An epithelioid leiomyoma (leiomyoblastoma) is composed of nests and sheets of cells with relatively abundant cytoplasm, which may be clear or have perinuclear clearing. Most of these tumours are biologically benign, but some show malignant changes. Negative Desmin staining is seen infrequently. Based on immunohistochemical and ultrastructural studies, it has been suggested, perhaps incorrectly, that this tumor mimics fetal myocytes.268,269 Both epithelioid and clear cell leiomyomata are characterized by rounded or polygonal cells, rather than by spindle cells. Wardelmann and colleagues69 found that c-kit mutations were absent in seven cases they examined with an epithelioid component, whereas all GISTs with a spindle cell histology expressed this mutation. Leiomyoblastoma is not at all a common tumor especially in old age. Figure 4. Cell membranes may be especially prominent. Patients with unresectable or metastatic disease are given imatinib mesylate (Gleevec), an oral tyrosine kinase inhibitor that targets c-kit, and then reevaluated for potential resection if they respond to this treatment. Leiomyoblastoma of the stomach: endoscopic treatment. Leiomyocytes express muscle specific actin, desmin, and vimentin, are usually S100 negative, and ultrastructurally are characterized by elongated, clefted nuclei, thin myofilaments with dense bodies, pinocytotic vesicles, and basal lamina. ROBERT A. ERLANDSON, in Modern Surgical Pathology (Second Edition), 2009. The cytotoxicity is linked with a depletion of intracellular K+, and an increase in Cl− and Na+, but the increase in Ca2+ develops later [114]. 59 persone ne parlano. Lee and associates68 found that the clinicopathologic, histologic, immunohistologic, and molecular features of GANT are similar to GIST, indicating that GANT merely represents a phenotypic variant of GIST. Leiomyoblastoma has been regarded as a neoplasm of smooth muscle origin. Department of Pathology Stanford University School of Medicine Stanford CA 94305-5342 . These processes contain variable numbers of microtubules, dense-core neurosecretory-type granules, and occasional synapses (Fig. Original posting/updates: 12/5/09, 11/27/09 . In the review by Dropkin and colleagues,410 five of 14 patients had developed metastases. Capillary angioleiomyomas are composed of bundles of leiomyocytes with interspersed capillary clusters. Although pathologies affecting the esophagus are diverse they tend to Khan MY, Schweitzer EJ, Mahal PS. Margaret S. Brandwein-Gensler, ... Douglas R. Gnepp, in Diagnostic Surgical Pathology of the Head and Neck (Second Edition), 2009. We use cookies to help provide and enhance our service and tailor content and ads. Mutation of c-kit proto-oncogene results in ligand-independent activation of the Kit receptor tyrosine kinase and unopposed cell cycle. Cavernous type vascular leiomyomas have dilated, cystic vascular spaces separated by leiomyocytes. There is also a less common epithelioid cell variant of GIST (formerly designated leiomyoblastoma). ScienceDirect ® is a registered trademark of Elsevier B.V. ScienceDirect ® is a registered trademark of Elsevier B.V. URL: https://www.sciencedirect.com/science/article/pii/B978141602589400005X, URL: https://www.sciencedirect.com/science/article/pii/B9781416025894000048, URL: https://www.sciencedirect.com/science/article/pii/B9781416025894000036, URL: https://www.sciencedirect.com/science/article/pii/B978141604580900006X, URL: https://www.sciencedirect.com/science/article/pii/B9780323340625000960, URL: https://www.sciencedirect.com/science/article/pii/B978141605766600008X, URL: https://www.sciencedirect.com/science/article/pii/B9780123971692000561, URL: https://www.sciencedirect.com/science/article/pii/B9781416039662000059, URL: https://www.sciencedirect.com/science/article/pii/B9781416045809000125, URL: https://www.sciencedirect.com/science/article/pii/B9780323447324000200, Nonsquamous Pathologic Diseases of the Hypopharynx, Larynx, and Trachea, Margaret S. Brandwein-Gensler, ... Douglas R. 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