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Head and Neck Pathology Jul 2013Myoepithelial neoplasms are tumors composed almost exclusively of cells with myoepithelial differentiation. They frequently contain spindle, plasmacytoid or epithelioid... (Review)
Review
Myoepithelial neoplasms are tumors composed almost exclusively of cells with myoepithelial differentiation. They frequently contain spindle, plasmacytoid or epithelioid shaped cells and may have oncocytic or clear cytoplasmic features. They are uncommon, accounting for 1.5 % of all salivary gland tumors and for 2.2-5.7 % of major and minor salivary gland tumors, respectively. Recently this author, together with several colleagues, have described three unusual myoepithelial tumors, two benign and one malignant that contained abundant intracellular mucin material, which they termed the mucinous variant of myoepithelioma. This represents a unique, previously undescribed subtype that does not fit in the current classification system. A literature review revealed several similar cases reported as "signet ring-cell" adenocarcinomas of salivary gland, which stained for myoepithelial markers, in addition to containing intracellular mucin material, that are more accurately classified as mucinous myoepithelioma. To date, there are 17 reported mucinous myoepitheliomas; four were classified as benign and 13 as malignant. Thirteen arose in minor salivary glands and four in the parotid gland. One patient presented with a lymph node metastasis. With minimal follow-up currently available, this appears to be a benign to low-grade malignancy.
Topics: Biomarkers, Tumor; Cystadenocarcinoma, Mucinous; Humans; Immunohistochemistry; Myoepithelioma; Salivary Gland Neoplasms
PubMed: 23821216
DOI: 10.1007/s12105-013-0464-x -
Advances in Therapy Aug 2019A vast increase in knowledge of numerous aspects of malignant salivary gland tumours has emerged during the last decade and, for several reasons, this has not been the... (Review)
Review
INTRODUCTION
A vast increase in knowledge of numerous aspects of malignant salivary gland tumours has emerged during the last decade and, for several reasons, this has not been the case in benign epithelial salivary gland tumours. We have performed a literature review to investigate whether an accurate histological diagnosis of the 11 different types of benign epithelial salivary gland tumours is correlated to any differences in their clinical behaviour.
METHODS
A search was performed for histological classifications, recurrence rates and risks for malignant transformation, treatment modalities, and prognosis of these tumours. The search was performed primarily through PubMed, Google Scholar, and all versions of WHO classifications since 1972, as well as numerous textbooks on salivary gland tumours/head and neck/pathology/oncology. A large number of archival salivary tumours were also reviewed histologically.
RESULTS
Pleomorphic adenomas carry a considerable risk (5-15%) for malignant transformation but, albeit to a much lesser degree, so do basal cell adenomas and Warthin tumours, while the other eight types virtually never develop into malignancy. Pleomorphic adenoma has a rather high risk for recurrence while recurrence occurs only occasionally in sialadenoma papilliferum, oncocytoma, canalicular adenoma, myoepithelioma and the membranous type of basal cell adenoma. Papillomas, lymphadenoma, sebaceous adenoma, cystadenoma, basal cell adenoma (solid, trabecular and tubular subtypes) very rarely, if ever, recur.
CONCLUSIONS
A correct histopathological diagnosis of these tumours is necessary due to (1) preventing confusion with malignant salivary gland tumours; (2) only one (pleomorphic adenoma) has a considerable risk for malignant transformation, but all four histological types of basal cell adenoma can occasionally develop into malignancy, as does Warthin tumour; (3) sialadenoma papilliferum, oncocytoma, canalicular adenoma, myoepithelioma and Warthin tumour only occasionally recur; while (4) intraductal and inverted papilloma, lymphadenoma, sebaceous adenoma, cystadenoma, basal cell adenoma (apart from the membranous type) virtually never recur. No biomarker was found to be relevant for predicting recurrence or potential malignant development. Guidelines for appropriate treatment strategies are given.
Topics: Adenoma; Adenoma, Pleomorphic; Adult; Aged; Aged, 80 and over; Female; Humans; Male; Middle Aged; Salivary Glands
PubMed: 31209701
DOI: 10.1007/s12325-019-01007-3 -
Human Pathology Apr 2019Many sarcomas contain gene fusions that can be pathogenetic mechanisms and diagnostic markers. In this article we review selected fusion sarcomas and techniques for... (Review)
Review
Many sarcomas contain gene fusions that can be pathogenetic mechanisms and diagnostic markers. In this article we review selected fusion sarcomas and techniques for their detection. CIC-DUX4 fusion sarcoma is a round cell tumor now considered an entity separate from Ewing sarcoma with a more aggressive clinical course, occurrence in older age, and predilection to soft tissues. It is composed of larger cells than Ewing sarcoma and often has prominent necrosis. Nuclear DUX4 expression is a promising immuno histochemical marker. BCOR-CCNB3 fusion sarcoma is cyclin B3-positive, usually occurs in bone or soft tissue of children, and may mimic a poorly differentiated synovial sarcoma. EWSR1-NFATC2 sarcoma may present in bone or soft tissue. It is typically composed of small round cells in a trabecular pattern in a myxoid matrix resembling myoepithelioma. ACTB-GLI1 fusion sarcoma may mimic a skin adnexal carcinoma, showing focal expression of epithelial markers and S100 protein. NTRK-fusion sarcomas include, in addition to infantile fibrosarcoma with ETV6-NTRK3 fusion, LMNA-NTRK1 fusion sarcoma, a low-grade spindle cell sarcoma seen in peripheral soft tissues in children and young adults. Methods to detect gene fusions include next-generation sequencing panels, anchored multiplex polymerase chain reaction systems to detect partner for a known fusion gene, and comprehensive RNA sequencing to detect virtually all gene fusions. In situ hybridization testing using probes for both fusion partners can be used as an alternative confirmation technique, especially in the absence of satisfactory RNA yield. In addition, fusion protein-related and other immunohistochemical markers can have a high specificity for fusion sarcomas.
Topics: Humans; Oncogene Fusion; Oncogene Proteins, Fusion; Sarcoma; Soft Tissue Neoplasms
PubMed: 30633925
DOI: 10.1016/j.humpath.2018.12.006 -
Indian Journal of Otolaryngology and... Dec 2022Tumors of the salivary glands are rare and have various histo-pathological subtypes. The overall incidence is 2.5-3 per 100,000 patients per year. Myoepithelioma of the...
Tumors of the salivary glands are rare and have various histo-pathological subtypes. The overall incidence is 2.5-3 per 100,000 patients per year. Myoepithelioma of the parotid gland is very rare with an incidence of only 1-1.5% among all salivary gland tumors. The diagnostic criteria for benign myoepithelioma were laid down on the basis of the work of Barnes and Sciubba. A 30-year-old lady presented to our university with a mass slowly increasing in size. There were no aggravating and/or relieving factors found. On examination a 3 × 3 cm diffuse swelling was found around the right pre auricular area. Fine Needle Aspiration Cytology (FNAC) was performed. The findings were suggestive of a pleomorphic adenoma. The patient underwent a right sided superficial parotidectomy to remove the tumor. The final histopathology report revealed the tumor to be a myoepithelioma. Myoepithelioma is a benign salivary gland neoplasm formed almost entirely of myoepithelial cells arranged in a sheet, island or cord-like fashion. On CT scans, it presents as a well-circumscribed homogenous lesion with lobulated or smooth margins. Myoepitheliomas may also have nodules that enhance under contrast and areas of linear bands that do not. The recommended treatment for a myoepithelioma is complete surgical excision with wide margins. It has a very low recurrence rate compared to the 15-18% recurrence rate for pleomorphic adenomas. Utilization of immunohistochemical staining is of utmost importance in suspected myoepithelioma cases to ensure proper treatment and follow-up. Myoepitheliomas are rare tumors which must be included in the differential if a patient presents with a tumor of characteristics similar to those described above. Immunohistochemistry and electron microscopy are useful tools for the diagnosis of myoepitheliomas.
PubMed: 36742563
DOI: 10.1007/s12070-021-02763-x -
Head and Neck Pathology Mar 2015Myoepithelial tumors in skin and soft tissue are uncommon but have been increasingly characterized over the past decade. Men and women are equally affected across all... (Review)
Review
Myoepithelial tumors in skin and soft tissue are uncommon but have been increasingly characterized over the past decade. Men and women are equally affected across all age groups and lesions arise most frequently on the extremities and limb girdles. Approximately 20 % of cases occur in pediatric patients, in whom they are frequently malignant. Similar to their salivary gland counterparts, myoepithelial tumors of soft tissue demonstrate heterogeneous morphologic and immunophenotypic features. Tumors are classified as mixed tumor/chondroid syringoma, myoepithelioma, and myoepithelial carcinoma; in soft tissue, tumors having at least moderate cytologic atypia are classified as malignant. Mixed tumor and myoepithelioma show a benign clinical course, with recurrence in up to 20 % (typically secondary to incomplete excision), and do not metastasize. In contrast, myoepithelial carcinoma shows more aggressive behavior with recurrence and metastasis in up to 40-50 % of cases. The majority of myoepithelial neoplasms typically coexpress epithelial antigens (cytokeratin and/or EMA) and S-100 protein; GFAP and p63 are frequently positive and a subset of malignant neoplasms lose INI1 expression. Up to 45 % of myoepitheliomas and myoepithelial carcinomas harbor EWSR1 gene rearrangements, unlike mixed tumor/chondroid syringoma which is characterized by PLAG1 gene rearrangement. While mixed tumor/chondroid syringoma are likely related to primary salivary myoepithelial tumors, soft tissue myoepithelioma and myoepithelial carcinoma appear to be pathologically distinct neoplasms.
Topics: Female; Humans; Immunophenotyping; Male; Myoepithelioma; Soft Tissue Neoplasms
PubMed: 25804378
DOI: 10.1007/s12105-015-0618-0 -
Journal of Medical Case Reports Sep 2014Soft tissue myoepithelial carcinoma and myoepithelioma are rare entities, part of myoepithelial tumors. They were incorporated into the World Health Organization... (Review)
Review
INTRODUCTION
Soft tissue myoepithelial carcinoma and myoepithelioma are rare entities, part of myoepithelial tumors. They were incorporated into the World Health Organization classification of soft tissue tumors in 2002. Here we present an exceptional case of myoepithelial carcinoma and myoepithelioma association. To the best of our knowledge, such an association has never been reported in the literature.
CASE PRESENTATION
We report a case of myoepithelial carcinoma combined with myoepithelioma occurring in the soft tissue of the right forearm of an 84-year-old Arabian man. We describe the clinical, radiological and pathological features dominated by histological polymorphism. We will also describe the proposed histological criteria of malignancy and the major role of immunohistochemistry in positive and differential diagnosis. We finally mention the therapeutic arsenal available.
CONCLUSION
Through this work, we report that myoepithelioma of soft tissue can progress to malignant myoepithelioma.
Topics: Aged, 80 and over; Forearm; Humans; Male; Myoepithelioma; Soft Tissue Neoplasms
PubMed: 25253093
DOI: 10.1186/1752-1947-8-317 -
International Journal of Clinical and... 2014In this article, we described a malignant myoepithelioma of the breast (MMB) in a 69-year-old woman. Breast cancer derived from myoepithelial cells is very rare, usually... (Review)
Review
In this article, we described a malignant myoepithelioma of the breast (MMB) in a 69-year-old woman. Breast cancer derived from myoepithelial cells is very rare, usually benign. The diagnosis of MMB based on histological and immunohistochemical finding. In this case, the author diagnosed the tumor as MMB, because tumor tissues were immunopositive for 34βE12, P63, SMA, S-100, CD10, E-Cad and Ki-67, and immunnegative for CK5/6, desmin, ER, PR and C-erbB-2, because tumor tissue showed invasive growth and local hemorrhage or necrosis, suggesting malignant, and also because there was a transition between the tumor cells and hyperplastic myoepithelium of non-tumorous ducts. The patient's postoperative recovery is smooth and regular following of patient is essential.
Topics: Aged; Biomarkers, Tumor; Biopsy; Breast Neoplasms; Cell Proliferation; Female; Humans; Hyperplasia; Immunohistochemistry; Mastectomy, Radical; Myoepithelioma; Necrosis; Neoplasm Invasiveness
PubMed: 24966981
DOI: No ID Found -
Acta Medica Okayama Dec 2020Soft tissue myoepitheliomas are often misdiagnosed due to their rarity. Herein, we describe a case of soft tissue myoepithelioma of the shoulder. A 72-year-old woman had...
Soft tissue myoepitheliomas are often misdiagnosed due to their rarity. Herein, we describe a case of soft tissue myoepithelioma of the shoulder. A 72-year-old woman had a suspected sarcoma on her shoulder and under-went open biopsy. She was referred to our hospital, where the tumor was widely resected and the diagnosis of myoepithelioma was histologically confirmed. No recurrence has been observed in the 3 years since the sur-gery. Careful and prompt planning is necessary for the effective treatment of myoepithelioma.
Topics: Aged; Diagnosis, Differential; Female; Humans; Magnetic Resonance Imaging; Myoepithelioma; Shoulder; Soft Tissue Neoplasms
PubMed: 33361874
DOI: 10.18926/AMO/61213 -
Neurology India 2019Intracranial myoepithelial tumors are extremely rare with <10 cases reported outside the sellar region. The authors describe a case of a 43-year-old male patient who... (Review)
Review
Intracranial myoepithelial tumors are extremely rare with <10 cases reported outside the sellar region. The authors describe a case of a 43-year-old male patient who presented with headache, numbness in the face, and a dumbbell-shaped lesion in the Meckel's cave clinically and radiologically suggestive of a Schwannoma. The histopathological and immunohistochemical evaluation led to a diagnosis of myoepithelioma. A review of literature reveals that this is only the ninth case of intracranial myoepithelial tumor reported, fifth benign case, and the first to be reported in the Meckel's cave region.
Topics: Adult; Brain Neoplasms; Humans; Male; Myoepithelioma
PubMed: 31744974
DOI: 10.4103/0028-3886.271273 -
Dermatopathology (Basel, Switzerland) Jul 2023Cutaneous syncytial myoepithelioma is a recently characterized variant of cutaneous myoepithelioma with a distinct histopathological and immunohistochemical profile. It...
Cutaneous syncytial myoepithelioma is a recently characterized variant of cutaneous myoepithelioma with a distinct histopathological and immunohistochemical profile. It is more common in men and predominately involves upper and lower extremities. Microscopically, it is a dermal tumor with a characteristic solid syncytial growth pattern displaying positivity with EMA and S100 immunohistochemical stains. Lately, EWSR1-PBX3 fusion has been documented in a vast majority. Although it follows a benign clinical course, its histopathological differential diagnosis includes clinically aggressive neoplasia. This contribution summarizes the derivation, clinical presentation, histopathological and immunohistochemical features, molecular genetics, pertinent differential diagnosis, and behavior of this unique cutaneous appendageal tumor.
PubMed: 37489454
DOI: 10.3390/dermatopathology10030030