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BMC Musculoskeletal Disorders Jul 2022The purpose of this study was to investigate the feasibility of using a three-dimensional (3D)-printed arthrodesis prosthesis for reconstruction of the proximal humeral...
Efficacy and safety of a 3D-printed arthrodesis prosthesis for reconstruction after resection of the proximal humerus: preliminary outcomes with a minimum 2-year follow-up.
BACKGROUND
The purpose of this study was to investigate the feasibility of using a three-dimensional (3D)-printed arthrodesis prosthesis for reconstruction of the proximal humeral defect after tumor resection.
METHODS
A novel proximal humeral prosthesis was designed to restore bone continuity and shoulder arthrodesis and was fabricated via 3D printing technology. Ten patients with primary malignancies in the proximal humerus underwent intra-articular resection and replacement with this prosthesis from 2017 to 2019. Baseline and operative data, oncological and prosthetic survival, and functional status were summarized.
RESULTS
This cohort consisted of 9 males and 1 female with a mean age of 32.1 ± 16.1 years. Diagnoses included 5 cases of osteosarcoma, 3 cases of chondrosarcoma and 1 each case of undifferentiated pleomorphic sarcoma and malignant myoepithelioma. The mean operative duration, intraoperative hemorrhage and postoperative length of hospitalization were 151.5 ± 61.0 min, 410.0 ± 353.4 ml and 5.3 ± 1.9 d, respectively. The mean follow-up duration was 29.3 ± 6.4 months, with a minimum of 24 months for the surviving patients. Two patients experienced local recurrence, and four patients developed distant metastases. Detachment of the taper occurred in two patients. One was managed conservatively, and the other received amputation due to concurrent tumor recurrence. The mean MSTS-93 and ASES scores and ranges of forwards flexion and abduction were 24.9 ± 3.1, 79.4 ± 8.3, 71.3 ± 19.4°, and 61.3 ± 16.4°, respectively. The functional outcomes were independent of the preservation of the axillary nerve. Histological study of the glenoid component showed evidence of bone ingrowth at the bone-prosthesis porous interface.
CONCLUSION
Application of the 3D-printed arthrodesis prosthesis might be a safe and efficacious method for functional reconstruction in patients who underwent resection of the proximal humerus, especially for those without preservation of the axillary nerve.
Topics: Adolescent; Adult; Arthrodesis; Artificial Limbs; Bone Neoplasms; Female; Follow-Up Studies; Humans; Humerus; Male; Middle Aged; Printing, Three-Dimensional; Shoulder; Young Adult
PubMed: 35787280
DOI: 10.1186/s12891-022-05581-6 -
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 -
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 -
Cold Spring Harbor Molecular Case... Dec 2022Myoepithelial carcinomas (MECs) of soft tissue are rare and aggressive tumors affecting young adults and children, but their molecular landscape has not been...
Myoepithelial carcinomas (MECs) of soft tissue are rare and aggressive tumors affecting young adults and children, but their molecular landscape has not been comprehensively explored through genome sequencing. Here, we present the whole-exome sequencing (WES), whole-genome sequencing (WGS), and RNA sequencing findings of two MECs. Patients 1 and 2 (P1, P2), both male, were diagnosed at 27 and 37 yr of age, respectively, with shoulder (P1) and inguinal (P2) soft tissue tumors. Both patients developed metastatic disease, and P2 died of disease. P1 tumor showed a rhabdoid cytomorphology and a complete loss of INI1 (SMARCB1) expression, associated with a homozygous deletion. The tumor from P2 showed a clear cell/small cell morphology, retained INI1 expression and strong S100 positivity. By WES and WGS, tumors from both patients displayed low tumor mutation burdens, and no targetable alterations in cancer genes were detected. P2's tumor harbored an rearrangement, whereas the tumor from P1 showed a novel fusion. WGS evidenced a complex genomic event involving mainly Chromosomes 17 and 22 in the tumor from P1, which was consistent with chromoplexy. These findings are consistent with previous reports of rearrangements (50% of cases) in MECs and provide a genetic basis for the loss of SMARCB1 protein expression observed through immunohistochemistry in 10% of 40% of MEC cases. The lack of additional driver mutations in these tumors supports the hypothesis that these alterations are the key molecular events in MEC evolution. Furthermore, the presence of complex structural variant patterns, invisible to WES, highlights the novel biological insights that can be gained through the application of WGS to rare cancers.
Topics: Child; Young Adult; Humans; Male; Myoepithelioma; Soft Tissue Neoplasms; Carcinoma; Biomarkers, Tumor
PubMed: 36577525
DOI: 10.1101/mcs.a006227 -
Journal of Medicine and Life May 2022This study is a systematic review and meta-analysis to assess the overexpression rate of HER2 in patients with salivary gland tumors. We included peer-reviewed... (Meta-Analysis)
Meta-Analysis Review
This study is a systematic review and meta-analysis to assess the overexpression rate of HER2 in patients with salivary gland tumors. We included peer-reviewed publications from 1995 to 2020, indexed in medical databases, using search terms such as "human epidermal growth factor receptor 2 (HER2)" and "salivary gland tumors", and extracted relevant data. The extracted data were analyzed with RevMan 5.3 software. Intra-and intergroup post hoc analyses of outcome variables were performed using t-tests, and the rates of HER2 positivity among studies were evaluated. 80 studies were included in the analysis. The positive rates of HER2 ranged from 3.3% to 84.0% and 1% to 9% in malignant and benign subtypes, respectively. The highest HER2 overexpression rate among malignant tumors was in salivary ductal carcinomas (SDC), with a 45% positive rate (CI 95%: 21.9-70.3%). Mucoepidermoid carcinoma (MEC) had the highest positive rate of 84% (CI 95%: 74.1-90.0%). Among benign salivary gland tumors, the highest rate was found in myoepithelioma, with a positive rate of 9% (CI 95%: 1.7-33.6%). The highest rate of HER2 overexpression is present in malignant subtypes of salivary gland tumors, more specifically in salivary ductal carcinoma, mucoepidermoid carcinomas, salivary duct carcinoma in situ, and carcinoma ex pleomorphic adenoma.
Topics: Biomarkers, Tumor; Carcinoma, Mucoepidermoid; Humans; Receptor, ErbB-2; Salivary Gland Neoplasms; Salivary Glands
PubMed: 35815077
DOI: 10.25122/jml-2021-0394 -
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 -
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 -
Journal of Cardiothoracic Surgery May 2022Endotracheal tumors are rare in the respiratory system. Myoepitheliomas are benign tumors, which are rarely reported in the respiratory system. Herein, we report a rare... (Review)
Review
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.
Topics: Adult; Airway Obstruction; Bronchoscopy; Humans; Male; Myoepithelioma; Neoplasm Recurrence, Local; Trachea
PubMed: 35606819
DOI: 10.1186/s13019-022-01880-0 -
Case Reports in Oncology 2022Myoepithelial 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...
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.
PubMed: 35949910
DOI: 10.1159/000524769 -
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