It has been reported that the receptor of epsilon toxin is correlated to a 34- or 46-kDa protein or glycoprotein in MDCK cells and to a 26-kDa sialyglycoprotein in rat brain [7]. An epithelioid leiomyoma (, Lindsay Montague, ... Jerry Elmer Bouquot, in, Nonsquamous Lesions of the Nasal Cavity, Paranasal Sinuses, and Nasopharynx, Manju L. Prasad, Bayardo Perez-Ordonez, in, Diagnostic Surgical Pathology of the Head and Neck (Second Edition), The Mont Reid Surgical Handbook (Sixth Edition), Differential Diagnosis in Surgical Pathology (Second Edition), Hepatic artery embolization and chemoembolization of liver tumors, Blumgart's Surgery of the Liver, Biliary Tract and Pancreas, 2-Volume Set (Sixth Edition). (GIST) encompasses a variety of nonepithelial neoplasms most commonly originating in the wall of the stomach and small intestine, most of which were formerly considered to be smooth muscle tumors (e.g., Brooke E. Howitt, ... Bradley J. Quade, in, Diagnostic Gynecologic and Obstetric Pathology (Third Edition), In tumors with plexiform differentiation, it would seem that their capacity to synthesize extracellular matrix, particularly type I collagen (unpublished data), is particularly accentuated. However, it is not yet clear whether hepatitis A virus cellular receptor 1 is a functional receptor of epsilon toxin. Leiomyoblastoma of stomach Br J Surg. Epithelioid leiomyosarcoma (malignant leiomyoblastoma) of the colon. Epsilon toxin added to lipid bilayer induces an increase in conductance across the membrane in a stepwise fashion after about 2 min [114]. Figure 2. In Diagnostic Pathology: Molecular Oncology, 2016, Gastrointestinal leiomyoma/leiomyoblastoma, gastrointestinal autonomic nerve tumor (GANT), Used in older literature but are no longer considered accurate terminology, Mural-based mesenchymal tumor of gastrointestinal (GI) tract with characteristic histologic, immunohistochemical, and molecular features, Composed of spindled &/or epithelioid cells, CD117 &/or DOG1 immunohistochemical positivity, 85-90% driven by mutually exclusive KIT or PDGFRA mutations, Rare autosomal dominant germline KIT or, less commonly, PDGFRA mutations define hereditary GIST syndrome, Most demonstrate lack of function of succinate dehydrogenase (SDH) complex (SDH-deficient GISTs), Majority are sporadic and thus occur in absence of known syndrome, 10-20% are seen in Carney-Stratakis syndrome and Carney triad, Comprise 7-13% of all wild-type GISTs in stomach and small intestine, Few wild-type GISTs arise in patients with neurofibromatosis type 1 (NF1), Manju L. Prasad, Bayardo Perez-Ordonez, in Diagnostic Surgical Pathology of the Head and Neck (Second Edition), 2009, The sinonasal tract is a rare site for smooth muscle neoplasms. Molecular studies have indicated that their gene expression profile distinguishes them from typical leiomyomata, and their cytogenetic abnormalities are distinct from. Nine cases were studied with respect to their clinical, radiological, and pathological features. The role of radiotherapy is controversial.408,410, Jean E. Blair, Robert E. Petras, in Differential Diagnosis in Surgical Pathology (Second Edition), 2010, Historically, spindle cell neoplasms of the gastrointestinal tract were thought to arise from smooth muscle and were thus termed leiomyoma, leiomyosarcoma, or leiomyoblastoma, Subsequently, ultrastructural and immunohistochemical studies demonstrated that cells composing these tumors were either undifferentiated or only rarely showed evidence of smooth muscle or neural differentiation, or both, Currently most of these stromal tumors are thought to arise from or are differentiated toward interstitial cell of Cajal; a cell that may control motility (intercalating between autonomic nerves and muscle cells), possibly explaining the prior studies showing neural and muscle differentiation, Currently, stromal tumors of the gastrointestinal tract are generally split into two groups, Recognizable diagnostic entities identical to soft tissue tumors found elsewhere in the body (e.g., schwannoma, leiomyoma), Spindle cell neoplasms, most of which overexpress CD117 (c-Kit) and are referred to as gastrointestinal stromal tumors (GISTs), Account for 0.1% to 1% of all gastrointestinal tumors, Histologic features overlap with other mesenchymal tumors, Most common in the stomach and small intestine, Most patients are older (50 to 70 years of age), About 50% of these tumors ulcerate and bleed, The tumor may occur in young women (<20 years) alone or may be associated with Carney triad, which includes, Chang Jin Yoon, Jin Wook Chung, in Blumgart's Surgery of the Liver, Biliary Tract and Pancreas, 2-Volume Set (Sixth Edition), 2017. 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