Search

Myoepithelioma is an exceptionally rare tumor, primarily arising in glandular tissues but occasionally found in soft tissues, including the hand. Its occurrence in the hand is particularly uncommon, presenting unique clinical challenges due to the limited number of documented cases and the unusual location. We conducted a literature review in June 2024, with the aim to evaluate the current understanding of hand myoepithelioma, recent diagnostic advances, treatment options, and the diverse presentations of this neoplasm. Articles confirmed that patients present with a painless, slow-growing mass in the hand, often misdiagnosed as more common soft tissue tumors like lipomas or fibromas. Imaging, particularly MRI and ultrasound, aids in assessing the tumor, but definitive diagnosis relies on histopathology, including immunophenotyping. Managing spindle cell myoepithelioma in the hand requires a multidisciplinary approach, with surgical excision being the primary treatment. Achieving clear margins is critical yet challenging due to the hand's complex anatomy. In some cases, adjuvant therapies such as radiation or chemotherapy may be necessary. The prognosis depends on factors like tumor size, location, and the success of surgical removal, with complete excision typically leading to a favorable outcome. [ABSTRACT FROM AUTHOR]

Ethmoid myoepithelial carcinoma is a rare tumor, with only 14 cases reported to date. This report discusses the largest tumor of this type ever recorded in the ethmoid region. The tumor caused extensive damage to facial structures, complicating treatment. The patient’s age and comorbidities increased the risk of intraoperative bleeding, presenting challenges to the complete removal of the tumor and the reconstruction of the damaged structures. To reduce the risk of intraoperative hemorrhage, shorten the surgery time, and manage potential heartrelated complications, arterial embolization was performed using gelatin sponges and coils. Definitive surgery was then carried out using a skin flap and mucosal flap to successfully reconstruct the defect. Postoperative radiotherapy was deemed unnecessary. The patient recovered well, with a satisfactory aesthetic outcome. No recurrence was observed during a 3-year follow-up period. [ABSTRACT FROM AUTHOR]

Myoepithelial carcinoma (MECA) is a rare type of carcinoma for which the clinicopathological features and prognostic factors have not yet been fully clarified. A retrospective study of 42 patients diagnosed with salivary gland MECA was performed, focusing on the clinicopathological features and prognostic factors. Of the 42 patients, 20 died of cancer, 20 lived without tumour, one lived with distant metastasis, and one was lost to follow-up. Overall, 69.0% had tumour recurrence, 16.7% had cervical nodal metastasis, and 21.4% had distant metastasis. The 5-year overall survival rate was 70.2%. Kaplan–Meier analysis revealed that patients with pathological positive lymph nodes (pN+), multiple recurrences of tumour, and higher histological grade had worse overall survival. Multivariate Cox analysis indicated pN+ and higher histological grade to be independent predictors of decreased survival. The 5-year overall survival rate in the pN0 group was 87.5%, while that in the pN+ group was 28.6%. In conclusion, myoepithelial carcinoma can be defined as a tumour with a high incidence of recurrence and poor prognosis, especially in pN+ patients. Pathological positive lymph nodes and histological grade may serve as predictors of survival. [ABSTRACT FROM AUTHOR]

Myoepitheliomas are uncommon neoplasms of major and minor salivary glands. These tumours are encapsulated and slow growing in nature and are benign in majority of the cases. The mean age of presentation is 40 years and this entity is rarely reported in younger age groups. Here, we seek to report a rare case of a 10-year-old patient who presented with a swelling below the right ear. Clinical and radiological evaluation revealed a mass in the parotid gland. This patient was treated with a superficial parotidectomy and subsequent histopathological examination revealed it to be a myoepithelioma. [ABSTRACT FROM AUTHOR]

Myoepithelioma is a benign salivary gland tumor. Central myoepitheliomas are very rare. The aim of this report was to describe a case of maxillary myoepithelioma. A 14-year-old female patient presented with an multilocular lesion in the anterior maxilla, with nearly 8 months of duration. The lesion was asymptomatic, and the patient's dental history was unremarkable. The diagnostic hypothesis was an odontogenic tumor. Biopsy specimen consisted of nests of plasmacytoid cells in a myxoid stroma without duct formation. No cellular atypia or bone and cartilage formation were noted. The neoplastic cells were positive for Pan-cytokeratin, S100, CK7, and CK8. The final diagnosis was myoepithelioma. The patient was treated by surgical excision followed by bone curettage, and no signs of recurrence were found after 8 years of treatment. [ABSTRACT FROM AUTHOR]

Myoepithelioma is an exceptionally rare tumor, primarily arising in glandular tissues but occasionally found in soft tissues, including the hand. Its occurrence in the hand is particularly uncommon, presenting unique clinical challenges due to the limited number of documented cases and the unusual location. We conducted a literature review in June 2024, with the aim to evaluate the current understanding of hand myoepithelioma, recent diagnostic advances, treatment options, and the diverse presentations of this neoplasm. Articles confirmed that patients present with a painless, slow-growing mass in the hand, often misdiagnosed as more common soft tissue tumors like lipomas or fibromas. Imaging, particularly MRI and ultrasound, aids in assessing the tumor, but definitive diagnosis relies on histopathology, including immunophenotyping. Managing spindle cell myoepithelioma in the hand requires a multidisciplinary approach, with surgical excision being the primary treatment. Achieving clear margins is critical yet challenging due to the hand’s complex anatomy. In some cases, adjuvant therapies such as radiation or chemotherapy may be necessary. The prognosis depends on factors like tumor size, location, and the success of surgical removal, with complete excision typically leading to a favorable outcome.

Introduction: Myoepithelioma is a rare neoplasm of the salivary glands and accounts for less than 1% of salivary gland neoplasms. Only 7 cases of myoepithelioma in the nasal cavity have been reported till date in literature. Case Report: A 61 year old male presented to us with epistaxis. There was a pinkish fleshy mass occupying the right nasal cavity. Biopsy showed evidence of an epithelial tumor of intermediate aggressiveness. We did an endoscopic complete excision of nasal cavity mass. Histopathological examination of the resected tumor was consistent with myoepithelioma. Due to its rarity, the nature of the tumor is not known and regular follow-ups are needed for early detection of recurrence and malignancy. There has been no evidence of tumor recurrence in the 18 months following surgery. [ABSTRACT FROM AUTHOR]

Aims: Cutaneous syncytial myoepithelioma (CSM) is a rare myoepithelioma variant of skin, characterized by intradermal syncytial growth of spindle cells with a distinct immunophenotype of EMA and S100 positivity and infrequent keratin expression. While CSM was first described as a cutaneous tumor, singular non-cutaneous cases have since been reported in bone. We aimed to investigate the clinicopathological features of this variant across all anatomic sites through a large multi-institutional study. Methods and results: We complied a total of 24 myoepitheliomas with syncytial growth from our files. The tumors occurred in 12 male and 12 female patients (M:F = 1:1), with a median age of 31 years (range, 9–69 years). While the majority of tumors (75%, n = 18) occurred in skin, a significant subset (25%, n = 6) arose in non-cutaneous sites, including bone (n = 3), bronchus/trachea (n = 2), and interosseous membrane of tibia/fibula (n = 1). Tumor size ranged from 0.4 to 5.9 cm. Clinical follow-up (7 patients; range 14–202 months; median 56.5 months) showed a single local recurrence 8 years after incomplete skin excision but no metastases; all patients were alive at the time of last follow-up without evidence of disease. Histologically, all tumors were pink at low-power and characterized by a syncytial growth of bland ovoid, spindled, or histiocytoid cells with eosinophilic cytoplasm and prominent perivascular lymphoplasmacytic inflammation. One-third displayed adipocytic metaplasia (8/24). Rare cytologic atypia was seen but was not associated with increased mitotic activity. All tumors expressed S100, SMA, and/or EMA. Keratin expression was absent in most cases. Molecular analysis was performed in 16 cases, all showing EWSR1-rearrangments. In total, 15/15 (100%) harbored an EWSR1::PBX3 fusion, whereas 1 case EWSR1 FISH was the only molecular study performed. Conclusion: Syncytial myoepithelioma is a rare but recognizable morphologic variant of myoepithelioma which may have a predilection for skin but also occurs in diverse non-cutaneous sites. Our series provides evidence supporting a reappraisal of the term "cutaneous syncytial myoepithelioma," as 25% of patients in our series presented with non-cutaneous tumors; thus, we propose the term "syncytial myoepithelioma" to aid pathologist recognition and avoidance of potentially confusing terminology when referring to non-cutaneous examples. The behavior of syncytial myoepithelioma, whether it arises in cutaneous or non-cutaneous sites, is indolent and perhaps benign with a small capacity for local recurrence. [ABSTRACT FROM AUTHOR]

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.

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. [ABSTRACT FROM AUTHOR]

Background: Myoepithelioma is a rare type of salivary gland tumor that mainly occurs in the parotid glands and palate. Case presentation: A 43‐year‐old woman presented with swelling and pain in the right maxillary molar. We performed enucleation and curettage under general anesthesia. The tumor was cyst‐like. Histopathological examination revealed nests of tumor cells diffusely interspersed within fibrotic connective tissues. A few cells were positive for cytokeratin AE1/AE3, α‐SMA, GFAP, and vimentin; S‐100 protein and calponin were also positive. Final diagnosis was myoepithelioma. Conclusion: Over 7 years after the surgery, the postoperative course was good, with no recurrence of the tumor. [ABSTRACT FROM AUTHOR]

Myoepithelioma-like hyalinizing epithelioid tumors are rare neoplasms that share morphological characteristics of myoepitheliomas but lack traditional immunophenotypic findings. Though little is known about these tumors at present, a handful of recent studies have confirmed that they harbor a novel fusion gene known as “OGT-FOXO.” Though closely resembling myoeptheliomas, Myoepithelioma-like hyalinizing epithelioid tumors are considered a distinct tumor entity, and few studies have explored their clinical characteristics or their potential for malignancy. Furthermore, literature describing imaging findings of these tumors is virtually non-existent. Understanding the radiological and pathological differences between Myoepithelioma-like hyalinizing epithelioid tumors and myoepitheliomas is helpful in developing a comprehensive differential for soft tissue neoplasms of the foot. We describe a case of MHET of the foot and correlate MRI findings with pathology in addition to describing surgical technique and implications to care.

Cutaneous myoepithelioma is a rare benign soft tissue neoplasm of myoepithelial cells involving the skin and subcutis. These tumors can be diagnostically challenging. The plasticity of myoepithelial cells leads to wide variability in the cytomorphology, immunophenotype, and genetic features of myoepithelioma. Their protean presentations may mimic malignant neoplasms. Therefore, distinction from malignancy is essential. Herein, we report a case of cutaneous myoepithelioma presenting similarly to Ewing sarcoma, with small round blue cells and an EWSR1 rearrangement. Our case highlights the important morphologic, immunohistochemical, and cytogenetic features of this benign basaloid cutaneous tumor. [ABSTRACT FROM AUTHOR]

Introduction: Oral cavity can be host to multitude of neoplastic, premalignant or non neoplastic pathological lesions. Diagnosis of lesions of oral cavity is always of interest to clinician and pathologist and rely on clinical appearance of lesions. There can be variation in diagnosis of clinical lesion with histopathology. Many oral carcinomas arise within the sites that previously had premalignant lesion. Incidence of oral cancers in population has increased among younger generations related to habits and lifestyle. These lesions during clinical presentation are misleading and create diagnostic dilemma owing to age, sex and distribution of lesions. Understanding distribution of oral mucosal lesions helps to diagnose lesions of oral cavity. Purpose of this study is to observe the variation in clinical diagnosis with histopathological diagnosis in patients with inflammatory, premalignant, benign and malignant lesions of oral cavity and oropharynx and also clinical distribution of lesions of oral cavity and oropharynx lesions by histopathology. Observations: Out of total 105 lesions, ulcer in oral cavity seen in 58 (55.23%) of patients, followed by swelling or feeling of lump in oral cavity in 36 (34.29%) of patients and foreign body sensation in 23 (21.90%) of patients with tongue as most frequent site for most of lesions of oral cavity accounting in 33 (31.43%) of cases, and less frequently lesions were seen in retro molar trigone area in 2 (1.90%) patients. Histopathological diagnosis of premalignant, non neoplastic and inflammatory lesions was made in 24 (22.85%) cases, benign tumours were diagnosed in 14 (13.33%) cases and rest of 67 (63.81%) lesions were malignant. Mucocoel were seen in five (4.76%) cases, radicular cyst was seen in one (0.95%) case of female patient and four cases of Leukoplakia with one case showing mild dysplasia. Among benign tumours 11 (10.47%) patients presented with gingivitis turned out to be squamous papillomas were seen in five (4.76%) cases, fibroma was diagnosed in four (3.80%) cases, pyogenic granuloma was diagnosed in four (3.80%) cases most commonly seen over gingiva and myoepithelioma of minor salivary gland was observed in one (0.95%) case over soft palate. Out of 67 cases of malignant lesions squamous cell carcinomas were seen in 59 (88.05%) cases followed by verrucous carcinoma in 3 (4.47%) cases, 2 (2.99%) cases were basaloid squamous cell carcinomas, mucoepidermoid carcinoma was seen in 2 (2.99%) cases and 1 (1.49%) case of adenoid cystic carcinoma was seen. Majority of squamous cell carcinomas cases in study were well differentiated in 49 (73.13%) cases followed by moderately differentiated in 16 (23.88%) cases and poorly differentiated in 2 (2.99%) cases. Malignant transformation of tonsil tissue post operatively was observed in 1 (0.95%) patients on histopathology. One (2.5%) case of myoepithelioma was seen in 60 years male over soft palate. Conclusion: Of all oral biopsies reported in study, increasing trend of malignancies in lower age groups of population making it an emerging threat to community and highlighting need to take effective measures to increase public awareness about risk factors and consequences of this condition. Screening programmes targeted to population over 25 years are recommended to overcome this. [ABSTRACT FROM AUTHOR]

We present a rare case of myoepithelioma in the subcutaneous layer of the shoulder with ultrasonography (US) and magnetic resonance imaging (MRI). US showed a lobulated hyperechoic mass, leading to an impression of lipoma. MRI showed the mass with low signal intensity on T1-weighted images (T1WI), high signal intensity on fat-suppressed T2-weighted images (T2WI), intermediate signal intensity on T2WI, and intense enhancement with adjacent fascial thickening. Imaging findings of soft tissue myoepithelioma have not been established. We report its US and MRI features mimicking features from a lipomatous tumor to infiltrative malignancy. Although soft tissue myoepithelioma has nonspecific image findings to confirm its diagnosis, some findings may help to make the differential diagnosis. Preoperative pathologic confirmation is recommended in a soft tissue neoplasm. [ABSTRACT FROM AUTHOR]

Background: Myoepithelial neoplasms of the salivary gland are benign or malignant neoplasms composed exclusively of neoplastic myoepithelial cells. These tumors, including the benign myoepithelioma and the malignant counterpart myoepithelial carcinoma, exhibit a wide range of cytomorphologic features and architectural patterns. Methods: Review. Results: Myoepithelial cells can be epithelial, plasmacytoid, clear cell, spindle cell, and/or oncocytic cell, arranging as trabeculae, solid sheets, nests, cords, and/or single cells. A stromal component is commonly but not universally present, Therefore, their differential diagnoses are quite broad, including salivary gland neoplasms especially those with a myoepithelial component, plasmacytoma, melanoma, and various mesenchymal tumors. Conclusion: In this review, we summarize the characteristic histologic features, useful immunohistochemical panel, and common molecular alterations of myoepithelial tumors and their top differential diagnoses. A logical stepwise algorithmic approach and an immunohistochemical panel to include multiple myoepithelial markers are essential to establish the correct diagnosis. [ABSTRACT FROM AUTHOR]

Myoepithelial neoplasms (MNs) of the lung are extremely rare tumors. Approximately 40 cases of pulmonary MNs have been reported to date. Herein, we report extremely rare cases of different types of pulmonary MN, including cytological features. Case 1 is an 18-year-old female, and case 2 is a 73-year-old female patient. They presented to our hospital with nodules of the lung. Histological examination revealed tumor cells with round to oval nuclei and acidophilic cytoplasm that formed nests or fascicles with mild hyalinized stroma in case 1 and tumors containing the bi-phasic components of a nest-like and fascicle pattern with pleomorphism in case 2. Immunohistochemically, these tumors were positive for cytokeratin (CK) AE1/AE3, CK5/6, vimentin, calponin, and EMA, and focal positive for S-100a protein and alpha smooth muscle actin. The pathological diagnoses in cases 1 and 2 were myoepithelioma and myoepithelial carcinoma, respectively. In conclusion, we encountered two cases of extremely rare MNs that occurred in the lung. This disease can be diagnosed by collecting appropriate cytological and histological findings and should be listed as a differential diagnosis.

Abstract Background Endotracheal tumors are rare in the respiratory system. Myoepitheliomas are benign tumors, which are rarely reported in the respiratory system. Herein, we report a rare case of endotracheal myoepithelioma, which was resected by rigid bronchoscopy. Case presentation A 36-year-old man, presenting with chest pain, dyspnea, stridor, and hemoptysis, was referred to our center with radiological features of near-total tracheal obstruction due to mass. Fiberoptic bronchoscopy with argon plasma coagulation and rigid bronchoscopy with grasper forceps was utilized to resect the mass. Pathological evaluation of the mass demonstrated myoepithelioma. The patient was discharged in good condition. Now, after 6 months, the patient is symptom-free with no evidence of tumor recurrence or re-growth. Conclusions Despite being extremely rare, myoepithelioma should be considered a possible differential diagnosis for endotracheal tumors. Fiberoptic and rigid bronchoscopy management is an effective method for the resection of endotracheal tumors.

Abstract Background Parotid tumors are frequently encountered by otolaryngologists, and the vast majority of these are of the pleomorphic variety. Myoepitheliomas represent a small subset of parotid tumors. Due to their lack of specificity, they can easily be mistaken by an untrained eye for a pleomorphic adenoma. However, the recent advent of immunohistochemical staining of the tumors has aided in their diagnosis. These tumors show both benign and malignant potential; therefore, careful evaluation and subsequent appropriate management are warranted. Case presentation Here, we present a case of a young female patient who presented with a parotid lesion diagnosed as a case of myoepithelioma and dealt with accordingly. She underwent superficial parotidectomy under general anesthesia. A 2 × 2 cm firm nodular swelling was excised from the left parotid gland, and the facial nerve was identified and preserved. Conclusions The management of these lesions is usually centered on the surgeon’s experience and opinion as there have not been enough reported cases required for the formulation of an evidence-based guideline, hence more single-center studies are essential for evidence-based management.

A myoepithelioma is a type of salivary gland tumor composed entirely of myoepithelial cells. Previously, this was considered a subtype of pleomorphic adenoma. Here, we report an extremely rare case of myoepithelioma of the upper lip. A 33-year-old female patient presented with a painless mass on her upper lip. Magnetic resonance imaging revealed a 23 mm × 18 mm well-defined tumor. We clinically suspected a benign minor salivary gland tumor and the patient underwent complete resection of the tumor under general anesthesia. Since the tumor was diagnosed as a myoepithelioma by rapid pathological examination, it was resected with a safety margin of approximately 5 mm. Pathologically, this tumor showed mild to moderate cell atypia, and focal invasion into the capsule. Immunohistochemical findings showed that the tumor was positive for the S-100 protein and vimentin. The Ki-67 labeling index was less than 1%, and there were no malignant findings. The postoperative clinical course has been good for approximately 5 years, and satisfactory results were obtained both functionally and esthetically. Herein, we also discussed the clinical and immunohistochemical features of myoepitheliomas.

Background: Myoepithelial tumors have been widely described as a rare form of salivary gland neoplasm, although currently soft-tissue phenotypes have also been identified. These are tumors composed entirely of myoepithelial cells that exhibit a dual epithelial and smooth muscle phenotype. The occurrence of myoepithelial tumors within the central nervous system is also extremely rare, with only a few cases reported. Treatment options include surgical resection, chemotherapy, radiotherapy, or a combination of these approaches. Case Description: The authors present a case of soft-tissue myoepithelial carcinoma with an unusual brain metastasis, rarely described in the literature. The purpose of this article is to present an update on the diagnosis and treatment of this pathology when affecting the central nervous system, through the review of the current evidence. Conclusion: However, despite complete surgical resection, there is about a significative high rate of local recurrence and metastasis. Careful patient follow-up and staging is essential for better characterization and understanding of this tumor's behavior. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Myoepithelioma is a rare benign neoplasm, most commonly derived from salivary glands, but there are limited cases of extra salivary gland involvement too. There is little knowledge on typical investigative findings and, instead, diagnosis relies on immunohistochemistry analysis. To our knowledge, this paper reports the 13th case of sinonasal myoepithelioma in the English literature. Case report: This paper presents a 25-year-old man who complained of chronic nasal obstruction. A sinonasal mass was noted on examination that appeared benign on imaging. Biopsy revealed a grade 2 chondrosarcoma that was endoscopically resected; however, excisional margins were positive. On histopathological review at the multidisciplinary team meeting, the lesion was more in keeping with chondromyxoid fibroma, but immunohistochemistry analysis confirmed a myoepithelioma lesion. In light of this revised diagnosis, quorate opinion was for follow up with active monitoring. Conclusion: Sinonasal tumours require a thorough history, examination and investigation before a treatment plan can be formulated. If there is diagnostic uncertainty, it is important to keep a wide differential list and seek a second specialist opinion where possible. [ABSTRACT FROM AUTHOR]

A 15-year-old girl presented with a mobile lesion with yellowish hue on the posterior lamella of the right lower eyelid adjacent to the punctum. Four years prior, a lesion thought to be a chalazion was excised from the same location. There was subsequent progressive painless enlargement. The patient ultimately underwent an internal excision of the mass. Histopathology demonstrated infiltrative nests and cords of epithelioid and plasmacytoid cells with abundant eosinophilic myxoid-collagenous stroma along with focal infiltration of skeletal muscle with immunohistochemical staining consistent with an invasive myoepithelioma of mixed-cell type. Given concern for invasive disease, the patient underwent subsequent Mohs resection resulting in a 25% full thickness eyelid defect, which was repaired with direct closure of the wedge defect. There has been no recurrence of the disease for 7 months since the Mohs resection. This case illustrates the atypical presentation of an invasive myoepithelioma of the eyelid in a pediatric patient. [ABSTRACT FROM AUTHOR]

Background: Endotracheal tumors are rare in the respiratory system. Myoepitheliomas are benign tumors, which are rarely reported in the respiratory system. Herein, we report a rare case of endotracheal myoepithelioma, which was resected by rigid bronchoscopy.Case Presentation: A 36-year-old man, presenting with chest pain, dyspnea, stridor, and hemoptysis, was referred to our center with radiological features of near-total tracheal obstruction due to mass. Fiberoptic bronchoscopy with argon plasma coagulation and rigid bronchoscopy with grasper forceps was utilized to resect the mass. Pathological evaluation of the mass demonstrated myoepithelioma. The patient was discharged in good condition. Now, after 6 months, the patient is symptom-free with no evidence of tumor recurrence or re-growth.Conclusions: Despite being extremely rare, myoepithelioma should be considered a possible differential diagnosis for endotracheal tumors. Fiberoptic and rigid bronchoscopy management is an effective method for the resection of endotracheal tumors. [ABSTRACT FROM AUTHOR]

Myoepithelial neoplasms (MNs) of the lung are extremely rare tumors. Approximately 40 cases of pulmonary MNs have been reported to date. Herein, we report extremely rare cases of different types of pulmonary MN, including cytological features. Case 1 is an 18-year-old female, and case 2 is a 73-year-old female patient. They presented to our hospital with nodules of the lung. Histological examination revealed tumor cells with round to oval nuclei and acidophilic cytoplasm that formed nests or fascicles with mild hyalinized stroma in case 1 and tumors containing the bi-phasic components of a nest-like and fascicle pattern with pleomorphism in case 2. Immunohistochemically, these tumors were positive for cytokeratin (CK) AE1/AE3, CK5/6, vimentin, calponin, and EMA, and focal positive for S-100a protein and alpha smooth muscle actin. The pathological diagnoses in cases 1 and 2 were myoepithelioma and myoepithelial carcinoma, respectively. In conclusion, we encountered two cases of extremely rare MNs that occurred in the lung. This disease can be diagnosed by collecting appropriate cytological and histological findings and should be listed as a differential diagnosis. [ABSTRACT FROM AUTHOR]

Myoepithelioma, which is benign, is a salivary gland neoplasm representing 1.5% of all the neoplasms of salivary glands. The commonest location of its occurrence is parotid gland. In this condition, there is proliferation of myoepithelial cells arranged in cords, mantles, or nests. A 20-year-old woman presented with a well-circumscribed, asymptomatic swelling on the left half of the hard palate, which was gradually increasing in size for 10 years. A provisional diagnosis of pleomorphic adenoma was made. Computed tomography scan showed a well-defined soft tissue density lesion, which on histopathologic examination revealed a well-circumscribed, partially encapsulated neoplasm comprising monomorphic tumor cells in clusters and lobules with plasmacytoid characteristics. Therefore, it is necessary to consider myoepitheliomas also as one of the differential diagnoses while dealing with palatal swellings.

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 (

Tumours of salivary glands are rare and have various histo-pathological subtypes. Myoepitheliomas were first classified by Sheldon et al. and the criterion to classify or diagnose it was first defined by Barnes et al. and Sciubba and Brannon. Myoepithelioma accounts for less than 1 % of all salivary gland tumours, 40 % of these tumours occur in the parotid gland while 21 % occur in the minor salivary glands. A case of myoepithelioma of a minor salivary gland of the cheek is described, emphasizing the problems of the differential diagnosis. A 40-year-old female reported to the department with a complaint of a cheek bite on her right side for a few months. The physical examination showed a presence of lobulated whitish mucosa on the right buccal mucosa at the level of the occlusal plane, on palpation it revealed a non-painful mass approximately 1.5 cm in radius, mobile to bimanual palpation. An excisional biopsy was performed under local anaesthesia. Microscopic and immunohistochemistry confirmed the tumour to be a myoepithelioma of a minor salivary gland with the absence of definitive features of malignancy. Due to their infrequency and multiplicity of histopathology, myoepitheliomas present difficulties in diagnosis. Cellular varieties can be misdiagnosed as malignancies. A key to determining diagnostic criteria for myoepitheliomas is to study cellular morphology, cytoplasmic filament expression, and ultrastructural features of the tumour and apply this information to defining myoepitheliomas. Myoepitheliomas are rare tumours, utilization of immunohistochemical staining and electron microscopy are useful tools for the diagnosis of myoepitheliomas to ensure proper treatment and follow-up. • Myoepithelioma of minor salivary gland • Buccal fat pad reconstruction • Electron microscopy and Immunohistochemistry • Benign salivary gland pathology [ABSTRACT FROM AUTHOR]

We present a rare case of myoepithelioma in the subcutaneous layer of the shoulder with ultrasonography (US) and magnetic resonance imaging (MRI). US showed a lobulated hyperechoic mass, leading to an impression of lipoma. MRI showed the mass with low signal intensity on T1-weighted images (T1WI), high signal intensity on fat-suppressed T2-weighted images (T2WI), intermediate signal intensity on T2WI, and intense enhancement with adjacent fascial thickening. Imaging findings of soft tissue myoepithelioma have not been established. We report its US and MRI features mimicking features from a lipomatous tumor to infiltrative malignancy. Although soft tissue myoepithelioma has nonspecific image findings to confirm its diagnosis, some findings may help to make the differential diagnosis. Preoperative pathologic confirmation is recommended in a soft tissue neoplasm.

Cutaneous syncytial myoepithelioma (CSM) is a recently recognized variant of myoepithelioma characterized by an intradermal syncytial proliferation of spindled, ovoid, and histiocytoid cells. Immunohistochemically, tumor cells usually show strong expression of S‐100 protein and epithelial membrane antigen (EMA). Here we report a case of CSM in the thigh of a 51‐year‐old Japanese woman. Histopathological findings showed a sheet‐like growth of ovoid cells and histiocytoid cells with an eosinophilic syncytial cytoplasm, and adipocytic metaplasia was widely observed in the tumor. Immunohistochemical staining revealed a diffuse, strong pattern for EMA, smooth muscle actin (SMA), and HHF35, and variable expression of S‐100 protein and p63 in ovoid and histiocytoid cells without significant mitotic figures or pleomorphism. In addition, EWSR1‐PBX3 gene fusion, which is characteristic of CSM, was observed in the tumor. Based on these findings, we diagnosed the patient as having CSM. Our case shows that CSM can exhibit extensive adipocytic metaplasia, which could make its histopathological diagnosis challenging. [ABSTRACT FROM AUTHOR]

Keywords: myoepithelial; acral carcinoma; myoepithelioma EN myoepithelial acral carcinoma myoepithelioma 111 115 5 01/12/22 20220201 NES 220201 INTRODUCTION Cutaneous myoepithelial neoplasms whether benign or malignant are quite uncommon. Other areas showed tumor cells in a nested growth pattern embedded in a fibromyxoid stroma. Sox10-a marker for not only schwannian and melanocytic neoplasms but also myoepithelial cell tumors of soft tissue: a systematic analysis of 5134 tumors. The study found 45% of deep-seated soft tissue and bone tumors showed the presence of an I EWSR1 i gene rearrangement by FISH SP .12 sp The same study revealed an I EWSR1-POU5F1 i fusion in myoepithelial tumors with clear cell morphology. [Extracted from the article]

Myoepithelioma‐like tumours of the vulvar region (MELTVR) are a newly described group of spindle cell neoplasm. Morphologically, they consist of epithelioid to spindled cells in a myxoid to collagenous stroma and can resemble epithelioid sarcomas, myoepithelial carcinomas or extraskeletal myxoid chondrosarcomas. However, they have a distinct pattern of immunohistochemical staining characterised by positivity for EMA, ER, PR and negativity for cytokeratin, GFAP and S100. Nuclear staining for INI‐1 is lost. In addition, they lack the characteristic gene arrangements of these other lesions. MELTVR can recur locally when incompletely excised, however, do not appear to metastasize. We report a case of a MELTVR in a 49‐year‐old woman arising in the right mons pubis. [ABSTRACT FROM AUTHOR]

Soft tissue myoepithelioma (STM), a rare mesenchymal neoplasm morphologically analogous to its more common salivary gland (SG) counterpart, is the subject of single case reports regarding its fine-needle aspiration (FNA) biopsy. To our knowledge, ours is the first case series of STM. A search was made of our pathology databases for cases diagnosed as STM. FNA biopsy smears and cell blocks were performed using standard techniques. Seven cases were retrieved from 4 men and 3 women (M:F = 1.3:1; age range: 25-79 years, x = 54 years). All but 1 presented as a primary neoplasm. Six aspirates were from the extremities, and 1 from the abdominal wall. Mean tumor size was 5.7 cm. Cytologic diagnosis of STM or suspicious for STM was made in 3 cases (43%). Remaining FNA diagnoses were spindle cell neoplasm/lesion (2), spindle cell sarcoma (1), and extraskeletal myxoid chondrosarcoma (1). Three cases were composed primarily or solely of uniform spindle cells, 3 primarily of uniform epithelioid cells with plasmacytoid features, and 1 case a mixture of these 2 cell types. Myxoid/chondromyxoid stroma was relatively abundant except in the single hypocellular example. Immunohistochemical (IHC) testing performed in 71% was nonspecific, but positive with S-100 in 4 of 5, EMA in 3 of 3, calponin in 2 of 2, and keratin in 1 of 3 examples. FNA biopsy smears of STM are remarkably similar cytomorphologically to their SG equivalent. However, STM can be misidentified principally as extraskeletal myxoid chondrosarcoma, thus requiring a relatively broad IHC panel for a specific diagnosis. • A series of 7 fine-needle aspiration (FNA) biopsy cases of soft tissue myoepithelioma (STM) is presented. • Correct FNA diagnosis was suggested in 43% of cases. • STM cytomorphology is composed of spindle and epithelioid cells, but 1 cell type is usually predominant. • STM FNA cytopathology imitates its salivary gland counterpart. • Extraskeletal myxoid chondrosarcoma is a close mimic of STM in FNA smears. [ABSTRACT FROM AUTHOR]

Copyright of Revista Salud Bosque is the property of Universidad El Bosque and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

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. [ABSTRACT FROM AUTHOR]

Copyright of Polish Otorhinolaryngological Review / Polski Przegląd Otorynolaryngologiczny (Index Copernicus) is the property of Index Copernicus International and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Myoepithelial tumors are rare neoplasms that develop from myoepithelial cells in glandular structures and soft tissues. Primary intracranial myoepithelial neoplasms are even rarer with around ten cases reported. On the other hand, adrenocortical carcinoma (ACC) is also uncommon with an annual incidence of 0.7–2 per million and carries a poor prognosis. It is known to have an association with certain familial cancer syndromes. Even in sporadic cases, a significant portion of them had other malignancies before and after diagnosis of ACC. We reported a 34-year-old gentleman who was diagnosed to have ACC without known familial cancer syndrome. After that, he was also found to have right occipital myoepithelioma that was confirmed by excisional biopsy. There was no known association between these two pathologies. This is the first report of coincidence of ACC and intracranial myoepithelioma. [ABSTRACT FROM AUTHOR]

Myoepitheliomas (MEs) are extremely rare benign neoplasms composed of ectodermally derived contractile smooth muscle cells (myoepithelial cells). Various tissues such as the salivary glands, breast, larynx, and sweat glands show the presence of these myoepithelial cells. They occur, principally, in the parotid gland and infrequently in minor salivary glands. The term "Myoepitheliomas" was first coined by Sheldon in 1943. It is an uncommon salivary gland tumor which accounts for < 1% of all major and minor salivary gland tumors. Batasakis considers the ME to be "one-sided" variant at the opposite end of the spectrum from the pleomorphic adenoma. There are distinct histological and immunohistochemical characteristics of the tumor which aid in the diagnosis. ME of the palate is uncommon, and only a limited number of cases have been reported in the English literature. It shows a benign clinical course with recurrence in up to 20% of cases without metastasis. The present article sheds light on the presence of ME of minor salivary glands in the palate of 9-year-old child. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

SMARCB1/INI1-deficient soft tissue tumors with epithelioid and myxoid features are diverse and mainly include soft tissue myoepithelial tumor, extraskeletal myxoid chondrosarcoma, and the recently described myoepithelioma-like tumor of the vulvar region and myxoepithelioid tumor with chordoid features. Because of their overlapping features, the accurate diagnosis and classification of these tumors are often challenging. Herein, we report two unique cases of SMARCB1/INI1-deficient soft tissue neoplasm with epithelioid and myxoid features occurring in male paratesticular region. The first case was a 52-year-old man presented with an intermittent painful left paratesticular mass for 1 year. The second case was a 41-year-old man presented with a painless paratesticular mass on the right side for 3 months. Both patients underwent an orchiectomy. After 6 and 26 months of follow-up, both were alive with no evidence of recurrence or metastasis. In both cases, the tumor was relatively well-demarcated and showed monomorphic round to epithelioid cells arranged in a nested, trabecular, reticular, and corded pattern, setting in a myxohyalinized and vascularized matrix. The tumor cells showed relatively uniform round nuclei with vesicular chromatin and variably prominent nucleoli. No rhabdoid cells were identified. Mitoses numbered 3 and 2 per 10 high-power fields. Tumor necrosis or lymphovascular invasion was absent. Immunohistochemically, both tumors expressed epithelial membrane antigen (focal), calponin (focal), and CD99. SMARCB1/INI1 expression was deficient in both cases. In addition, case 1 diffusely expressed pan-cytokeratin, and case 2 diffusely expressed CD34 and synaptophysin. Molecular genetically, case 1 showed SMARCB1 homozygous deletion as detected by fluorescence in-situ hybridization (FISH), and case 2 demonstrated SMARCB1 copy number deletions by next-generation sequencing and SMARCB1 monoallelic deletion by FISH. Both cases lacked EWSR1 rearrangements by FISH. The overall clinicopathologic profiles of the two cases made it difficult to classify them as one of the established categories of SMARCB1/INI1-deficient mesenchymal tumors. Our study further expands the clinicopathologic and molecular spectrum of SMARCB1/INI1-deficient epithelioid and myxoid neoplasms and highlights the challenges to diagnose these tumors. • SMARCB1/INI1-deficient soft tissue neoplasms with epithelioid and myxoid features in paratesticular region are exceptional. • Heterogeneity in morphology, immunohistochemistry, and molecular genetics could elicit a wide range of differential diagnoses. • These tumors may have close relationship to myoepithelioma-like tumors of the vulvar region or myxoepithelioid tumors with chordoid features. [ABSTRACT FROM AUTHOR]

Myoepithelioma is an extremely rare subtype of salivary gland tumors, and its diagnosis is made on a wide variation of cellular morphology. Though benign, it has a tendency for local metastasis and has a low recurrence rate. Its diagnosis can be challenging due to its poorly characterized clinical, histological, and immunochemical behavior. Here, we present a rare presentation of this tumor in the lateral pharyngeal wall.

Myoepithelial tumors are rare neoplasms that primarily occur in the salivary gland but over the last decade have also been described in other locations, including soft tissue. Myoepithelial carcinoma of soft tissue has shown to have a recurrence rate of 39–42% and distant metastases in 32–53% of patients. The only known predictor of malignancy in soft tissue myoepithelial tumors described is cytologic atypia. There are no other validated criteria for differentiating benign and malignant tumors, neither for grading nor for standard of care for these tumor types. Herein, we present a case of myoepithelial carcinoma of inguinal soft tissue in a 37‐year male. We also describe the molecular and previously sparingly described detailed electron microscopic features of this case. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Copyright of Jornal Brasileiro de Patologia e Medicina Laboratorial is the property of Sociedade Brasileira de Patologia Clinica and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Introduction: Myoepithelioma (ME) is a rare salivary gland tumor. Constructed aroung a clinical case, this article aims to gather up up-to-date epidemiological, clinical and histological data about myoeptihelioma with emphasis on the diagnostic approach and differential diagnoses, paraclinical exams and the main histological features reported for its characterization. Observation: A 41-year-old female, presenting a 1-year slowly enlarging palatine nodule was referred to the Oral Pathology Consultation. Clinical data and paraclinic examination were non-specific. A thorough histological examination, comparing clinical data with cyto-architectural and immunostaining profile of the tumor allowed a positive diagnosis of ME. Discussion: The clinical aspect of ME is close from other more frequent tumors within the same areas. Accordingly, its discovery is often incidental and its diagnosis histological. ME display variable architecture and composition, requiring full tumor examination for proper diagnosis. When benign, ME act as mixed tumor regarding local extension, prognosis and recurrence. Malignant ME behaves as a low-grade malignant tumor with metastatic potential. Conclusion: Despite its rarity, ME should be hypothesized in front of a palatine nodule. Clinician and pathologist should be particularly cautious regarding nature, malignancy and follow-up of this tumor, since few data are up-to-now available.

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. [ABSTRACT FROM AUTHOR]

Cutaneous syncytial myoepithelioma (CSM) is a rare tumor with a nondescript clinical presentation in the skin. It represents a relatively uncommon diagnostic entity with approximately 50 examples in the literature to date. We present a 36-year-old man with a new, tender 3-mm firm pink papule on the left bicep, in which a superficial shave technique produced a diagnostic challenge. CSM often poses a diagnostic quandary given shared histomorphological and immunohistochemical attributes with superficial mesenchymal or neurocristic tumors, namely, melanocytic proliferations, fibrous histiocytoma, and epithelioid sarcoma. The molecular profile of CSM may reveal EWSR1 or FUS gene rearrangement, but as we showcase, the diagnosis remains possible in the absence of this oncogenic fusion. Ultimately, there are pitfalls to avoid to correctly distinguish this benign myoepithelial lesion from more biologically aggressive neoplasia. [ABSTRACT FROM AUTHOR]

Myoepithelioms are rare benign salivary gland tumors representing 1–1.5% of all salivary gland tumors. It was once considered to be one end of the histologic spectrum of pleomorphic adenoma (PA), but myoepitheliomas today are believed to be distinct entity. Herein we report a case of myoepithelioma in a 49 years old female patient with the history of swelling in the soft and hard palate. The diagnosis was made on the basis of histopathological findings and immunohistochemical report.