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Head and Neck Pathology Jun 2021Myoepithelial neoplasms are rare tumors of the salivary glands with predominant myoepithelial differentiation and a broad histologic spectrum. Their histological...
Myoepithelial neoplasms are rare tumors of the salivary glands with predominant myoepithelial differentiation and a broad histologic spectrum. Their histological features, immunohistochemical profile and biological behavior are not well characterized and pose a diagnostic challenge. A total of 15 myoepithelial tumors, diagnosed during 2012 and 2019 were subcategorized and correlated with MIB-1 labeling index (LI) and various histological parameters. Immunohistochemical stains for MIB-1 and other antibodies were performed. Statistical analysis was done by chi-square test, Fisher's exact test and Kaplan Meier curve. Nine patients were male and six were female with the median age of 44 years (range 21-83 years). Of the 15 patients, 6 cases were classified as myoepithelioma (ME) and 9 cases as myoepithelial carcinoma (MECA). Parotid gland was the most common site (46.7%) followed by the palate. MEs showed well circumscribed tumor borders whereas MECAs exhibited focal capsular to extensive invasion into adjacent tissues. Epithelioid cell morphology was most common followed by mixed cell morphology. MIB-1 LI was significantly associated with invasive tumor borders, necrosis and high mitosis. Increased frequency of recurrence was noted with high MIB-1 LI, though it was not statistically significant. MIB-1 LI was high in nearly all MECAs with focal capsular to extensive invasion while low in MEs. Myoepithelial tumor with multinodular growth pattern and focal capsular invasion may have an indolent behavior if mitotic activity and MIB-1 LI is low. Early diagnosis and treatment of MECAs significantly improves the patient's survival and prognosis.
Topics: Adult; Aged; Aged, 80 and over; Antibodies, Antinuclear; Antibodies, Monoclonal; Biomarkers, Tumor; Carcinoma; Female; Humans; Immunohistochemistry; Ki-67 Antigen; Male; Middle Aged; Mitotic Index; Myoepithelioma; Neoplasm Recurrence, Local; Prognosis; Retrospective Studies; Salivary Gland Neoplasms
PubMed: 32959208
DOI: 10.1007/s12105-020-01225-0 -
Head and Neck Pathology Mar 2020Primary myoepithelial neoplasms of soft tissue have been shown to be related to their salivary gland counterparts, with which they often share morphologic,... (Review)
Review
Primary myoepithelial neoplasms of soft tissue have been shown to be related to their salivary gland counterparts, with which they often share morphologic, immunophenotypic, and molecular genetic features, such as the presence of PLAG1 rearrangement in both soft tissue mixed tumor and salivary pleomorphic adenoma. However, important distinctions remain between soft tissue and salivary myoepithelial neoplasms, namely differing criteria for malignancy. This review provides an overview of the current understanding of the clinicopathologic and molecular features of soft tissue myoepithelial neoplasms, including discussion of the similarities and differences between soft tissue and salivary counterparts and relevant diagnostic issues specific to head and neck pathology practice.
Topics: Diagnosis, Differential; Head and Neck Neoplasms; Humans; Myoepithelioma; Salivary Gland Neoplasms; Soft Tissue Neoplasms
PubMed: 31950472
DOI: 10.1007/s12105-019-01109-y -
Journal of Medical Cases Jun 2022We report the case of a 53-year-old man with psoriatic arthritis, suffering from a malignant and recidivant myoepithelioma in his right axilla and arm, and undergoing...
We report the case of a 53-year-old man with psoriatic arthritis, suffering from a malignant and recidivant myoepithelioma in his right axilla and arm, and undergoing two surgeries, with the last one being performed a month prior to actual admission. After the last surgery, he was admitted to hospital with fever without a source. After physical examination, laboratory tests, blood cultures and transthoracic and transesophageal echocardiography, he was diagnosed with infectious endocarditis (IE) on a bicuspid aortic valve (BAV) caused by (PA). Antibiogram-guided antibiotic therapy with meropenem and tobramicin was initiated. However, in the presence of repetitive spleen infarctions and a large vegetation, 12 days after admission, a bioprosthesis aortic valve implantation was performed. The postsurgical evolution was favorable and prolonged antibiotic course with meropenem and tobramicin was completed. The pathological anatomy and the native valve cultured confirmed an IE caused by PA. Gram-negative non-HACEK IE cases are infrequent, accounting for 1.8% of the total IE cases. PA is the second most frequent bacillus in this group, causing endocarditis more prevalently when associated with healthcare procedures rather than injectable drug use. No prior case study has identified IE caused by PA related to a BAV in the last years.
PubMed: 35837082
DOI: 10.14740/jmc3943 -
The American Journal of Surgical... Jan 2018We hypothesized that there is a relationship between the preexisting pleomorphic adenoma [PA]), histologic grade of epithelial-myoepithelial carcinomas (EMCAs), and...
Epithelial-Myoepithelial Carcinoma: Frequent Morphologic and Molecular Evidence of Preexisting Pleomorphic Adenoma, Common HRAS Mutations in PLAG1-intact and HMGA2-intact Cases, and Occasional TP53, FBXW7, and SMARCB1 Alterations in High-grade Cases.
We hypothesized that there is a relationship between the preexisting pleomorphic adenoma [PA]), histologic grade of epithelial-myoepithelial carcinomas (EMCAs), and genetic alterations. EMCAs (n=39) were analyzed for morphologic and molecular evidence of preexisting PA (PLAG1, HMGA2 status by fluorescence in situ hybridization, FISH, and FGFR1-PLAG1 fusion by next-generation sequencing, NGS). Twenty-three EMCAs were further analyzed by NGS for mutations and copy number variation in 50 cancer-related genes. On the basis of combined morphologic and molecular evidence of PA, the following subsets of EMCA emerged: (a) EMCAs with morphologic evidence of preexisting PA, but intact PLAG1 and HMGA2 (12/39, 31%), (b) Carcinomas with PLAG1 alterations (9/39, 23%), or (c) HMGA2 alterations (10/39, 26%), and (d) de novo carcinomas, without morphologic or molecular evidence of PA (8/39, 21%). Twelve high-grade EMCAs (12/39, 31%) occurred across all subsets. The median disease-free survival was 80 months (95% confidence interval, 77-84 mo). Disease-free survival and other clinicopathologic parameters did not differ by the above defined subsets. HRAS mutations were more common in EMCAs with intact PLAG1 and HMGA2 (7/9 vs. 1/14, P<0.001). Other genetic abnormalities (TP53 [n=2], FBXW7 [n=1], SMARCB1 deletion [n=1]) were seen only in high-grade EMCAs with intact PLAG1 and HMGA2. We conclude that most EMCAs arose ex PA (31/39, 80%) and the genetic profile of EMCA varies with the absence or presence of preexisting PA and its cytogenetic signature. Progression to higher grade EMCA with intact PLAG1 and HMGA2 correlates with the presence of TP53, FBXW7 mutations, or SMARCB1 deletion.
Topics: Adenoma, Pleomorphic; Adult; Aged; Aged, 80 and over; Biomarkers, Tumor; DNA-Binding Proteins; Disease-Free Survival; F-Box-WD Repeat-Containing Protein 7; Female; Follow-Up Studies; HMGA2 Protein; Humans; In Situ Hybridization, Fluorescence; Male; Middle Aged; Mutation; Myoepithelioma; Neoplasm Grading; Neoplasms, Glandular and Epithelial; Proto-Oncogene Proteins p21(ras); SMARCB1 Protein; Salivary Gland Neoplasms; Sequence Analysis, DNA; Tumor Suppressor Protein p53
PubMed: 29135520
DOI: 10.1097/PAS.0000000000000933 -
The American Journal of Case Reports Apr 2022BACKGROUND Breast adenomyoepithelioma is a rare benign breast tumor characterized by a biphasic proliferation of epithelial and myoepithelial cells with variable...
BACKGROUND Breast adenomyoepithelioma is a rare benign breast tumor characterized by a biphasic proliferation of epithelial and myoepithelial cells with variable clinical and diagnostic features. Establishing the diagnosis, determining optimal therapy, and predicting outcome are problematic because of the rarity of this entity. There have been only 2 large series of adenomyoepitheliomas of the breast, reported by Tavassoli and Rosen, which included 27 and 18 patients, respectively. In this report, we present 3 cases of breast adenomyoepithelioma. CASE REPORT Herein, we report 3 cases of breast adenomyoepithelioma. The first case is of a 64-year-old woman who was found to have right breast microcalcification on a screening mammogram. The second case is of a 74-year-old woman who had a right breast mass. These 2 patients were managed by wide local excision. Postoperative microscopic examination revealed adenomyoepithelioma. The third case is of a 49-year-old woman with bilateral saline breast implants who presented with a left breast mass. A core needle biopsy was done and revealed adenomyoepithelioma associated with usual ductal hyperplasia and ductal carcinoma in situ. CONCLUSIONS Breast adenomyoepithelioma is a rare condition that can pose diagnostic challenges due to variable imaging presentations, necessitating percutaneous core biopsy for initial diagnosis. Correct diagnosis is usually possible only on excisional biopsy and confirmed by demonstrating the biphasic nature of the tumor by IHC. Clinical suspicion coupled with utilizing both radiological and histopathological facilities can aid in the accurate diagnosis and management. For the most part, they are considered to be benign, but they can locally recur.
Topics: Adenomyoepithelioma; Aged; Breast; Breast Neoplasms; Carcinoma, Intraductal, Noninfiltrating; Female; Humans; Middle Aged; Myoepithelioma; Neoplasm Recurrence, Local
PubMed: 35400730
DOI: 10.12659/AJCR.936070 -
BMJ Case Reports Jan 2021Myoepithelial tumours are a rare form of salivary gland neoplasm, and their occurrence in the central nervous system is exceedingly rare. The authors report the case of... (Review)
Review
Myoepithelial tumours are a rare form of salivary gland neoplasm, and their occurrence in the central nervous system is exceedingly rare. The authors report the case of an 18-year-old Filipino man presenting with headache and weakness, and on imaging showing an extensive parasagittal tumour at the left posterior parietal area with extracalvarial extension. There was no systemic disease. The patient underwent surgery to excise the tumour, with histopathology showing findings consistent with myoepithelioma. There was no further treatment, given the benign histology of the lesion, but there was recurrence after 8 months. Repeat surgery was done for the patient and he is for adjuvant radiotherapy. This appears to be the 10th reported case of a central nervous myoepithelioma, and the first case in the Philippines of a primary parasagittal myoepithelioma in a paediatric patient. Further information is needed to provide diagnostic and therapeutic recommendations.
Topics: Adolescent; Brain Neoplasms; Cerebral Angiography; Humans; Male; Myoepithelioma; Neoplasm Recurrence, Local; Parietal Lobe; Philippines; Radiotherapy, Adjuvant
PubMed: 33504518
DOI: 10.1136/bcr-2020-236479 -
Indian Journal of Otolaryngology and... Nov 2019Myoepithelioma is a rare tumour of the salivary glands. Only 3 cases of myoepithelioma in the nasal cavity have been reported. A 29 year old male presented to us with...
Myoepithelioma is a rare tumour of the salivary glands. Only 3 cases of myoepithelioma in the nasal cavity have been reported. A 29 year old male presented to us with epistaxis and nasal obstruction. There was a fleshy mass occupying the right middle meatus. Biopsy showed evidence of myoepithelioma and an endoscopic excision of the tumour was done. Histopathological examination of the resected tumour was consistent with myoepithelioma. Due to its rarity, the nature of the tumour is not known and regular follow-ups are needed for early detection of recurrence and malignancy.
PubMed: 31763237
DOI: 10.1007/s12070-017-1093-6 -
Hand Surgery & Rehabilitation Dec 2023Cutaneous myoepithelioma is a rare neoplasm of the skin that has become more widely recognized in recent years despite significant diagnostic pitfalls. It is a benign...
Cutaneous myoepithelioma is a rare neoplasm of the skin that has become more widely recognized in recent years despite significant diagnostic pitfalls. It is a benign neoplasm with a high recurrence rate if not excised radically, and must be distinguished from its malignant counterpart. Few cases have been described so far and, to our knowledge, no cases in the finger of a child exist in the literature. We report the case of a 15 year-old boy affected by a rare form of locally aggressive spindle-cell myoepithelioma, and suggest a new multidisciplinary approach combining surgical excision and custom brachytherapy.
Topics: Male; Child; Humans; Adolescent; Myoepithelioma; Skin Neoplasms; Fingers; Upper Extremity
PubMed: 37714516
DOI: 10.1016/j.hansur.2023.09.001 -
Head and Neck Pathology Sep 2022Pleomorphic adenoma (PA) is the most common biphasic type of salivary gland tumour to arise in adults. It is a biphasic tumour composed of both luminal (ductal) cells...
Pleomorphic adenoma (PA) is the most common biphasic type of salivary gland tumour to arise in adults. It is a biphasic tumour composed of both luminal (ductal) cells and abluminal (basal and myoepithelial) cells. Other biphasic salivary gland type tumours, both benign and malignant, can mimic PA, especially on small biopsies. Previous studies have shown that glial fibrillary acidic protein (GFAP) is preferentially expressed in PA and can be useful in the distinction from other salivary gland tumours. However, most of these studies were performed on a small subset of tumour types at a time when the classification of salivary gland type tumours was less refined. The purpose of this study was to assess the expression of glial fibrillary acidic protein (GFAP) in a broad group of both benign and malignant salivary gland tumours. The expression of GFAP was assessed in 99 tumours including 54 PAs, 5 basal cell adenomas, 1 myoepitheliomas, 5 adenoid cystic carcinomas, 6 epithelial-myoepithelial carcinomas (EMCA), 6 mucoepidermoid carcinomas, 7 salivary duct carcinomas, 1 adenocarcinomas NOS, 2 myoepithelial carcinomas, 4 basal cell adenocarcinomas, 5 acinic cell carcinomas and 3 polymorphous adenocarcinomas. Of the malignant cases, 8 were classified as carcinomas ex PA. GFAP was also assessed in 19 concurrent biopsy specimens. GFAP was expressed in the resections of 51 PAs examined (94%). Expression was predominantly strong and diffusely seen in myoepithelial cells. Strong and diffuse GFAP expression was also seen in two EMCAs (33%) and one myoepithelial carcinoma (50%). On biopsy specimens, 100% of PAs and basal cell adenomas expressed GFAP. GFAP was also seen in 1 out of 3 carcinomas ex PAs on biopsies. Almost all PAs show strong and diffuse expression of GFAP. In contrast, most malignant neoplasms that can mimic PA on biopsies show only rare, focal expression. Other benign tumours composed of abluminal/myoepithelial cells also show focal expression of GFAP, highlighting the spectrum these tumours share with PA. Overall, the presence of strong and diffuse GFAP expression can favour a benign neoplasm, specifically a PA, on limited biopsy specimens.
Topics: Adenoma; Adenoma, Pleomorphic; Adult; Biomarkers, Tumor; Carcinoma; Carcinoma, Acinar Cell; Glial Fibrillary Acidic Protein; Humans; Immunohistochemistry; Myoepithelioma; Salivary Gland Neoplasms
PubMed: 35064902
DOI: 10.1007/s12105-021-01409-2 -
The American Journal of Surgical... Oct 2019Cutaneous syncytial myoepithelioma (CSM) is a rare but distinctive benign variant in the family of myoepithelial neoplasms of skin and soft tissue. CSM has unique...
Cutaneous syncytial myoepithelioma (CSM) is a rare but distinctive benign variant in the family of myoepithelial neoplasms of skin and soft tissue. CSM has unique morphologic and immunohistochemical features, characterized by intradermal syncytial growth of spindled, ovoid, and histiocytoid cells and consistent staining for S-100 protein and EMA, and differs from other myoepithelial tumors by showing only infrequent keratin staining. Rearrangement of the EWSR1 gene is now known to occur in up to half of all skin and soft tissue myoepithelial tumors, with a wide family of documented fusion partners. In 2013, we reported frequent (80%) EWSR1 rearrangements in CSM, but were unable to identify the fusion partner using available studies at that time. After recent identification of an index case of CSM harboring an EWSR1-PBX3 fusion, we used a combination of targeted RNA sequencing and fluorescence in situ hybridization (FISH) studies to investigate the genetic features of a cohort of CSM. An EWSR1-PBX3 fusion was identified in all 13 cases successfully tested. RNA sequencing was successful in 8/13 cases, all of which were found to have identical breakpoints fusing exon 8 of EWSR1 to exon 5 of PBX3. FISH confirmed both EWSR1 and PBX3 rearrangements in 9/9 cases tested, which included 4 confirmed to have EWSR1-PBX3 fusion by RNA-Seq, 3 cases that failed RNA-Seq, and 2 cases examined by FISH alone. Two cases failed RNA sequencing but had no additional tissue remaining for FISH studies. Our findings demonstrate that EWSR1-PBX3 fusions occur in most (and possibly all) cases of CSM.
Topics: Adolescent; Adult; Aged; Biomarkers, Tumor; Female; Gene Fusion; Gene Rearrangement; Genetic Predisposition to Disease; Homeodomain Proteins; Humans; In Situ Hybridization, Fluorescence; Male; Middle Aged; Myoepithelioma; Proto-Oncogene Proteins; RNA-Binding Protein EWS; Sequence Analysis, RNA; Skin Neoplasms; Young Adult
PubMed: 31135487
DOI: 10.1097/PAS.0000000000001286