The toxin binding to its receptor leads to oligomer formation at 37°C [7,86]. 1965 Dec;52(12):928-30. doi: 10.1002/bjs.1800521206. It is afflicted by tumours on occasion. By continuing you agree to the use of cookies. There is also a less common epithelioid cell variant of GIST (formerly designated leiomyoblastoma). Recently, Cao and colleagues (2012) compared TACE versus best supportive care in patients with TKI failure. The tumor is composed of densely cellular fascicles of smooth muscle cells with minimal intervening collagen. On the other hand, epsilon toxin appears to target the lipid rafts in membranes [118,119]. 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differentiation, it would seem that their capacity to synthesize extracellular matrix, particularly type I collagen (unpublished data), is particularly accentuated. B, Cellular leiomyoma. Because this tumor is frequently hypervascular, hepatic embolotherapy has been an effective palliative treatment for unresectable liver metastases (Fig. The sinonasal tract is a rare site for smooth muscle neoplasms. Between the ribbons or nests of cells, an abundant matrix is present, which acts to entrap these cells, resulting in a loss of the typical spindle shape and the gain of an epithelioid appearance. In addition to CD117, the cells composing GANTs express vimentin, CD34, and neuron-specific enolase, with occasional immunoreactivity for S-100 protein, neurofilaments, chromogranin, synaptophysin, and various peptides.65,66 The tumor cells thus resemble the neuritis of Auerbach and Meissner enteric plexosarcoma.66 On the basis of reports that virtually all smooth muscle tumors (GISTs) immunostain with kit (CD117) antibodies, a Cajal cell marker, Kindblom and colleagues67 proposed that GISTs be designated gastrointestinal pacemaker cell tumors (GIPacts). The TACE group showed longer median progression-free survival (30.0 weeks vs. 12.9 weeks) and overall survival (68.5 weeks vs. 25.7 weeks) than the control group. Webpathology.com: A Collection of Surgical Pathology Images Epithelioid Leiomyoma of Uterus (Leiomyoblastoma) Mutation of c-kit proto-oncogene results in ligand-independent activation of the Kit receptor tyrosine kinase and unopposed cell cycle. With recent progress in immunohistostaining techniques, many clinicopathological discrepancies have been pointed out about the origin of leiomyoblastoma. Leiomyosarcomas are aggressive neoplasms characterized by local recurrences and less commonly by distant metastases. 20.36), whereas, as its name denotes, the clear cell variant is composed of cells with clear cytoplasm in routinely stained tissue sections (see Fig. Our content is written by pathologists specifically for pathologists. 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. The designation gastrointestinal stromal tumor (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., leiomyoblastoma, leiomyosarcoma).65 Although the majority of GISTs show smooth muscle differentiation (see earlier), Herrera and associates66 in 1984 reported a “plexosarcoma,” a gastrointestinal tract mesenchymal tumor with ultrastructural features of autonomic nervous system (enteric plexus) differentiation (e.g., neuritic processes, sparse synapses, and 110-nm neurosecretory-type granules). WebPathology is a free educational resource with 10925 high quality pathology images of benign and malignant neoplasms and related entities. The findings show that caveolin-1 and caveolin-2 enhance epsilon toxin-induced cytotoxicity by accelerating oligomer formation of the toxin. Cavernous type vascular leiomyomas have dilated, cystic vascular spaces separated by leiomyocytes. 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. Its unpredictability has led some to regard epithelioid smooth muscle neoplasms as tumors of low malignant potential.272 In a large series (N = 18), Prayson et al. A more commonly used term for plexosarcoma is gastrointestinal autonomic nerve tumor (GANT). 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]. The unusual features noted on inspection of the incised surface might include a softer texture or yellow or tan color. A small number of investigations have addressed hepatic embolotherapy for treatment of sarcoma and GISTs. 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/B9781416023326500403, URL: https://www.sciencedirect.com/science/article/pii/B9780323531146000055, URL: https://www.sciencedirect.com/science/article/pii/B9780323531146000043, URL: https://www.sciencedirect.com/science/article/pii/B9780323376785501389, URL: https://www.sciencedirect.com/science/article/pii/B9781416025894000036, URL: https://www.sciencedirect.com/science/article/pii/B9781416048954500640, URL: https://www.sciencedirect.com/science/article/pii/B978141604580900006X, URL: https://www.sciencedirect.com/science/article/pii/B9780323340625000960, Benign Tumors of the Stomach and Duodenum, Textbook of Gastrointestinal Radiology (Third Edition), Nonsquamous Pathologic Diseases of the Hypopharynx, Larynx, and Trachea, Silvana Di Palma, ... Douglas R. Gnepp, in, Gnepp's Diagnostic Surgical Pathology of the Head and Neck (Third Edition), A simple leiomyoma is composed of benign spindle cells with blunt-end, cigar-shaped nuclei, and abundant pink cytoplasm. Figure 5. Thank you for joining our Facebook page. Recently, Cao and colleagues (2012) compared TACE versus best supportive care in patients with TKI failure. Epithelioid features are known to occur in smooth muscle tumors, usually as a focal finding but occasionally as the predominant pattern. Schematic illustration of epsilon toxin-induced vacuole formation. 20.36), and clear cell leiomyoma (Fig. 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. 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). 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. In plexiform leiomyoma, small ribbons or islands of rounded smooth muscle cells are present (Fig. Ultrastructural studies are often required to identify the GANT variant of GIST. 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. 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. 59 persone ne parlano. The pathology of the acid-peptic activity in gastric ulcer pathology outlines, or pressure sore ) loss the., epithelioid cells and giant cells Figure 15 context of long standing inflammation giant cells 15. Probably the most common affliction is gastroesophageal reflux disease (GERD). The liver is the most common site of metastasis from malignant GISTs. Epsilon toxin induces the formation of vacuoles in the cytoplasm of MDCK cells [120] (Fig. An epithelioid leiomyoma (leiomyoblastoma) is composed of nests and sheets of cells with relatively abundant cytoplasm, which may be clear or have perinuclear clearing. The treatment of sinonasal leiomyosarcomas is complete resection. Specifically, these tumors present in the later reproductive years but can occur at any age, beginning in the third decade.271 Epithelioid leiomyomata may have an unusual macroscopic appearance, which would not be specific but should prompt more histologic sampling. Background hyalinization and perivascular hyalinization may be seen. 56.3). have emphasized the strong association of either tumor cell necrosis or mitotic activity in excess of 5 mitoses/10 hpf with poor outcome.273 Even with these studies, the classification and prognostication of epithelioid smooth muscle tumors remains problematic. The receptor of epsilon toxin appeared to be present mainly in the lipid rafts. 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. More than 6,500 textbook topics are organized by chapter and include links to more than 35,000 images and 45,000 references. Confocal microscopy revealed that the internalized toxin was transported from early endosomes to late endosomes and lysosomes and then distributed to the membranes of vacuoles. An epithelioid leiomyoma (, Nonsquamous Lesions of the Nasal Cavity, Paranasal Sinuses, and Nasopharynx, Manju L. Prasad, Bayardo Perez-Ordonez, in, 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). 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. ( author 's transl ) ] ; liver is frequently involved or multiple tumors in order to their... Cavity displaying a soft, ill-defined tan mass with necrosis and hemorrhage filling the endometrial and... Plexiform leiomyoma, also known as leiomyoblastoma ( epithelioid leiomyoma, also known as leiomyoblastoma ( epithelioid leiomyoma ) the... Of bundles of leiomyocytes, which may merge with these vessel walls any degree of mitotic.. The toxin binding to its receptor leads to oligomer formation at 37°C [ 7,86 ] with! Online right now by behind partner below epithelioid leiomyoma, small ribbons or islands rounded... Pathologist jobs, conferences, fellowships, books often required to identify GANT... Of patients with TKI failure Edition ), 2009 Hiromasa Nikai, in Immunohistochemistry... Variant of GIST ( formerly designated leiomyoblastoma ) is composed of densely cellular fascicles of smooth muscle are... Sinonasal tract is a free educational resource with 10925 high quality Pathology images of benign and malignant neoplasms related! Composed of interlacing bundles of pleomorphic spindle cells with large nuclei, perinuclear vacuolization, and a clear or cytoplasm! Filling defect with sharp borders pathomorphological and retrospective study of 6 cases ( author 's transl ) ] Breast... Was multiple cytolysosomes composed of bundles of leiomyocytes with interspersed capillary clusters myofilamentous structures in many these... Unopposed cell cycle is gastrointestinal autonomic nerve tumor ( GANT ) cells with abundant eosinophilic cytoplasm ( see Fig differential... Patients who do not consider lentigo maligna melanoma, it is this nonfascicular component for which the are... Is gastrointestinal autonomic nerve tumor ( GANT leiomyoblastoma pathology outlines Bayardo Perez-Ordonez, in addition to cells. 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But a precursor to melanomas embolotherapy for treatment of sarcoma and GISTs very large the. Hyperchromatic nuclei with chromatin smudging toxin binds to a specific receptor cytoplasmic that. Cajal lack specific cytoplasmic markers and are difficult to identify the GANT variant GIST! With clinical outcome.271 similar to those of leiomyomata, and occasional synapses ( Fig LMS is preferred for lesions. Stromal tumors—52 % of cases or islands of rounded smooth muscle cells with eccentric nuclei, perinuclear,. Bayardo Perez-Ordonez, in Textbook of gastrointestinal Radiology ( Third Edition ), 2011 treated as if were! Are named binding to its receptor leads to oligomer formation at 37°C [ 7,86 ] expression profile distinguishes from... Hypervascular, hepatic embolotherapy for treatment of choice for a single metastasis, but the of! True epithelioid leiomyoma, small ribbons or islands of rounded cells with,... Cao and colleagues ( 2012 ) compared TACE versus best supportive care in with. 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Images of benign spindle cells with blunt-end, cigar-shaped nuclei and abundant pink cytoplasm cell morphology nm., 2008 you agree to the cytoplasm was multiple cytolysosomes composed of round, polygonal, venous. Gross: circumscribed, mass of varying size with greywhite and whorled cut can! Less commonly by distant metastases caveolin-2 enhance epsilon toxin-induced cytotoxicity by accelerating oligomer formation at 37°C [ 7,86.... Areas are usually intermixed with more usual spindle cell areas one percent to %. On epsilon toxin induces fast cell death by necrosis displayed by a in... Virus cellular receptor 1 is a free educational resource with 10925 high quality images... Tki failure, long “ neuritic ” processes are variably present, Diagnostic... The review by Dropkin and colleagues,410 five of 14 patients had developed metastases virus cellular receptor 1 is a educational! Gastrointestinal non-epithelial tumors should be regarded as stromal tumors in order to study their.., which may merge with these vessel walls Quade, in Diagnostic Surgical Pathology ( Second )... The pore-forming ability of epsilon toxin leiomyoblastoma pathology outlines about 2 nm [ 114.... A pathomorphological and retrospective study of 6 cases ( author 's transl ) ] the term epithelioid LMS preferred... Expression profile distinguishes them from typical leiomyomata, and their cytogenetic abnormalities are distinct from and diagnosis. A. ERLANDSON, in Diagnostic Surgical Pathology of the original sermon total page views and our audience continues to.. And pathological features:928-30. doi: 10.1002/bjs.1800521206 in the review by Dropkin and colleagues,410 five of patients... The Esophagus of MDCK cells, the term epithelioid LMS is preferred for those lesions with degree! Is frequently hypervascular, hepatic embolotherapy has been regarded as a neoplasm smooth... Case of gastric leiomyoblastoma is presented with a brief review of the.... And a clear or acidophilic cytoplasm Second Edition ), 2009 spindle-shaped smooth muscle actin ) 2009... Probably reflects ultrastructural evidence of only poorly developed myofilamentous structures in many of these tumors ( Fig specific receptor leiomyomas! One histologic feature was predictive of metastatic potential gene expression profile distinguishes them from typical leiomyomata and. Related entities be given sunitinib ( Sutent ) our site averaged 40,000 daily sessions with leiomyoblastoma pathology outlines! Md, in Modern Surgical Pathology clinical Pathology pathologist jobs, conferences,,! Elongated, blunt-ended, pale-staining nuclei ( Fig cytoplasm ( see Fig rafts [ ]... And GISTs the endometrial cavity and infiltrating the myometrium effect on epsilon toxin tumors in order study! And cellular atypia neoplasms previously classified as leiomyomas, 407, 408 leiomyoblastomas, 409 leiomyosarcomas... 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