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Head and Neck Pathology Mar 2022Myoepithelial carcinoma (MECA) is a rare salivary gland (SG) neoplasm (0.1-0.45% of all SG tumors) that often presents with bland cytomorphology and can be misclassified...
Myoepithelial carcinoma (MECA) is a rare salivary gland (SG) neoplasm (0.1-0.45% of all SG tumors) that often presents with bland cytomorphology and can be misclassified as cellular pleomorphic adenoma (PA) or myoepithelioma. This is particularly challenging in MECA ex-PA cases, especially if tumor shows minimal to no capsular invasion. We report a rare case of a 76-year-old female; history of left superficial parotidectomy with diagnosis (outside hospital) of cellular PA, who re-presented 9 months post surgery with enlarging left parotid mass, neck lymphadenopathy and facial nerve deficits. FNAB of parotid and neck lymph node revealed cellular aspirates with loosely cohesive clusters of myoepithelial cells with occasional chondromyxoid stroma. Prior resection slides were reviewed, and diagnosis of MECA ex-PA was made. Patient underwent left radical parotidectomy, selective neck dissection, with facial nerve sacrifice (due to extensive encasing by tumor). Histology showed a multinodular tumor with pushing borders, zonal arrangement comprising of a hypocellular, necrotic/myxoid center, and a peripheral rim of myoepithelial cells, confirmed by positive S100, and p63. Tumor extensively infiltrated peri parotid soft tissues with multiple foci of lymphovascular and perineural invasion; and metastatic neck lymph nodes. Next generation sequencing revealed a novel TERT promoter mutation (c.-124C > T), not usually described in SG neoplasms. Further, PD-L1 immunohistochemistry showed positive expression, making patient eligible for anti-PDL-1 immunotherapy. This case highlights importance of recognizing the subtle malignant features of MECA in distinguishing it from benign mimics like PA. In addition, presence of TERT mutation opens a new arena for future research to explore potential treatment targets.
Topics: Adenocarcinoma; Adenoma, Pleomorphic; Aged; B7-H1 Antigen; Carcinoma; Diagnostic Errors; Female; Humans; Mutation; Myoepithelioma; Parotid Neoplasms; Salivary Gland Neoplasms; Telomerase
PubMed: 34128136
DOI: 10.1007/s12105-021-01346-0 -
Case Reports in Oncology 2021Myoepithelial carcinoma, also known as malignant myoepithelioma, is considered an extremely rare (0.45-1%) malignant salivary gland neoplasm. Approximately 100 cases...
Myoepithelial carcinoma, also known as malignant myoepithelioma, is considered an extremely rare (0.45-1%) malignant salivary gland neoplasm. Approximately 100 cases have been reported in the English-language literature on myoepithelial carcinoma. The majority of the myoepitheliomas described in the literature have been benign, and the malignant counterpart is considered rare (<1%). Such a tumor may appear de novo or rarely develop from a preexisting pleomorphic adenoma (<20%), and in exceedingly rare cases (<0.5%), it has arisen from a benign myoepithelioma (i.e., plasmacytoid myoepithelioma). To our knowledge, no case of myoepithelial carcinoma of the parotid gland arising in a plasmacytoid myoepithelioma synchronized with melanoma has been reported to date. The treatment of myoepithelial carcinoma has been mainly surgical, including wide excision with free margins, with or without nodal dissection. The roles of chemotherapy and radiotherapy have not yet been established. We report a case of myoepithelial carcinoma of the parotid gland arising in a plasmacytoid myoepithelioma synchronized with melanoma in a 40-year-old woman. In our case, a complete response was achieved with surgery followed by adjuvant chemotherapy based on carboplatin and paclitaxel concurrent with radiotherapy.
PubMed: 33776700
DOI: 10.1159/000510937 -
Pediatric and Developmental Pathology :... 2021Overall, neonatal cancer is uncommon. Because of its rarity and heterogeneity, diagnosis can be challenging. We report a unique case of a myoepithelial carcinoma in a 7...
Overall, neonatal cancer is uncommon. Because of its rarity and heterogeneity, diagnosis can be challenging. We report a unique case of a myoepithelial carcinoma in a 7 week old girl. Molecular diagnostic workup revealed a gene fusion which was previously described in only six cases of myoepithelial tumors so far. All cases occurred in children and adolescents. To our knowledge, this is the first report of a congenital fusion positive myoepithelial tumor in an infant.
Topics: Biomarkers, Tumor; Female; Gene Fusion; Humans; Infant; Kruppel-Like Transcription Factors; Myoepithelioma; RNA-Binding Protein EWS; Soft Tissue Neoplasms
PubMed: 33734915
DOI: 10.1177/1093526621999020 -
Asian Journal of Neurosurgery 2020Myoepithelial tumor (MET) of bone is an unusual tumor of uncertain differentiation and histogenesis. Although its presence in various bones has been reported sparsely,...
Myoepithelial tumor (MET) of bone is an unusual tumor of uncertain differentiation and histogenesis. Although its presence in various bones has been reported sparsely, the presentation in clivus as primary myoepithelial carcinoma (MEC) has never been reported. They resemble their salivary gland counterparts morphologically and immunohistochemically, but harbor distinct molecular phenotype. At present, moderate nuclear atypia is the acceptable criteria to differentiate MEC from myoepithelioma. Because of their rarity, wide histopathological spectrum, and intraosseous location, MET of bone is easily confused with a variety of primary bone and cartilaginous tumors. Application of immunohistochemistry and, if required, molecular testing are required for making a correct diagnosis. In this article, we describe an extremely rare case of a primary MEC arising from the clivus, which owing to unusual location and immunohistochemical profile was diagnostically challenging.
PubMed: 33708682
DOI: 10.4103/ajns.AJNS_144_20 -
Gland Surgery Jan 2021We report 2 cases of rare thyroid malignancy: angiosarcoma and myoepithelial carcinoma (MC). Thyroid angiosarcomas (TAS) is extremely rare and comprises less than 1% of...
We report 2 cases of rare thyroid malignancy: angiosarcoma and myoepithelial carcinoma (MC). Thyroid angiosarcomas (TAS) is extremely rare and comprises less than 1% of primary thyroid cancer worldwide. MC usually presents as a slow-growing painless mass arising in the salivary glands. It has not been reported in the thyroid gland. The first case describes a 59-year-old patient who was admitted to hospital with the discovery of thyroid nodule for 1 month. The tumor thrombus was found in the left internal jugular vein and superior thyroid artery during the operation. Diagnosis of angiosarcoma of the thyroid was based on positive endothelial markers such as thrombomodulin and CD31 after total thyroidectomy. The left internal jugular vein, left recurrent laryngeal nerve and anterior cervical banding muscle were invaded by thyroid tumor. No lymph node metastasis was observed. The patient died after 4 years. The second case describes a 55-year-old woman who presented with the discovery of thyroid nodule for 1 month. Right thyroid lobectomy and right neck lymph node functional dissection were carried out. The results from postoperative pathology revealed that papillary carcinoma in right lobe of thyroid and MC next to thyroid were found. Besides, the metastasis of MC was observed at right II-IV level and right VI level. Five years later, the patient was re-admitted to hospital, primarily due to the discovery of anterior cervical tumor for one year. Then, she underwent left thyroid lobectomy and right tumor resection. Postoperative routine pathology showed recurrent MC in the right thyroid. After surgery and radiotherapy, the patient was followed up for 2 years. Angiosarcoma and myoepithelioma should be kept in mind in diagnosis of thyroid malignant tumor.
PubMed: 33633996
DOI: 10.21037/gs-20-601 -
Head and Neck Pathology Dec 2021The presence of melanin pigment and melanocytic markers expression have been rarely reported in salivary gland tumors. Herein, two cases of carcinoma arising in...
The presence of melanin pigment and melanocytic markers expression have been rarely reported in salivary gland tumors. Herein, two cases of carcinoma arising in pleomorphic adenoma of the parotid gland and showing diffuse expression of myoepithelial and melanocytic markers are described. The clinical-pathological clues useful in the differential diagnosis with melanoma are discussed. In addition, a review of the pertinent literature is also proposed, discussing the pathologic mechanisms potentially involved in this phenomenon.
Topics: Adenoma, Pleomorphic; Aged; Diagnosis, Differential; Fatal Outcome; Humans; Image-Guided Biopsy; Lymph Node Excision; Male; Melanoma; Middle Aged; Myoepithelioma; Neoplasm Staging; Parotid Neoplasms; Positron-Emission Tomography
PubMed: 33591488
DOI: 10.1007/s12105-021-01299-4 -
BMJ Case Reports Feb 2021Cavernous sinus thrombosis is a rare but fatal condition arising due to various infectious and noninfectious causes. Although its incidence is very low in the setting of...
Cavernous sinus thrombosis is a rare but fatal condition arising due to various infectious and noninfectious causes. Although its incidence is very low in the setting of head and neck surgery, including radical neck dissection, a high index of suspicion with prompt treatment is the key to a successful outcome. We report a case of a 50-year-old woman with a chondrosarcoma of left lower alveolobuccal complex who underwent en bloc tumour resection with infratemporal fossa clearance and left modified radical neck dissection. Subsequently, she developed cavernous sinus thrombosis, which was successfully managed with a multidisciplinary approach. This case highlights the importance of high clinical suspicion in the postoperative setting to diagnose this potentially lethal complication.
Topics: Cavernous Sinus Thrombosis; Chondrosarcoma; Female; Head and Neck Neoplasms; Humans; Mandibular Osteotomy; Middle Aged; Myoepithelioma; Neck Dissection; Postoperative Complications; Tomography, X-Ray Computed
PubMed: 33541954
DOI: 10.1136/bcr-2020-240269 -
Surgical Pathology Clinics Mar 2021Myoepithelial carcinoma (MECA) may overlap histologically with other salivary gland neoplasms, especially pleomorphic adenoma. MECA is characterized by cellular, uniform... (Review)
Review
Myoepithelial carcinoma (MECA) may overlap histologically with other salivary gland neoplasms, especially pleomorphic adenoma. MECA is characterized by cellular, uniform growth of myoepithelial cells and multinodular expansile invasive pattern with zonal cellular distribution. It may arise de novo or in association with pleomorphic adenoma (myoepithelial carcinoma ex pleomorphic adenoma). By immunohistochemistry, MECA is positive for cytokeratins and at least one of the myoepithelial markers, including S100. PLAG1 fusion is the most common genetic alteration. Carcinoma ex pleomorphic adenoma and necrosis correlate with worse clinical outcome in MECA, and necrosis can be used to stratify MECA as high grade.
Topics: Adenoma, Pleomorphic; Diagnosis, Differential; Epithelial Cells; Humans; Immunohistochemistry; Myoepithelioma; Necrosis; Neoplasm Grading; Neoplasm Metastasis; Neoplasm Recurrence, Local; Neoplasms, Multiple Primary; Prognosis; Salivary Gland Neoplasms
PubMed: 33526224
DOI: 10.1016/j.path.2020.09.008 -
Virchows Archiv : An International... Nov 2021Myoepithelial neoplasms of soft tissue are rare tumors with clinical, morphological, immunohistochemical, and genetic heterogeneity. The morphological spectrum of these...
Myoepithelial neoplasms of soft tissue are rare tumors with clinical, morphological, immunohistochemical, and genetic heterogeneity. The morphological spectrum of these tumors is broad, and the diagnosis often requires immunostaining to confirm myoepithelial differentiation. Rarely, tumors show a morphology that is typical for myoepithelial neoplasms, while the immunophenotype fails to confirm myoepithelial differentiation. For such lesions, the term "myoepithelioma-like" tumor was introduced. Recently, two cases of myoepithelioma-like tumors of the hands and one case of the foot were described with previously never reported OGT-FOXO gene fusions. Here, we report a 50-year-old woman, with a myoepithelial-like tumor localized in the soft tissue of the forearm and carrying a OGT-FOXO1 fusion gene. Our findings extend the spectrum of mesenchymal tumors involving members of the FOXO family of transcription factors and point to the existence of a family of soft tissue tumors that carry the gene fusion of the OGT-FOXO family.
Topics: Biomarkers, Tumor; Female; Forearm; Forkhead Box Protein O1; Gene Fusion; Humans; Immunohistochemistry; In Situ Hybridization, Fluorescence; Middle Aged; Myoepithelioma; N-Acetylglucosaminyltransferases; Soft Tissue Neoplasms; Treatment Outcome
PubMed: 33506328
DOI: 10.1007/s00428-021-03026-4 -
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