a 62-year-old male patient with who was hospitalized due to acute bleeding. DIAGNOSES: The patient was diagnosed as GIST with low risk. INTERVENTIONS: The patient was treated endoscopically with polidocanol sclerotherapy...
March 2018: Medicine (Baltimore)
Paul N Manley, Suzan Abu-Abed, Richard Kirsch, Andrea Hawrysh, Nicole Perrier, Harriet Feilotter, Aaron Pollett, Robert Riddell, Lawrence Hookey, Jagdeep S Walia
Germline activating platelet derived growth factor receptor alpha (PDGFRA) mutations have been described in four families. All the index patients have presented with multiple mesenchymal tumors of the gastrointestinal tract. We identified a fifth family with four first-degree relatives that harbor a PDGFRA exon 18 (D846V) germline mutation. The affected kindred have a unique phenotype including coarse facies and skin, broad hands and feet, and previously undescribed premature tooth loss. While the index patient presented with multiple small bowel inflammatory fibroid polyps (IFPs) and has a gastric gastrointestinal stromal tumor (GIST), no tumors have yet been identified in other family members...
February 24, 2018: Human Pathology
Jun Hihara, Hidenori Mukaida, Naoki Hirabayashi
Gastrointestinal stromal tumors (GISTs) often arise in the stomach and small intestine, while esophageal GISTs are rare. Due to their rarity, clinicopathological data on esophageal GISTs are extremely limited, and this results in a lack of clear recommendations concerning optimal surgical management for esophageal GISTs. It is difficult to distinguish esophageal GIST from leiomyoma, the most frequent esophageal mesenchymal tumor, prior to resection, because the two types of tumors appear similar on computed tomography (CT), endoscopic ultrasound (EUS), and 18F-fluorodeoxyglucose positron emission tomography (FDG-PET)...
2018: Translational Gastroenterology and Hepatology
Hirohiko Kuroda, Hiroaki Saito, Yusuke Kono, Yuki Murakami, Yuji Shishido, Tomoyuki Matsunaga, Yoji Fukumoto, Tomohiro Osaki, Keigo Ashida, Kanae Nosaka, Yoshihisa Umekita, Yoshiyuki Fujiwara
Carcinosarcoma is a malignant tumor composed of both epithelial and mesenchymal malignant tumor components. A 78-year-old man was transferred to our hospital because of hematemesis and tarry stool. An emergency gastrointestinal endoscopic examination revealed active bleeding from an ulcerative lesion on the posterior wall of the gastric body; endoscopic hemostasis was successfully performed. A gastrointestinal endoscopy performed two months later showed a polypoid lesion at the same place where the ulcer had been...
December 2017: Yonago Acta Medica
Boubacar Efared, Gabrielle Atsame-Ebang, Layla Tahiri, Ibrahim Sory Sidibé, Fatimazahra Erregad, Nawal Hammas, Samia Arifi, Ihsane Mellouki, Abdelmalek Ousadden, Khalid Mazaz, Hinde El Fatemi, Laila Chbani
Background: Gastrointestinal stromal tumors (GIST) are the most common primary mesenchymal tumors of the digestive system. The assessment of their biological behavior still remains a scientific challenge. To date, there are no well-established biological prognostic markers of GIST. Our aim is to study the expression of the MDM2 oncoprotein in GIST through an immunohistochemical analysis. Methods: It was a retrospective study of 35 cases of GIST diagnosed from 2009 to 2012 in the department of pathology of Hassan II university hospital, Fès, Morocco...
2018: BMC Clinical Pathology
Robert Roskoski
Platelet-derived growth factor (PDGF) was discovered as a serum-derived component necessary for the growth of smooth muscle cells, fibroblasts, and glial cells. The PDGF family is a product of four gene products and consists of five dimeric isoforms: PDGF-AA, PDGF-BB, PDGF-CC, PDGF-DD, and the PDGF-AB heterodimer. This growth factor family plays an essential role in embryonic development and in wound healing in the adult. These growth factors mediate their effects by binding to and activating their receptor protein-tyrosine kinases, which are encoded by two genes: PDGFRA and PDGFRB...
February 2, 2018: Pharmacological Research: the Official Journal of the Italian Pharmacological Society
Kyra B Berg, David F Schaeffer
Tumor budding, defined as single cells or clusters of less than five cells, is thought to be a histomorphologic marker of an aggressive tumor behavior mimicking the embryologic epithelial-mesenchymal transition, and has been well established in the past two decades as a poor prognostic factor in colorectal carcinoma. Slow uptake in routine reporting of this important pathologic prognostic feature was in part due to differing methods of assessment of budding reported in the literature, but has recently been clarified at a consensus conference on tumor budding in colorectal carcinoma...
February 6, 2018: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
Juozas Kupcinskas
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of gastrointestinal tract. GISTs have very different clinical phenotypes and underlying molecular characteristics that are not yet completely understood. microRNAs (miRNAs) have been shown to participate in carcinogenesis pathways through post-transcriptional regulation of gene expression in different tumors. Over the last years emerging evidence has highlighted the role of miRNAs in GISTs. This review provides an overview of original research papers that analyze miRNA deregulation patterns, functional role, diagnostic, therapeutic and prognostic implications in GIST as well as provides directions for further research in the field...
January 30, 2018: International Journal of Molecular Sciences
Bo-Jing Li, Xiao-Dan Yang, Wei-Xiang Chen, Yi-Hai Shi, Zhi-Hong Nie, Jian Wu
RATIONALE: Calcifying fibrous tumor (CFT) is a rare benign soft tissue mesenchymal neoplasm. Although the gastrointestinal (GI) tract is the most common predilection site of CFT, the clinicians, even including pathologist, generally consider it as GI stromal tumor (GIST) or other submucosal tumors such as schwannoma and leiomyoma. PATIENT CONCERNS: A 55-year-old man presented with complaints of epigastric discomfort and abdominal distention for more than 1 year...
November 2017: Medicine (Baltimore)
Rong Niu, Jian-Feng Wang, Da-Chuan Zhang, Xiao-Liang Shao, Chun Qiu, Yue-Tao Wang
RATIONALE: Low-grade myofibroblastic sarcoma (LGMS) is a rare mesenchyme-derived tumor, which usually occurs in head, neck (especially tongue and mouth), and limbs. In this report, we described a case of gastric LGMS by F-fluoro-2-deoxy-d-glucose (FDG) positron emission tomography/computed tomography (PET/CT), which has not been reported previously. PATIENT CONCERNS: A 51-year-old female patient was admitted to our hospital with upper abdominal discomfort for 1 year and gradually increased eating difficulties over the last 3 months...
January 2018: Medicine (Baltimore)
Inga-Marie Schaefer, Jason L Hornick, Judith V M G Bovée
The discovery of mutations in genes encoding the metabolic enzymes isocitrate dehydrogenase (IDH), succinate dehydrogenase (SDH), and fumarate hydratase (FH) has expanded our understanding not only of altered metabolic pathways but also epigenetic dysregulation in cancer. IDH1/2 mutations occur in enchondromas and chondrosarcomas in patients with the non-hereditary enchondromatosis syndromes Ollier disease and Maffucci syndrome and in sporadic tumors. IDH1/2 mutations result in excess production of the oncometabolite (D)-2-hydroxyglutarate...
January 16, 2018: Laboratory Investigation; a Journal of Technical Methods and Pathology
Emine Elif Ozkan
OBJECTIVE: Gastrointestinal stromal tumors (GISTs) are rare mesenchymal tumors of the gastrointestinal tract, which frequently cause intraabdominal metastases. The current standard of care is surgery for localized cases, and adjuvant imatinib is recommended for tumors with a high risk of recurrence. To date, radiotherapy has not been commonly accepted as a part of multimodality treatment approach other than palliation. However, recently published case reports and some small series suggest that radiotherapy is a valuable option for controlling locally progressive, drug-resistant disease...
January 20, 2018: Chinese Medical Journal
Pieter A Boonstra, Jourik A Gietema, Albert J H Suurmeijer, Matthew R Groves, Fernando de Assis Batista, Ed Schuuring, Anna K L Reyners
Gastrointestinal stromal tumors (GISTs) are rare mesenchymal malignancies of the gastrointestinal tract. Most GISTs harbor a c-KIT (80%) or a PDGFRα (10%) mutation that leads to constitutive activation of the tyrosine kinase receptor. Response to treatment with tyrosine kinase inhibitors (TKIs) is dependent on mutational status of the tumor. The most common mutation in PDGFRα, D842V, is known to be imatinib resistant. Almost all other PDGFRα mutations are imatinib sensitive. We describe two patients with a PDGFRα exon 18 mutated GIST responding to treatment with TKIs...
December 12, 2017: Oncotarget
Yiming Chu, Qingqu Guo, Dan Wu
RATIONALE: Gastrointestinal stromal tumor and mesenteric fibromatosis are rare mesenchymal tumors. Coexistence of these two diseases is uncommon, with only a few anecdotal reports of individuals. PATIENT CONCERNS: Clinical data and treatment of a 43-year-old man with subsequent mesenteric fibromatosis from gastrointestinal stromal tumor are summarized. The Ethics Committee of The Second Affiliated Hospital, College of Medicine, Zhejiang University approved this study, and the patient provided written informed consent form...
December 2017: Medicine (Baltimore)
Jesse K McKenney
Mesenchymal tumors of the prostate are rare but often cause considerable diagnostic difficulty when encountered. These may be either benign or malignant and can arise within the prostate gland or in adjacent tissues. This review covers prostatic stromal proliferations (prostatic stromal tumor of uncertain malignant potential and stromal sarcoma), solitary fibrous tumor, myofibroblastic proliferations, smooth muscle neoplasms (leiomyoma and leiomyosarcoma), gastrointestinal stromal tumor, schwannoma, rhabdomyosarcoma, sarcomatoid carcinoma, postradiation sarcoma, and mixed epithelial stromal tumor of the seminal vesicle...
January 2018: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
Taro Aoba, Takehito Kato, Kazuhiro Hiramatsu, Yoshihisa Shibata, Motoi Yoshihara, Naoya Yamaguchi, Tadahiro Kamiya
INTRODUCTION: Gastric glomus tumors are rare submucosal mesenchymal tumors. Because gastric glomus tumors are submucosal tumors and resemble gastrointestinal stromal tumors (GISTs), preoperative diagnosis is difficult. We present a case of gastric glomus tumor that was removed by laparoscopy endoscopy cooperative surgery (LECS). CASE PRESENTATION: A 67-year-old female was taken to the emergency room at our hospital due to epigastric pain. An upper gastrointestinal endoscopy revealed a submucosal tumor (SMT) located in the lesser curvature of the upper body of the stomach...
December 16, 2017: International Journal of Surgery Case Reports
Minsun Jung, Sung-Hye Park, Yoon Kyung Jeon, Jae-Kyung Won, Han-Kwang Yang, Woo Ho Kim
RATIONALE: Gastrointestinal stromal tumor (GIST) is the most common tumor of mesenchymal origin in gastrointestinal tract. Immunohistochemical (IHC) staining combined with a typical morphology is used for the diagnosis of GIST. Typically, IHC staining for v-kit Hardy-Zuckerman 4 feline sarcoma viral oncogene (KIT) and discovered on GIST-1(DOG1) is positive in almost all GISTs. However, imatinib mesylate, a specific inhibitor of KIT tyrosine kinase, frequently involves changes in the morphology and IHC staining of GIST, impeding the diagnosis...
December 2017: Medicine (Baltimore)
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