-
Clinical and pathological analysis of 10 cases of salivary gland epithelial-myoepithelial carcinoma.Medicine Oct 2020Epithelial-myoepithelial carcinoma (EMC) is a rare neoplasm of the salivary glands. The aim of this study is to review and evaluate clinicopathological features and...
Epithelial-myoepithelial carcinoma (EMC) is a rare neoplasm of the salivary glands. The aim of this study is to review and evaluate clinicopathological features and treatment of EMC of salivary gland for better sensitivity and specificity of the diagnosis.The clinical and pathological data of the 10 salivary gland EMC cases from 2008 to 2017 were analyzed.Six cases of EMC were diagnosed to be originated from parotid gland and 4 cases were from the minor salivary gland including palate, tongue, and oropharynx. Seven cases were performed radical surgery and 3 cases had radiotherapy postoperation, 2 cases had a local recurrence. The follow-up period was 4 to 104 months and the survival rate was 100%. Histopathology showed the tumors had a dominant prototypical biphasic tubular structure consisting of inner, cuboidal ductal cells and an outer layer of clear, myoepithelial cells, which grew infiltratively. The immunohistochemistry (IHC) showed the marker proteins CK, S-100, CD117, and Calponin were strongly positive in most EMC.EMC is a rare and low-grade malignant tumor with good overall survival but relatively high tendency for local recurrence. Surgery is the priority choice for EMC therapy. Complete surgical excision and negative margins are necessary for good prognosis. Imaging techniques should be used to assess the neck dissection and it is unclear whether adjuvant radiotherapy is beneficial. To ensure the sensitivity and specificity of the EMC diagnosis, we should perform both pathological and IHC analysis.
Topics: Adult; Aged; Aged, 80 and over; Carcinoma; Female; Humans; Male; Middle Aged; Myoepithelioma; Retrospective Studies; Salivary Gland Neoplasms; Salivary Glands
PubMed: 33031333
DOI: 10.1097/MD.0000000000022671 -
Medicine Oct 2020Epithelial-myoepithelial carcinoma (EMC) is a rare, low-grade malignancy that occurs primarily in the parotid gland and is most common in women aged 60 to 70 years....
INTRODUCTION
Epithelial-myoepithelial carcinoma (EMC) is a rare, low-grade malignancy that occurs primarily in the parotid gland and is most common in women aged 60 to 70 years. Cases of parotid EMC have been reported previously. Furthermore, some studies have suggested an increased risk of salivary gland tumors with secondary primary malignancies. There have been few reports of parotid EMC with other primary tumors.
PATIENT CONCERNS
A 62-year-old Chinese man visited the hospital with a complaint of a mass on his left cheek that had persisted for 20 years. Routine pulmonary computed tomography showed a local ground glass shadow in the lower lobe of the right lung.
DIAGNOSIS
The pathological diagnosis of lung was right lower lobe lung adenocarcinoma (pT1N0). Immunohistochemistry analysis showed that cytokeratin (CK)-7, NapsinA, and thyroid transcription factor-1 tested positive, while CK5/6, P40, and ALKD5F3 tested negative. The pathological diagnosis of left parotid gland: EMC. On immunohistochemistry staining, the outer cells expressed myoepithelial markers, such as CK5/6, P63, smooth muscle actin, while the inner cells expressed glandular epithelial markers, such as low-molecular-weight CK7 and CK8.
INTERVENTIONS
The patient underwent resection of the lung and parotid tumors, and received preventive radiotherapy in the parotid gland area.
OUTCOMES
The patient is in good condition. No symptom recurrence, distant metastatic spread or significant toxicity occurred during or after the treatment. The patient remains under regular surveillance.
CONCLUSION
We report a rare case of parotid EMC with a second primary lung adenocarcinoma. This case is the third case of primary lung cancer associated with parotid EMC reported to date and the first to be reported in nearly 30 years.
Topics: Adenocarcinoma of Lung; Humans; Immunohistochemistry; Lung Neoplasms; Male; Middle Aged; Myoepithelioma; Neoplasms, Second Primary; Parotid Neoplasms
PubMed: 33019442
DOI: 10.1097/MD.0000000000022483 -
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 -
Diagnostic Pathology Aug 2020Pulmonary epithelial-myoepithelial carcinoma is a rare subtype of lung cancer. Because of its rarity, the molecular information on this carcinoma is insufficient.
BACKGROUND
Pulmonary epithelial-myoepithelial carcinoma is a rare subtype of lung cancer. Because of its rarity, the molecular information on this carcinoma is insufficient.
CASE PRESENTATION
We report a case of pulmonary epithelial-myoepithelial carcinoma without AKT1, HRAS or PIK3CA mutations in a 76-year-old woman. Computed tomography revealed a tumor located in the left lower lung. Thoracoscopic left lower lobectomy was performed. Histopathologically, the tumor consisted of duct-like structures and polygonal and spindle cell features. The duct-like structures were composed of two distinct cell layers. The inner layer consisted of cuboidal cells that were positive for pan-cytokeratin and negative for p63, whereas the outer layer consisted of polygonal and spindle cells that were positive for p63 and weakly positive for pan-cytokeratin. We evaluated mutations in AKT1, BRAF, CTNNB1, HRAS, KRAS and PIK3CA but did not detect any mutations.
CONCLUSION
Pulmonary epithelial-myoepithelial carcinoma is a rare subtype of lung cancer, with only 56 previous cases reported in the English literature. The genetic alterations in pulmonary epithelial-myoepithelial carcinoma are still unknown. We examined the 6 genes mutation analysis, however no mutation was detected.
Topics: Aged; Biomarkers, Tumor; Carcinoma; Class I Phosphatidylinositol 3-Kinases; Female; Humans; Lung Neoplasms; Mutation; Myoepithelioma; Proto-Oncogene Proteins c-akt; Proto-Oncogene Proteins p21(ras)
PubMed: 32859224
DOI: 10.1186/s13000-020-01020-z -
Turk Patoloji Dergisi 2021Epithelial-myoepithelial carcinoma (EMC) is a rare, low-grade, malignant salivary neoplasm. Establishing an accurate cytological diagnosis is often challenging owing to...
Epithelial-myoepithelial carcinoma (EMC) is a rare, low-grade, malignant salivary neoplasm. Establishing an accurate cytological diagnosis is often challenging owing to its rarity, bland cytologic appearance and variable representation of cell populations in the smears. The diagnostic struggle is more so when the aspiration is from a metastatic site with an unknown primary, as in such cases the list of differential diagnoses expands further. A 58-year-old female presented with a low-back pain from last one month. On examination, she also had a level III, right cervical swelling for the last 20 years. Radiology revealed a lytic lesion in the left acetabulum. She had undergone surgery 35 years ago for a right-sided upper neck swelling, the medical records of which were not available. Fine needle aspiration (FNA) from the cervical swelling was performed. The smears were cellular and showed predominantly dispersed, round to polygonal tumor cells with mild pleomorphism, eccentric nuclei, coarse chromatin, occasional nucleoli and moderate cytoplasm with some showing vacuolations. The cell-block section revealed tumor cells arranged in the form of tubules lined by dual layer of tumor cells without any chondromyxoid stroma. On immunocytochemistry, the luminal cells showed positivity for CK7 (epithelial marker) and the abluminal cells showed positivity for p63 (myoepithelial marker). Based on these features, a final diagnosis of metastatic epithelial-myoepithelial carcinoma was rendered. The present report highlights the characteristic cytomorphological and immunocytochemical features of EMC and reiterates the diagnostic accuracy of FNAC for diagnosis of such challenging cases.
Topics: Acetabulum; Biomarkers, Tumor; Biopsy, Fine-Needle; Bone Neoplasms; Diagnosis, Differential; Female; Head and Neck Neoplasms; Humans; Middle Aged; Myoepithelioma; Neoplasms, Glandular and Epithelial; Neoplasms, Unknown Primary; Predictive Value of Tests
PubMed: 32779157
DOI: 10.5146/tjpath.2020.01501 -
Neoplasia (New York, N.Y.) Sep 2020Epithelial-myoepithelial carcinoma (EMC) is a rare subtype of salivary gland neoplasms. Since the initial description of the cancer, just over 300 cases have been...
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.
Topics: Adult; Antineoplastic Agents; Biomarkers, Tumor; Carcinoma; DNA Mutational Analysis; Everolimus; Female; Gene Expression Profiling; High-Throughput Screening Assays; Humans; Lung Neoplasms; Molecular Targeted Therapy; Mutation; Myoepithelioma; Prognosis; Salivary Gland Neoplasms; Tumor Cells, Cultured
PubMed: 32645560
DOI: 10.1016/j.neo.2020.06.007 -
Case Reports in Pathology 2020Cutaneous myoepithelioma (CM) is a rare tumor among the primary skin neoplasms. We present the case of a patient with a diagnosis of CM in the right hypothenar region....
Cutaneous myoepithelioma (CM) is a rare tumor among the primary skin neoplasms. We present the case of a patient with a diagnosis of CM in the right hypothenar region. Histological study showed a proliferation of myoepithelial cells with a solid, reticular growth pattern in a chondromyxoid stroma. The tumor cells were positive for CK AE, S-100, EMA, and p63.
PubMed: 32607268
DOI: 10.1155/2020/3747013 -
Diagnostic Pathology Jun 2020Primary myoepithelial carcinoma of the lung is a rare subtype in lung cancer. Comprehensive molecular profiling of myoepithelial carcinoma of the lung is absent, neither...
BACKGROUND
Primary myoepithelial carcinoma of the lung is a rare subtype in lung cancer. Comprehensive molecular profiling of myoepithelial carcinoma of the lung is absent, neither was clinical evidence of targeted therapy available for this disease. Therefore, the optimal treatment regimen of this tumor needs to be established.
CASE PRESENTATION
Here we present a case of a 68-year-old patient with stage IVB primary myoepithelial carcinoma of the lung who harbored EGFR exon 19 deletion and KRAS mutation and underwent icotinib targeted therapy, achieving partial response (PR) with progression free survival (PFS) of 3 months.
CONCLUSION
To our knowledge, this study describes the first documented case of primary myoepithelial carcinoma lung cancer patient harboring EGFR exon 19 deletion and KRAS mutation, and showed clinical efficacy of epidermal growth factor receptor tyrosine kinase inhibitors (EGFR-TKI) treatment in this patient.
Topics: Aged; Antineoplastic Agents; Crown Ethers; Disease-Free Survival; ErbB Receptors; Exons; Gene Deletion; Humans; Lung Neoplasms; Male; Molecular Targeted Therapy; Myoepithelioma; Quinazolines; Smoking
PubMed: 32505185
DOI: 10.1186/s13000-020-00986-0 -
Head and Neck Pathology Mar 2021Ectomesenchymal chondromyxoid tumor is a rare neoplasm of uncertain histogenesis that typically occurs in the anterior dorsal tongue. Recent reports in the literature...
Ectomesenchymal chondromyxoid tumor is a rare neoplasm of uncertain histogenesis that typically occurs in the anterior dorsal tongue. Recent reports in the literature have described rare examples of gingival, palatal and tonsillar lesions. Histologically, ectomesenchymal chondromyxoid tumors are typically well-circumscribed, lacking overtly aggressive features. Herein we report a tumor arising in the right mandible that is morphologically and molecularly consistent with ectomesenchymal chondromyxoid tumor. This case furthers awareness of the extra-glossal distribution of this neoplasm; moreover, it suggests that a subset of these tumors have the potential for locally aggressive behaviour.
Topics: Adult; Female; Humans; Mandibular Neoplasms; Mesenchymoma; Myoepithelioma
PubMed: 32372271
DOI: 10.1007/s12105-020-01169-5 -
Imaging Science in Dentistry Mar 2020Computed tomography (CT) and magnetic resonance imaging (MRI) can be useful for the evaluation of palatal lesions, and strain elastography (performed together with...
Computed tomography (CT) and magnetic resonance imaging (MRI) can be useful for the evaluation of palatal lesions, and strain elastography (performed together with intraoral ultrasonography) is a relatively new sonographic imaging modality. This report describes 2 clinical cases in which strain elastography was used to assess palatal tumors in conjunction with intraoral ultrasonography, CT, and MRI. In the first case, diagnosed as a myoepithelioma, the strain was determined to be 0.000% (strain of normal tissue, 0.556%). In the second case, diagnosed as an adenoid cystic carcinoma, the determined strain was 0.000% (strain of normal tissue, 1.077%). Therefore, we conclude that intraoral strain elastography can be useful for evaluating palatal lesions.
PubMed: 32206623
DOI: 10.5624/isd.2020.50.1.73