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The Journal of International Advanced... Aug 2020Parachordoma is a rare soft tissue mixed tumor, associated with soft tissue myoepithelioma. It is typically growing slowly and considered less aggressive than other...
Parachordoma is a rare soft tissue mixed tumor, associated with soft tissue myoepithelioma. It is typically growing slowly and considered less aggressive than other similar soft tissue tumors. However, it does recur sporadically, and on rare occasions, it has demonstrated the ability to metastasize. Although imaging is important, definitive diagnosis is achieved by histology, and it is typically treated by a wide local excision. We present the first reported case of a skull base parachordoma in a 15-year-old boy, managed with a wide local excision and with no signs of recurrence or metastases after 24 months of follow-up.
Topics: Adolescent; Chordoma; Diagnosis, Differential; Humans; Male; Myoepithelioma; Skull Base; Skull Base Neoplasms; Soft Tissue Neoplasms; Temporal Bone
PubMed: 32147599
DOI: 10.5152/iao.2020.7203 -
Zhongguo Fei Ai Za Zhi = Chinese... Feb 2020Pulmonary epithelial-myoepithelial carcinoma is a very rare type of salivary gland lung tumor. No standard treatment plan yet. This article intends to analyze the...
BACKGROUND
Pulmonary epithelial-myoepithelial carcinoma is a very rare type of salivary gland lung tumor. No standard treatment plan yet. This article intends to analyze the clinical characteristics of pulmonary epithelial-myoepithelial carcinoma and discuss the diagnosis and treatment of pulmonary epithelial-myoepithelial carcinoma.
METHODS
The clinical data of a patient with pulmonary epithelial-myoepithelial carcinoma were analyzed and other relevant clinical literatures were reviewed.
RESULTS
Epithelial cells immunohistochemically expressed cytokeratin and myoepithelial cells immunohistochemically expressed SMA and S-100. The next-generation sequencing was mainly HRAS gene mutation and the express of PD-L1 protein was negative.
CONCLUSIONS
Most of the patients with Pulmonary epithelial-myoepithelial carcinoma have a good prognosis. Diagnosis mainly depends on microscopic examination and immunohistochemistry. The treatment of pulmonary epithelial-myoepithelial carcinoma is mainly surgical resection. The effect of radiotherapy and chemotherapy is not clear.
Topics: B7-H1 Antigen; Female; Humans; Lung Neoplasms; Middle Aged; Mutation; Myoepithelioma; Proto-Oncogene Proteins p21(ras)
PubMed: 32093457
DOI: 10.3779/j.issn.1009-3419.2020.02.08 -
Medicine Jan 2020Epithelial-myoepithelial carcinoma is an extremely rare, malignant neoplasm that occurs most frequently in the major salivary glands and accounts for approximately 1% of... (Review)
Review
RATIONALE
Epithelial-myoepithelial carcinoma is an extremely rare, malignant neoplasm that occurs most frequently in the major salivary glands and accounts for approximately 1% of all salivary gland neoplasms. Few reports have described the presence of epithelial-myoepithelial carcinoma in the sinonasal region; hence, the treatment guideline and prognosis remain unclear.
PATIENT CONCERNS
We reported a case of a 75-year-old woman with complaint of nasal obstruction and frequent epistaxis for 3 years. During the nasal endoscopic examination, a mass in the left nasal cavity originating from the left nasal septum that caused bleeding on touch was observed.
DIAGNOSES
A diagnosis of epithelial-myoepithelial carcinoma was made based on the features of histopathology and immunohistochemistry of the surgical specimens. The patient was treated by surgical removal of the septal mass using the endonasal endoscopic approach.
OUTCOMES
In the serial follow-up paranasal sinus imaging and endoscopic inspection, evidence of recurrence was absent for 18 months after surgery.
LESSONS
This report highlights a case of epithelial-myoepithelial carcinoma originating from a minor salivary gland in the nasal septum, one of the most unusual locations. Diagnosis of epithelial-myoepithelial carcinoma should be made based on the findings of immunohistochemistry of the operative specimen. Clinicians should consider complete surgical resection as the effective treatment of choice.
Topics: Aged; Carcinoma; Diagnosis, Differential; Female; Humans; Myoepithelioma; Nasal Septum; Salivary Gland Neoplasms; Salivary Glands, Minor
PubMed: 32000457
DOI: 10.1097/MD.0000000000019072 -
Genes, Chromosomes & Cancer Jun 2020Myoepithelial tumors (MET) represent a clinicopathologically heterogeneous group of tumors, ranging from benign to highly aggressive lesions. Although MET arising in...
Myoepithelial tumors (MET) represent a clinicopathologically heterogeneous group of tumors, ranging from benign to highly aggressive lesions. Although MET arising in soft tissue, bone, or viscera share morphologic and immunophenotypic overlap with their salivary gland and cutaneous counterparts, there is still controversy regarding their genetic relationship. Half of MET of soft tissue and bone harbor EWSR1 or FUS related fusions, while MET arising in the salivary gland and skin often show PLAG1 and HMGA2 gene rearrangements. Regardless of the site of origin, the gold standard in diagnosing a MET relies on demonstrating its "myoepithelial immunophenotype" of positivity for EMA/CK and S100 protein or GFAP. However, the morphologic spectrum of MET in soft tissue and bone is quite broad and the above immunoprofile is nonspecific, being shared by other pathogenetically unrelated neoplasms. Moreover, rare MET lack a diagnostic immunoprofile but shows instead the characteristic gene fusions. In this study, we analyzed a large cohort of 66 MET with EWSR1 and FUS gene rearrangements spanning various clinical presentations, to better define their morphologic spectrum and establish relevant pathologic-molecular correlations. Genetic analysis was carried out by FISH for EWSR1/FUS rearrangements and potential partners, and/or by targeted RNA sequencing. Then, 82% showed EWSR1 rearrangement, while 18% had FUS abnormalities. EWSR1-POU5F1 occurred with predilection in malignant MET in children and young adults and these tumors had nested epithelioid morphology and clear cytoplasm. In contrast, EWSR1/FUS-PBX1/3 fusions were associated with benign and sclerotic spindle cell morphology. Tumors with EWSR1-KLF17 showed chordoma-like morphology. Our results demonstrate striking morphologic-molecular correlations in MET of bone, soft tissue and viscera, which might have implications in their clinical behavior.
Topics: Adolescent; Adult; Bone Neoplasms; Child; Child, Preschool; Female; Gene Fusion; Gene Rearrangement; Humans; Infant; Lung Neoplasms; Male; Middle Aged; Myoepithelioma; Octamer Transcription Factor-3; Phenotype; RNA-Binding Protein EWS; RNA-Binding Protein FUS; Soft Tissue Neoplasms; Transcription Factors; Viscera
PubMed: 31994243
DOI: 10.1002/gcc.22835 -
The American Journal of Case Reports Jan 2020BACKGROUND Myoepithelioma is a rare neoplasm that differentiates toward myoepithelial cells. This condition mainly occurs in the salivary gland and rarely in the soft...
BACKGROUND Myoepithelioma is a rare neoplasm that differentiates toward myoepithelial cells. This condition mainly occurs in the salivary gland and rarely in the soft tissue or internal organs. Long-term survival with repeated multiple rounds of resection for recurrence is rarely reported. CASE REPORT A 69-year-old man was diagnosed with metachronous pancreatic and thyroid metastases from myoepithelioma, which initially originated from a resected soft-tissue lesion in the left clavicular region in 2007. In addition, a locally recurrent lesion was resected and the patient received brachytherapy in 2015. Moreover, a metachronous metastatic lesion in the right lung was resected in 2017. Histopathological examination confirmed that all lesions were myoepithelioma. In the present case, pancreatoduodenectomy and right hemithyroidectomy for both metastatic lesions were successfully performed. Histopathology revealed small round-to-spindle-shaped tumor cells with atypia, proliferating in reticular formation, accompanied by myxoid stroma with chondromyxoid and hyalinized stroma, and the histology was similar to that observed in the previous specimen. Immunohistochemistry revealed positivity for cytokeratin (AE1/AE3), glial fibrillary acidic protein, vimentin, and S-100, and confirmed the diagnosis of myoepithelioma. To the best of our knowledge, this is the first study presenting a long-term survivor of soft-tissue myoepithelioma who underwent repeated multiple rounds of resection for recurrence in various organs. CONCLUSIONS We reported the case of a long-term survivor of soft-tissue myoepithelioma requiring multiple rounds of surgical resection for local recurrence and metachronous metastases in the lung, pancreas, and thyroid. When managed appropriately, some patients might benefit in terms of survival from repeated resection of recurrent lesions.
Topics: Aged; Clavicle; Humans; Lung Neoplasms; Male; Myoepithelioma; Pancreatic Neoplasms; Soft Tissue Neoplasms; Thyroid Neoplasms
PubMed: 31983728
DOI: 10.12659/AJCR.920702 -
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 -
Myoepithelioma-like tumor of the vulvar region: a case report in China and review of the literature.Diagnostic Pathology Jan 2020Myoepithelioma-like tumor of the vulvar region (MELTVR) is a recently described mesenchymal neoplasm which typically arising in vulvar regions of adult women. (Review)
Review
BACKGROUND
Myoepithelioma-like tumor of the vulvar region (MELTVR) is a recently described mesenchymal neoplasm which typically arising in vulvar regions of adult women.
CASE PRESENTATION
Here we report a case of a 65-year-old woman who presented with a 6-year history of subcutaneous mass in the vulvar region. The mass had recently increased in size continuously. Histologically, the tumor cells had an epithelioid to spindled shape. Epithelioid tumor cells proliferated singly or in a loosely cohesive manner with myxoid areas, while spindled tumor cells grew in diffuse sheets or storiform arrangements mainly in nonmyxoid areas. Immunohistochemically, the tumor cells were positive for vimentin, epithelial membrane antigen, calponin, and were partially mild to moderate positive for estrogen receptor, but completely negative for S100 protein, glial fibrillary acidic protein, CD34, desmin, SMA and cytokeratin. INI1/SMARCB1 expression was deficient. EWSR1 and FUS genes were intact tested by fluorescence in situ hybridization analysis. Based on these findings, we diagnose this case as MELTVR. The patient remained relapse-free after the lesion was widely excised during 8 months follow-up.
CONCLUSIONS
This disease should be included in the differential diagnostic list of vulvar tumors with epithelioid to spindled morphology. Recognition of its histopathological features and immunohistochemical reactivity will help to understand the tumor better.
Topics: Aged; Biomarkers, Tumor; China; Diagnosis, Differential; Female; Humans; Immunohistochemistry; In Situ Hybridization, Fluorescence; Myoepithelioma; SMARCB1 Protein; Vulvar Neoplasms
PubMed: 31915021
DOI: 10.1186/s13000-019-0923-0 -
Journal of Medical Cases Jan 2020Soft-tissue myoepithelioma (STM) is an extremely rare benign tumor with predominant occurrence in head, neck, pelvic girdle and limbs. These tumors lack specific...
Soft-tissue myoepithelioma (STM) is an extremely rare benign tumor with predominant occurrence in head, neck, pelvic girdle and limbs. These tumors lack specific clinical and morphological features and can easily be confused with more common neoplasms. It may lead to incorrect diagnosis and management. Here, we present a clinical case of a young man with retroperitoneal STM which simulated a pancreatic tumor and required a distal pancreatectomy. Performed literature review highlights current data about clinical, morphologic, immunohistochemical and genetic evaluation, treatment and prognosis of STM.
PubMed: 34434330
DOI: 10.14740/jmc3407 -
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 -
Neurology India 2019Intracranial myoepithelial tumors are extremely rare with <10 cases reported outside the sellar region. The authors describe a case of a 43-year-old male patient who... (Review)
Review
Intracranial myoepithelial tumors are extremely rare with <10 cases reported outside the sellar region. The authors describe a case of a 43-year-old male patient who presented with headache, numbness in the face, and a dumbbell-shaped lesion in the Meckel's cave clinically and radiologically suggestive of a Schwannoma. The histopathological and immunohistochemical evaluation led to a diagnosis of myoepithelioma. A review of literature reveals that this is only the ninth case of intracranial myoepithelial tumor reported, fifth benign case, and the first to be reported in the Meckel's cave region.
Topics: Adult; Brain Neoplasms; Humans; Male; Myoepithelioma
PubMed: 31744974
DOI: 10.4103/0028-3886.271273