Over the past two decades, primary myoepithelial neoplasms of soft tissue (as well as skin and bone) have been characterized on clinicopathologic, morphologic, and molecular grounds. BMETs have a wide age range, may involve any part of the skeleton, and have a variable spindle cell and epithelioid morphology. Here, we describe a case of clear cell myoepithelial carcinoma arising from the hard palate in an elderly male who underwent resection of the tumor and postop radiation. Myoepithelial tumor (MET) of bone is an unusual tumor of uncertain differentiation and histogenesis. A reciprocal dynamic interaction occurs between the tumor cells and their surrounding stroma under physiological and pathological conditions. It can be present in individuals of a wide age range, though many tumors are found in children. Over the past two decades, primary myoepithelial neoplasms of soft tissue (as well as skin and bone) have been characterized on clinicopathologic, morphologic, and molecular grounds. Although MET arising in soft tissue, bone, or viscera share morphologic and immunophenotypic overlap with their salivary gland and cutaneous counterparts, there is still controversy regarding their genetic relationship. A subset of myoepithelial carcinomas of soft tissue harbors EWSR1 translocations with fusion partners ZNF44, POU5F1, PBX1, and FUS. Myoepithelial carcinoma is an uncommon tumor of the salivary glands, most commonly the parotid gland. Low-grade tumors are considered to be myoepithelioma, whereas high-grade tumors are classified as myoepithelial carcinoma (when the epithelioid or spindle cells predominate), or malignant mixed tumor (when cytologically malignant cartilage or bone is present). BMETs have a wide age range, may involve any part of the skeleton, and have a variable spindle cell and epithelioid morphology. Diagnosis of a malignant myoepithelial Epithelial-myoepithelial carcinoma (EMC) of the lung is rare and is considered to be low-grade malignancy. This book comprehensively covers modern soft tissue pathology and includes both tumors and non-neoplastic entities. Soft tissues make up a large bulk of the human body, and they are susceptible to a wide range of diseases. Over the past two decades, primary myoepithelial neoplasms of soft tissue (as well as skin and bone) have been characterized on clinicopathologic, morphologic, and molecular grounds. Benign tumors are more common than malignant ones. ... – Ductal and myoepithelial component – Heterogeneous “Pleomorphic” stroma • Myxoid, Cartilage, bone, fat ... – Carcinoma ex Mixed Tumor – Salivary Duct Carcinoma – … Men and women are equally affected across all age groups and lesions arise most frequently on the extremities and limb girdles. This tumor forms a morphologic continuum with a mixed tumor and a parachordoma, but is different from an extra-axial chordoma or chordoma periphericium. Myoepithelial carcinoma (MC) is a rare type of carcinoma occurring mainly in the parotid gland, while other carcinomas occur in submandibular or the accessory glands of the oral cavity. METs that are cytologically benign are termed myoepitheliomas; METs with malignant histologic features are called myoepithelial carcinomas. Their histological features, immunohistochemical profile and biological behavior are not well characterized and pose a diagnostic challenge. Myoepithelial tumors (METs) of bone (BMETs) are a rare but distinct tumor entity. 11 The differential diagnosis of myoepithelial carcinoma of soft tissue includes ossifying fibromyxoid tumor and extraskeletal myxoid chondrosarcoma. Myoepithelial tumors (METs) of bone (BMETs) are a rare but distinct tumor entity. Glandular tumors of jaw bones present, most often, histopathologic features of salivary gland and, rarely, of cutaneous glandular neoplasms. Found inside – Page 559In contrast, myoepithelial carcinoma recur and metastasize in about 50% of cases (mainly to lungs, lymph nodes, bone, and soft tissues). Myoepithelial tumors (MET) represent a clinicopathologically heterogeneous group of tumors, ranging from benign to highly aggressive lesions. These tumors show a distinctive lobulation and in most cases a bone shell is evident. The tumor focally invaded to adhered bone tissue. Myoepithelial Tumors of ST/Bone/Lung • Morphology variable • IHC variable • EMA, S100 protein • Genetics variable • EWSR1, FUS • Many partners • Gold standard EM CCS-like Tumor of GI Tract • About 40 cases • M>F, median 41 years • Ileum > jejunum > stomach BMETs have a wide age range, may involve any part of the skeleton, and have a variable spindle cell and epithelioid morphology. This edition of ICD-O, the standard tool for coding diagnoses of neoplasms in tumour and cancer registrars and in pathology laboratories, has been developed by a working party convened by the International Agency for Research on Cancer / ... The tumors ranged from low-grade to high grade, depending on the degree of cytological atypia and abnormal cytomorphology. The genetics of myoepithelial tumors (ME) of soft tissue and bone have recently been investigated, with EWSR1-related gene fusions being seen in approximately half of the tumors. No heterotopia of the salivary gland was identified within the bone tissue. Soft tissue myoepithelial tumors were only recently recognized to occur primarily and rarely in soft tissue and skin and their clinicopathologic characteristics has been limited. Myoepithelial tumours are a heterogenous group of tumours which demonstrate myoepithelial differentiation. Molecular diagnostic workup revealed a EWSR1-KLF15 gene fusion which was previously described in only six cases of myoepithelial tumors so far. 1-3 It is considered to be relatively chemoresistant, with no standard therapy reported within the metastatic setting. This concise reference book provides an international standard for pathologists and oncologists and will serve as an indispensable guide for use in the design of studies monitoring response to therapy and clinical outcome. 133 Words1 Page. 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