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Abdom Radiol (NY) 2022 Jun 26;47(6):1988-2003. Epub 2022 Mar 26.

Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 10029, India.

Mesenchymal tumors of the stomach are uncommon, with gastrointestinal stromal tumor (GIST) being the most common among them. Majority of the tumors may arise from cells of Cajal, smooth muscle cells, neural cells, totipotent stem cells, adipocytes or fibroblasts. Imaging plays an important role not only in staging but also in characterizing these tumors. Read More

Gan To Kagaku Ryoho 2021 Dec;48(13):2055-2057

Dept. of Surgery, Osaka General Hospital of West Japan Railway Company.

The patient was a 43-year-old man. An upper gastrointestinal endoscopic examination revealed a gastric submucosal tumor(SMT)-like, elevated 8-mm lesion in the greater curvature of the upper body of the stomach. It was diagnosed as spindle cell tumor on the basis of biopsy findings, and a gastrointestinal stromal tumor(GIST)was suspected. Read More

Pathobiology 2021 4;88(6):434-442. Epub 2021 Oct 4.

Division of Pathology, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Introduction: Little information about clinical presentation of mesenchymal tumors of the lower gastrointestinal (GI) tract due to their extreme heterogeneity is available for clinical management. Usually, small solitary asymptomatic polyps are accidently found during a screening colonoscopy performed for hematochezia, abdominal pain, constipation, diarrhea, and bowel obstruction. In this case series, we illustrate our experience with mesenchymal tumors of the lower GI tract, which are a group of unusual and quite challenging lesions. Read More

J Med Case Rep 2021 Nov 16;15(1):559. Epub 2021 Nov 16.

Sarcoma Medical Oncology Clinic, BRA IRCH, All India Institute of Medical Sciences, New Delhi, India.

Background: Carney's triad is a rare syndrome comprising gastrointestinal stromal tumor, extra-adrenal paraganglioma, and pulmonary chondroma along with newer additions of adrenal adenoma and esophageal leiomyoma. The triad is completely manifest in only 25-30% cases, with most patients presenting with two out of three parts of the syndrome. Wild-type succinate-dehydrogenase-deficient gastric gastrointestinal stromal tumor forms the most common component of Carney's triad and is usually multicentric and multifocal. Read More

Surg Case Rep 2021 Jun 18;7(1):146. Epub 2021 Jun 18.

Department of Surgery, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke, Tochigi, 329-0498, Japan.

Background: Leiomyosarcoma is a rare tumor that could originate from the gastrointestinal tract, uterus, kidney, retroperitoneum, and the soft tissues of the extremities. It accounts for only 1% of all gastrointestinal mesenchymal tumors and primary leiomyosarcoma of the stomach is extremely rare. Most cases reported as leiomyosarcoma of the stomach before the development of KIT immunohistochemistry might be gastrointestinal stromal tumors (GISTs) of the stomach and only 18 cases of leiomyosarcoma of the stomach have been reported since early 2000s. Read More

Vet Radiol Ultrasound 2021 Sep 14;62(5):525-532. Epub 2021 May 14.

Department of Small Animal Clinical Sciences, College of Veterinary Medicine, University of Florida, Gainesville, Florida, USA.

Diagnosis of gastric tumors in dogs is difficult and is often obtained by biopsy following identification of a mass through ultrasound (US) or endoscopy. In human medicine, modalities such as CT and endoscopy are standard of care in the diagnosis and staging of gastric tumors. Although one veterinary study has described CT findings of gastric tumors in dogs using iatrogenic gas dilation, there are no veterinary studies that have directly compared the usefulness of US versus CT in the diagnosis and staging of these tumors. Read More

Animals (Basel) 2021 Mar 3;11(3). Epub 2021 Mar 3.

Faculty of Veterinary Medicine, University of Teramo, SP 18, 64100 Teramo, Italy.

Canine gastric disorders are common in veterinary clinical practice and among these neoplasms require rapid identification and characterization. Standard ultrasound (US) is the imaging modality of choice for gastric wall assessment. The aim of this prospective study is to describe the specific B-mode and contrast enhanced US (CEUS) features of normal, inflammatory, and neoplastic gastric wall in dogs. Read More

Cureus 2020 Sep 4;12(9):e10244. Epub 2020 Sep 4.

Pathology, Al-Noor Specialist Hospital, Makkah, SAU.

Primary mesenchymal lesions of the prostate are exceptionally rare. They comprise 1% of all prostatic neoplasms. Despite its rare location, the diagnosis of primary gastrointestinal stromal tumors (GISTs) of the prostate gland should never be missed. Read More

J Am Soc Cytopathol 2020 Nov - Dec;9(6):528-539. Epub 2020 Jun 10.

Department of Pathology, The Ohio State University Wexner Medical Center, Columbus, Ohio. Electronic address:

Introduction: Unlike epithelial malignancies, mesenchymal neoplasms arising within the tubular gut are less often encountered in endoscopic ultrasound-guided (EUS) fine-needle aspiration biopsies (FNABs). Nonetheless, preoperative diagnosis of such neoplasms has important therapeutic and prognostic value. We report our experience with this category of neoplasms from the past decade. Read More

Dig Dis Sci 2020 02 13;65(2):583-590. Epub 2019 Aug 13.

Department of Gastroenterology, Asan Medical Center, Asan Digestive Disease Research Institute, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, Korea.

Background: In cases of subepithelial tumors (SETs) located in the cardiac area, a preoperative histologic diagnosis might be helpful in determining the requirement of surgery.

Aim: To investigate the efficacy of endoscopic ultrasound-guided fine-needle biopsy (EUS-FNB) in gastric SETs located in the cardia.

Methods: The data of 107 patients who underwent EUS-FNB, from 2012 to 2017, for tissue sampling of gastric SETs located in the cardia were collected. Read More

Rev Esp Patol 2019 Jul - Sep;52(3):154-162. Epub 2018 Dec 4.

Digestive Service, Marqués de Valdecilla University Hospital, Santander, Spain.

We investigated the efficiency and accuracy of endoscopic ultrasound-guided fine-needle aspiration cytology (EUS-FNAC) in the diagnosis of gastrointestinal leiomyoma (GIL). Between January 2009 and May 2018 we performed 795 EUS-FNAC studies of lesions of the gastrointestinal (GI) tract for various clinical indications. A diagnosis of GIL by cytological and cell block study was made in 14 patients (57. Read More

Toxicol Pathol 2019 07 7;47(5):577-584. Epub 2019 May 7.

2 Division of National Toxicology Program, Cellular and Molecular Pathology Branch, National Institute of Environmental Health Sciences, Research Triangle Park, NC, USA. Venkannagari is now with the Bain and Company Inc, Boston, MA, USA. Mark is now with the Unit for Laboratory Animal Medicine, University of Michigan, Ann Arbor, MI, USA.

The majority of the tumors in the gastrointestinal (GI) tract of rats and mice, with spindle cell morphology, are diagnosed as smooth muscle tumors (SMTs). Similarly, several decades ago human GI tumors with spindle cell morphology were also diagnosed as SMTs. However, later investigations identified most of these tumors in humans as gastrointestinal stromal tumors (GISTs). Read More

Cytopathology 2018 12 12;29(6):531-536. Epub 2018 Oct 12.

Department of Pathology, Santa Casa Hospital, Porto Alegre, Brazil.

Objectives: To evaluate the diagnostic yield of the cell block (CB) technique with immunohistochemistry in patients with mesenchymal neoplasms of the gastrointestinal tract collected by endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA).

Methods: Tissue samples from consecutive patients with subepithelial lesions collected by EUS-FNA, without analysis by on-site cytopathology, were evaluated by the same pathologist only using CBs in AAF fixative. Sections were stained with haematoxylin-eosin and underwent complementary immunohistochemical staining for SMA, CD117, DOG-1 and S100 in the presence of mesenchymal neoplasms. Read More

World J Surg Oncol 2018 Jul 3;16(1):125. Epub 2018 Jul 3.

Department of Surgical Oncology, Institut Jules Bordet, Université Libre de Bruxelles, Brussels, Belgium.

Background: Schwannomas of the colon and rectum are rare among gastrointestinal schwannomas. They are usually discovered incidentally as a submucosal mass on routine colonoscopy and diagnosed on pathologic examination of the operative specimen. Little information exists on the diagnosis and management of this rare entity. Read More

Mod Pathol 2018 01;31(S1):S133-142

Robert J Tomsich Pathology and Laboratory Medicine Institute, Anatomic Pathology, Cleveland Clinic, Cleveland, OH, USA.

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. Read More

Gastrointest Endosc 2018 02 4;87(2):370-377. Epub 2017 Aug 4.

Department of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, USA.

Background And Aims: Subepithelial esophageal tumors (SETs) are frequent incidental findings. Although symptomatic tumors are surgically or endoscopically resected, there is no consensus on the management of asymptomatic esophageal leiomyomas.

Methods: Appropriate International Classification of Diseases, Ninth Revision codes followed by medical record review were used to identify patients with SETs from January 1992 to March 2017, with abstraction of basic demographics, surveillance intervals, and mortality. Read More

Rom J Morphol Embryol 2017 ;58(2):339-350

Department of Pathology, "Colentina" Clinical Hospital, Bucharest, Romania;

Since they were described, gastrointestinal stromal tumors (GISTs) are, for pathologists and not only for them, a subject of controversy regarding histological origin, differentiation, nomenclature, malignant potential and prognosis. Before 1998, there were no certainties that GISTs were fundamentally different from other types of abdominal cancers in the big family of mesenchymal tumors. Before the discovery of KIT gene mutations, GISTs were most often classified as leiomyoma, leiomyosarcoma, leiomyoblastoma, and gastrointestinal autonomic nerve tumor. Read More

Cancer Control 2015 Oct;22(4):498-504

Moffitt Cancer Center, Tampa, FL.

Background: DOG1 is a novel gene on gastrointestinal stromal tumors (GISTs) that encodes the chloride channel protein anoctamin 1, also known as discovered on GIST-1 (DOG1) protein. DOG1 antibodies are a sensitive and specific marker against GIST positive for CD117 and CD34 and negative for CD117 and CD34. DOG1 is also independent of KIT or PDGFRA mutation status and considered specific for GIST when it was first discovered in 2004. Read More

Niger J Med 2015 Apr-Jun;24(2):150-4

Background: Mesenchymal Gastrointestinal (GI) tumours are heterogenous group of tumours. Prior to 2005, immunohistochemistry (IHC) was not used in diagnostic pathology to characterize these tumours at our centre.

Objectives: To subclassify mesenchymal tumours in the GI tract and related locations into Gastrointestinal stromal tumour (GIST) and non-GIST tumours using specific IHC antibody markers. Read More

BMC Gastroenterol 2015 Aug 19;15:106. Epub 2015 Aug 19.

Department of Pathology, Faculty of Veterinary Medicine, University of Agricultural Science and Veterinary Medicine, Cluj-Napoca, 400372, Romania.

Background: Whitish intraluminal esophageal masses might represent the endoscopic feature of a bezoar or a pedunculated tumor, most likely a fibrovascular polyp, without exclusion of other mesenchymal tumors (leiomyoma, lipoma, gastrointestinal stromal tumor, leiomyosarcoma, granular cell tumor). If a process of dystrophic calcification is also encountered the differential diagnosis can be a challenge even after histological analysis, as it is highlighted by our case.

Case Presentation: A 65-year-old female whom took lactate calcium tablets for 5 years presented with progressive dysphagia. Read More

Vet Radiol Ultrasound 2015 Jul-Aug;56(4):432-8. Epub 2015 Apr 1.

University of Massachusetts, Worcester, MA, 01655.

Canine gastrointestinal stromal tumors (GISTs) are a recent subtype of gastrointestinal spindle cell tumor recognized with the increasing use of immunohistochemistry. To our knowledge, no imaging features have been described in immunostochemically confirmed canine GISTs. The objective of this retrospective, cross-sectional study was to describe ultrasonographic features of canine GISTs compared with other spindle cell tumors. Read More

Histol Histopathol 2015 May 9;30(5):581-8. Epub 2014 Dec 9.

Department of Pathology, School of Basic Medical Sciences, Peking University Third Hospital, Peking University Health Science Center, Beijing, China.

The presence of CD117 positive cells in esophageal leiomyoma may lead to a misdiagnosis of GIST. We reviewed 46 esophageal tumors which were smooth muscle tumors or GIST. Based on morphology, immunohistochemistry and mutation analysis, there were 44 (95. Read More

AJR Am J Roentgenol 2014 Aug;203(2):306-14

1 Department of Radiology, Weill Cornell Medical College, New York, NY.

Objective: The purpose of this article is to describe the imaging and clinicopathologic characteristics of esophageal gastrointestinal stromal tumors (GISTs) and to emphasize the features that differentiate esophageal GISTs from esophageal leiomyomas.

Materials And Methods: A pathology database search identified all surgically resected or biopsied esophageal GISTs, esophageal leiomyomas, and esophageal leiomyosarcomas from 1994 to 2012. Esophageal GISTs were included only if imaging studies (including CT, fluoroscopic, or (18)F-FDG PET/CT scans) and clinical data were available. Read More

Oman Med J 2014 Mar;29(2):138-41

Department of Gastroenterology, Nizwa Hospital, Sultanate of Oman.

Advances in the identification of gastrointestinal stromal tumors, its molecular and immunohiostochemical basis, and its management have been a watershed in the treatment of gastrointestinal tumors. This paradigm shift occurred over the last two decades and gastrointestinal stromal tumors have now come to be understood as rare gastrointestinal tract tumors with predictable behavior and outcome, replacing the older terminologies like leiomyoma, schwannoma or leiomyosarcoma. This report presents a case of gastric gastrointestinal stromal tumor operated recently in a 47-year-old female patient and the outcome, as well as literature review of the pathological identification, sites of origin, and factors predicting its behavior, prognosis and treatment. Read More

Hum Pathol 2014 Mar 23;45(3):481-7. Epub 2013 Oct 23.

Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka 812-8582, Japan.

Insulin-like growth factor II messenger RNA-binding protein 3 (IMP3) is a recently identified biomarker demonstrated to be useful in diagnosis and prognostic prediction for several kinds of malignant tumors. However, the clinicopathologic and diagnostic value of IMP3 in mesenchymal tumors of the gastrointestinal tract is not clear. In this study, we examined the immunohistochemical expression of IMP3 in gastrointestinal stromal tumor (GIST) (n = 150), malignant melanoma (n = 17), malignant mesothelioma (n = 6), leiomyosarcoma (n = 6), inflammatory myofibroblastic tumor (IMT) (n = 12), desmoid fibromatosis (n = 8), leiomyoma (n = 20), and schwannoma (n = 20). Read More

Int Surg 2013 Oct-Dec;98(4):315-8

1 Department of Surgery, Korea University Anam Hospital, Seoul, Korea.

Isolated colonic schwannomas are rare gastrointestinal mesenchymal tumors. Only a small number of cases have been reported. Occurrence of these tumors is more common in the stomach than in the large intestine. Read More

Hum Pathol 2013 Jun 15;44(6):1125-30. Epub 2013 Jan 15.

Department of Pathology, University of Toronto, Ontario, Canada M5S 1A8.

Sirtuin, silent mating-type information regulation 2 homolog Saccharomyces cerevisiae 1 (SIRT1), is a protein that has been implicated in multiple mammalian functions including cell aging, stress resistance, and differentiation. SIRT1 has also been shown to be involved in multiple tumors. In addition, new pharmacotherapies have recently been approved that target SIRT1. Read More

Crit Rev Oncol Hematol 2013 May 4;86(2):191-9. Epub 2012 Dec 4.

Cukurova University Medical Faculty, Department of Medical Oncology, Adana, Turkey.

Background: Neurofibromatosis type 1 (NF1) is a genetic syndrome that predisposes patients to benign and malignant tumor development. Patients with NF1 develop multiple neurofibromas that can transform into aggressive sarcomas known as malignant peripheral nerve sheath tumors. In contrast, malignant tumors unrelated to the nervous system rarely coexist with neurofibromatosis. Read More

J Hematol Oncol 2012 May 8;5:21. Epub 2012 May 8.

Division of Oncology/Hematology, New York Medical College and Westchester Medical Center, Valhalla, NY 10595, USA.

The discovery of CD117 mutation in almost all gastrointestinal stromal tumors (GISTs) marked a milestone. Other spindle cell neoplasms arising from the GI tract including lipoma, schwannoma, hemangioma, leiomyoma, and leiomyosarcoma are typically CD117-negative. GIST research and clinical care now represent a paradigm of translating discoveries in the molecular pathogenesis of cancer into highly effective targeted therapies that selectively inhibit etiologic "driver" pathways, leading to dramatically improved clinical outcomes. Read More

Nihon Rinsho 2011 Aug;69 Suppl 6:93-8

Department of Pathology, Tokyo Metropolitan Geriatric Hospital.