The epsilon toxin-activated cell signalling pathway resulting in necrosis remains unclear and involves depletion of intracellular ATP, activation of AMP-activated protein kinase, permeabilization of mitochondrial membrane and mitochondrial–nuclear translocation of apoptosis-inducing factor [119]. In the review by Dropkin and colleagues,410 five of 14 patients had developed metastases. The greater complexity of cytogenetics aberrations and rearrangements involving 17q21 in two epithelioid tumors, however, may distinguish epithelioid and nonepithelioid smooth muscle tumors.274. ROBERT A. ERLANDSON, in Modern Surgical Pathology (Second Edition), 2009. Endoscopy—endophytic lesion on gastric wall. 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. [Leiomyoblastoma (epithelioid leiomyoma) of the stomach: a pathomorphological and retrospective study of 6 cases (author's transl)]. F, Leiomyosarcoma. B, Cellular leiomyoma. The tumor is composed of densely cellular fascicles of smooth muscle cells with minimal intervening collagen. You are Here: Stanford Medicine » School of Medicine » Departments » Surgical Pathology Criteria » Gastrointestinal Tract Leiomyosarcoma The solid tumor is composed of interlacing bundles of spindle-shaped smooth muscle cells with elongated, blunt-ended, pale-staining nuclei (Fig. Leiomyoblastoma of stomach. A simple leiomyoma is composed of benign spindle cells with blunt-end, cigar-shaped nuclei and abundant pink cytoplasm. 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. The sinonasal tract is a rare site for smooth muscle neoplasms. 4-44). Capillary angioleiomyomas are composed of bundles of leiomyocytes with interspersed capillary clusters. Patients who do not respond to Gleevec can be given sunitinib (Sutent). E, Leiomyosarcoma. A more commonly used term for plexosarcoma is gastrointestinal autonomic nerve tumor (GANT). 20.36), and clear cell leiomyoma (Fig. lentigo maligna melanoma pathology outlines, Lentigo maligna is the non-invasive skin growth that some pathologists consider to be a melanoma-in-situ. Leiomyosarcomas are aggressive neoplasms characterized by local recurrences and less commonly by distant metastases. From all these studies, it is evident that further investigations are required to resolve the issue of the origin of these intriguing neoplasms. Malignant neoplasm exhibiting smooth muscle differentiation located predominantly in the subcutaneous tissues In our practice, the most frequent alternative pattern of differentiation has an epithelioid appearance, which was previously referred to as being plexiform. Background hyalinization and perivascular hyalinization may be seen. 96A.9). A few pathologists do not consider lentigo maligna to be a melanoma at all, but a precursor to melanomas. In synaptosomes and MDCK cells, the toxin receptor has been localized in lipid rafts [118]. Cavernous type vascular leiomyomas have dilated, cystic vascular spaces separated by leiomyocytes. Copyright © 2021 Elsevier B.V. or its licensors or contributors. Schematic illustration of epsilon toxin-induced vacuole formation. Nine cases were studied with respect to their clinical, radiological, and pathological features. Nuclei may be palisaded and must then be differentiated from neurilemoma, a task usually made easy by the lack of Verocay bodies and wavy, thin, spindled nuclei. On the other hand, epsilon toxin appears to target the lipid rafts in membranes [118,119]. Interlacing bundles of pleomorphic spindle cells with large nuclei, prominent nucleoli, and mitotic figures. Because this tumor is frequently hypervascular, hepatic embolotherapy has been an effective palliative treatment for unresectable liver metastases (Fig. Because imatinib mesylate (Gleevec), a tyrosine kinase inhibitor (TKI), proved to be highly effective against GISTs, TACE is currently recommended for patients who did not respond to therapy or became resistant to TKI. 59 persone ne parlano. Presents as bulky mass with central necrosis, Upper GI—smooth-lined filling defect with sharp borders. 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. Gnepp, in, Diagnostic Surgical Pathology of the Head and Neck (Second Edition), A simple leiomyoma is composed of benign spindle cells with blunt-end, cigar-shaped nuclei and abundant pink cytoplasm. Studies of leiomyoma cells have suggested that extracellular matrix constituents are an important part of their repertoire of expressed genes.262-264 In tumors with plexiform differentiation, it would seem that their capacity to synthesize extracellular matrix, particularly type I collagen (unpublished data), is particularly accentuated. retrospectively correlated pathologic features with clinical outcome.271 Similar to nonepithelioid smooth muscle tumors, no one histologic feature was predictive of metastatic potential. Venous angioleiomyomas contain numerous thick-walled blood vessels, with intervening bundles of leiomyocytes, which may merge with these vessel walls. Marc S. Levine MD, in Textbook of Gastrointestinal Radiology (Third Edition), 2008, Leiomyoblastomas consist histologically of round, polygonal, or epithelioid cells with eccentric nuclei, perinuclear vacuolization, and a clear or acidophilic cytoplasm.57–59 Because of the histologic findings, these tumors have also been called epithelioid leiomyomas.58,59 Most leiomyoblastomas are benign lesions, but metastases to the liver or other structures occur in about 10% of patients.60 As with other GISTs, malignant lesions usually have increased mitotic activity on microscopic examination.58 Size is also an important factor in predicting biologic behavior because metastases rarely occur with lesions smaller than 6 cm.58 Nevertheless, many authors believe that all leiomyoblastomas should be resected because of the difficulty in distinguishing benign and malignant lesions by histopathologic criteria.57,59, Gastric leiomyoblastomas tend to occur as solitary lesions, most frequently in the antrum, but multiple tumors have been reported.58,61 As with leiomyomas, most lesions appear as submucosal masses, often with central necrosis and ulceration.60,61 Occasionally, they may have an exogastric pattern of growth.61, Silvana Di Palma, ... Douglas R. Gnepp, in Gnepp's Diagnostic Surgical Pathology of the Head and Neck (Third Edition), 2021, A simple leiomyoma is composed of benign spindle cells with blunt-end, cigar-shaped nuclei, and abundant pink cytoplasm. Ultrastructural studies are often required to identify the GANT variant of GIST. Paul Chappell with editable outlines and mp3 files of the original sermon. Leiomyoblastoma of stomach. It was shown that membrane fluidity in the liposome plays an important role in the binding of the toxin to liposomes and oligomer formation [115]. Some (perhaps many) patients with pathology reports that were done prior to 2001 may think they have leiomyosarcoma, leiomyoma, leiomyoblastoma, or GANT when in fact their pathology slides were never tested for KIT and they might have GIST. Epsilon toxin was internalized into the cells. Department of Pathology Stanford University School of Medicine Stanford CA 94305-5342 . Endoscopic ultrasound—hypoechoic mass contiguous with muscularis propria, Heterogenous ranging from well-differentiated tumors (myoid, neural, or ganglionic) to incomplete or mixed differentiation. Figure 56.3. Recognizing the limited experience with uterine epithelioid smooth muscle tumors, we have adopted a conservative approach to their classification reflecting published criteria, summarized in Table 20.1.123,271,273, A small number of epithelioid leiomyomata have been analyzed cytogenetically to date. Visual survey of surgical pathology with 10861 high-quality images of benign and malignant neoplasms & related entities. Pincas Bitterman, in Differential Diagnosis in Surgical Pathology (Second Edition), 2010. The liver is the most common site of metastasis from malignant GISTs. Lindsay Montague, ... Jerry Elmer Bouquot, in Gnepp's Diagnostic Surgical Pathology of the Head and Neck (Third Edition), 2021. 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