Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Epithelial-myoepithelial carcinoma (EMC) of the salivary glands is a rare tumor first described by Donath et al in 1972 and recognized as a distinct pathologic entity in the 1991 WHO classification. The EMC comprises approximately 1% of all salivary gland tumors. The histological hallmark is a biphasic cell arrangement formed by an inner layer of duct-forming epithelial cells and an outer layer of myoepithelial cells. The epithelial-myoepithelial dualism is confirmed using immunohistochemical methods: the epithelial cells are immunoreactive for low molecular weight keratin; the myoepithelial cells are immunoreactive for S-100 protein, muscle specific actin and vimentin. Most reported follow-up information indicates that EMC is a low-grade malignant tumor; however, from the analysis of the various series it is demonstrated that tumors with a solid growing pattern, nuclear atypia, DNA aneuploidy and high proliferative activity, generally have a more aggressive behavior and a higher frequency of local recurrences and metastases.
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PMID:Epithelial-myoepithelial carcinoma of the salivary glands: a review of literature. 956 84

Epithelial-myoepithelial carcinoma (EMC) of the salivary glands is an uncommon, low-grade malignant tumor. A recent report demonstrates sebaceous differentiation in this tumor even though its significance has never been documented as a precise histologic variant. Six cases of EMC exhibiting sebaceous differentiation (sebaceous EMC) of the parotid gland were analyzed for their clinicopathologic features and immunohistochemical characteristics. In addition, primary salivary sebaceous carcinomas were also examined for comparison. In our series, the incidence of sebaceous EMC was 0.2% among 3012 cases of parotid gland tumors and 14.3% of all EMC cases. The 6 patients comprised 2 men and 4 women, age ranging from 77 to 93 years (mean, 83.7 y). Neither cervical lymph node nor distant organ metastases were found in any cases of sebaceous EMC and no patients died of disease, though local recurrences developed in 1 patient. Conversely, cervical lymph node metastasis was detected in 2 of 3 patients with sebaceous carcinoma, 1 of whom died of disease at 12 months. Histologically, all 6 tumors had an area of sebaceous differentiation admixed with features of bilayered ductal structures typical of EMC. A component of sebaceous differentiation was distributed diffusely in 4 tumors and focally in 2. Cytologic atypia of sebaceous EMCs was lesser than that of sebaceous carcinomas. Immunohistochemically, putative myoepithelial markers such as alpha-smooth muscle actin, calponin, p63, cytokeratin 14, S-100 protein, and vimentin were highly expressed in sebaceous EMC. However, the expression of the latter 4 markers was also observed in primary sebaceous carcinomas, whereas these tumors were all negative for alpha-smooth muscle actin and calponin. Positive immunoreactivity for epithelial membrane antigen, adipophilin, and perilipin confirmed sebaceous differentiation in EMC. These results indicate that sebaceous EMC is a low-grade malignancy, similar to conventional EMC. Our data also suggest that immunohistochemical examination of specific myoepithelial markers is helpful in distinguishing sebaceous EMC from sebaceous carcinoma, which may occasionally be associated with an aggressive clinical course.
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PMID:Sebaceous epithelial-myoepithelial carcinoma of the salivary gland: clinicopathologic and immunohistochemical analysis of 6 cases of a new histologic variant. 1842 42

Epithelial-myoepithelial carcinoma (EMC) is a rare low-grade salivary gland malignancy of presumed intercalated duct origin comprising 1% of all salivary gland tumours. High grade transformation (HGT) in EMC is a recently recognised entity with only a few cases reported in the literature. The authors report an additional case of EMC with HGT involving the submandibular gland. The patient was a 60-year-old woman who requested examination of the rapid growth of a mass in the left submandibular area, which she had first noticed 20 years previously. Histologically, the tumour had two distinct carcinomatous components. One component had features of a low grade EMC. The second component consisted of polygonal cells, arranged in a solid and nested pattern, with marked nuclear pleomorphism, brisk mitotic activity, and frequent necrosis. The Ki-67 labelling index of the EMC component was 9%, and that of the high grade component was 40%. The patient developed multiple pulmonary metastases 15 months after surgery. The aggressive behaviour of EMC with HGT suggests that it is important to recognise this variant of EMC to avoid misdiagnosis and inappropriate treatment.
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PMID:Epithelial-myoepithelial carcinoma with high grade transformation. 2220 83

Epithelial-myoepithelial carcinoma (EMC) is a rare tumor with predilection for the major salivary glands, mainly the parotid gland. It is a tumor of low grade malignancy, and despite its tendency to local recurrence with low metastatic potential, rare cases of EMC may have an aggressive behavior with distant metastasis. Treatment consists primarily of complete surgical resection whenever possible followed by radiotherapy (RT) in an attempt to prevent local recurrence. The authors describe two cases of patients with EMC from the parotid gland. Despite treatment and good local response both later developed pulmonary metastases, and also later cerebral metastization. In the sequence of these clinical cases, a literature review is made.
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PMID:[Epithelial-myoepithelial carcinoma of parotid gland: a tumor of low grade malignancy?]. 2285 11

Epithelial-myoepithelial carcinoma (EMC) of lung is a minor subset of salivary type carcinoma of lung of known low grade malignancy. Histologically, two-cell components forming duct-like structure with inner epithelial cell layer and outer myoepithelial cell layer are characteristics of EMC. In salivary gland, dedifferentiation of conventional low grade malignancy has been reported and is thought to be related with poor prognosis. However, precise histomorphology and prognostic factors of pulmonary EMC have not been clarified due to its rarity. Herein, we reported a rare case of EMC presented as endobronchial mass in a 53-year old woman, which showed predominant solid lobular growth pattern and lymph node metastases.
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PMID:A Rare Case of Bronchial Epithelial-Myoepithelial Carcinoma with Solid Lobular Growth in a 53-Year-Old Woman. 2650 39

Epithelial-myoepithelial carcinoma (EMC) is a rare subtype of salivary gland neoplasms. Since the initial description of the cancer, just over 300 cases have been reported. EMCs occupy a biphasic cellular differentiation-state defined by the constitution of two cell types representing epithelial and myoepithelial lineages, yet the functional consequence of the differentiation-state heterogeneity with respect to therapy resistance of the tumors remains unclear. The reported local recurrence rate of the cases is approximately 30%, and while distant metastases are rare, a significant fraction of these cases are reported to receive no survival benefit from radio- or chemotherapy given in addition to surgery. Moreover, no targeted therapies have been reported for these neoplasms. We report here the first use and application of ex vivo drug screening together with next generation sequencing to assess targeted treatment strategies for a rare metastatic epithelial-myoepithelial carcinoma. Results of the ex vivo drug screen demonstrate significant differential therapeutic sensitivity between the epithelial and myoepithelial intra-tumor cell lineages suggesting that differentiation-state heterogeneity within epithelial-myoepithelial carcinomas may present an outlet to partial therapeutic responses to targeted therapies including MEK and mTOR inhibitors. These results suggest that the intra-tumor lineage composition of EMC could be an important factor to be assessed when novel treatments are being evaluated for management of metastatic EMC.
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PMID:Ex vivo assessment of targeted therapies in a rare metastatic epithelial-myoepithelial carcinoma. 3264 60