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1.
Head Neck Pathol ; 18(1): 61, 2024 Jun 28.
Artículo en Inglés | MEDLINE | ID: mdl-38940938

RESUMEN

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.


Asunto(s)
Neoplasias Maxilares , Mioepitelioma , Humanos , Femenino , Mioepitelioma/patología , Adolescente , Neoplasias Maxilares/patología , Neoplasias Maxilares/cirugía , Biomarcadores de Tumor/análisis
2.
Hum Pathol ; 149: 10-20, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-38782103

RESUMEN

Myoepithelial tumors of the soft tissue and bone occurring in patients 21 years of age and younger are rare, and their clinicopathologic features remain incompletely understood. We studied a well-characterized series of 40 such tumors. Cases were retrieved from our archives for the period 2009-2022 and re-reviewed. Available immunohistochemical and molecular genetic data was collected. Clinical information including available follow-up was obtained. The tumors occurred in 18 males and 22 females, ranging from 3 months to 21 years of age (median 11.5 years), and involved a wide variety of soft tissue (n = 36) and bone (n = 4) locations. Histologically benign myoepithelial tumors tended to occur in adolescents (median age 14.5 years; range 5-21 years), whereas myoepithelial carcinomas occurred in younger patients (median age 8.5 years; range 3 months-20 years). Microscopically, the tumors showed a complex admixture of epithelioid, plasmacytoid and spindled cells in a variably hyalinized, myxoid, chondroid or chondromyxoid background. Small subsets of histologically malignant tumors had rhabdoid or "round cell" features. Immunohistochemistry showed 35/40 (88%) cases to be positive with at least one keratin antibody. The 5 keratin-negative tumors were uniformly positive for S100 protein and/or SOX10 and expressed EMA (4 cases) and/or p63 (3 cases). EMA, SMA and GFAP were positive in 21/25 (84%), 13/21 (62%), and 8/21 (38%) tumors, respectively. SMARCB1 and SMARCA4 expression was retained in 29/31 (94%) and 22/22 (100%) of cases, respectively. FISH for EWSR1 gene rearrangement was positive in 6/18 (33%) tested cases. Two EWSR1-negative tumors were also FUS-negative. NGS identified EWSR1::POU5F1, FUS::KLF17, and BRD4::CITED1 gene fusions in 3 tested cases. Clinical follow-up (22 patients; median 23 months; range 1-119 months) showed 3 patients with local recurrences and 5 with distant metastases (lymph nodes, lung, and brain). Three patients died of disease, 3 were alive with recurrent or unresectable disease, and 16 were disease-free. Adverse clinical outcomes were seen only in patients with malignant tumors. We conclude that myoepithelial neoplasms of soft tissue and bone are over-repesented in patients ≤21 years of age, more often histologically malignant, and potentially lethal. Histologic evaluation appears to reliably predict the behavior of these rare tumors.


Asunto(s)
Biomarcadores de Tumor , Neoplasias Óseas , Inmunohistoquímica , Mioepitelioma , Neoplasias de los Tejidos Blandos , Humanos , Masculino , Adolescente , Femenino , Niño , Adulto Joven , Mioepitelioma/patología , Mioepitelioma/genética , Preescolar , Neoplasias de los Tejidos Blandos/patología , Neoplasias de los Tejidos Blandos/genética , Neoplasias Óseas/patología , Neoplasias Óseas/genética , Lactante , Biomarcadores de Tumor/genética , Biomarcadores de Tumor/análisis , Reordenamiento Génico , Factores de Transcripción/genética , Factores de Transcripción/análisis
3.
Genes Chromosomes Cancer ; 63(5): e23244, 2024 05.
Artículo en Inglés | MEDLINE | ID: mdl-38747338

RESUMEN

We describe a case of a pleomorphic adenoma (PA) arising from the para-tracheal accessory salivary gland in a 44-year-old male harboring a novel WWTR1::NCOA2 gene fusion. To our knowledge, this novel gene fusion has not been described previously in salivary gland tumors. The patient presented with hoarseness of voice. The radiological exam revealed a mass in the upper third of the trachea involving the larynx. Histologically, the tumor consisted of bland-looking monocellular eosinophilic epithelial cells arranged in cords and sheets separated by thin fibrous stroma, focally forming a pseudo-tubular pattern. In immunohistochemistry, the tumor cells demonstrated positivity for CK7, PS100, SOX10, and HMGA2; and negativity for CK5/6, p40 p63, and PLAG1. In addition, the clustering analysis clearly demonstrates a clustering of tumors within the PA group. In addition to reporting this novel fusion in the PA spectrum, we discuss the relevant differential diagnoses and briefly review of NCOA2 and WWTR1 gene functions in normal and neoplastic contexts.


Asunto(s)
Proteína HMGA2 , Coactivador 2 del Receptor Nuclear , Transactivadores , Humanos , Masculino , Coactivador 2 del Receptor Nuclear/genética , Coactivador 2 del Receptor Nuclear/metabolismo , Adulto , Proteína HMGA2/genética , Proteína HMGA2/metabolismo , Transactivadores/genética , Proteínas Coactivadoras Transcripcionales con Motivo de Unión a PDZ , Neoplasias de las Glándulas Salivales/genética , Neoplasias de las Glándulas Salivales/patología , Neoplasias de las Glándulas Salivales/metabolismo , Péptidos y Proteínas de Señalización Intracelular/genética , Proteínas de Fusión Oncogénica/genética , Mioepitelioma/genética , Mioepitelioma/patología , Mioepitelioma/metabolismo
4.
Clin Oncol (R Coll Radiol) ; 36(7): e171-e181, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-38664176

RESUMEN

AIM: Epidemiological evidence on myoepithelial carcinoma is rare. This study aimed to investigate the effect of tumor primary site and treatment modality on survival in patients with head and neck myoepithelial carcinoma. MATERIALS AND METHODS: Data on adult patients diagnosed with head and neck myoepithelial carcinoma between 2000 and 2019 were extracted from the Surveillance, Epidemiology, and End Results (SEER) database. Uni- and multivariable Cox proportional hazard models were utilized to evaluate the effects of different tumor primary sites and treatment modalities on overall survival (OS) and cancer-specific survival (CSS), and expressed as hazard ratio (HR) and 95% confidence interval (CI). RESULTS: A total of 415 patients were enrolled. No significant differences in OS and CSS were found between different tumor primary sites (P > 0.05). Compared with partial excision, patients with total excision (HR = 1.65, 95%CI: 1.12-2.42) (partial or total removal of the organ in which the tumor is located and complete removal of the tumor) or no surgery (HR = 3.52, 95%CI: 2.05-6.03) had worse OS. Compared with surgery only, patients with radiotherapy only had poorer OS (HR = 4.69, 95%CI: 2.32-9.46) and CSS (HR = 6.72, 95%CI: 2.59-17.46), while no significant differences in OS (P = 0.120) and CSS (P = 0.847) were found among patients who received surgery combined with radiotherapy. In patients with AJCC III/IV, patients with radiotherapy only (HR = 4.51, 95%CI: 1.61-12.62) had poorer OS compared to those with surgery only, whereas patients who received surgery combined with radiotherapy had better OS (HR = 0.50, 95%CI: 0.29-0.89). CONCLUSION: The tumor primary site may not affect the prognosis of patients with myoepithelial carcinoma, while the effect of treatment modality on prognosis is related to the primary site and stage of the tumor.


Asunto(s)
Neoplasias de Cabeza y Cuello , Mioepitelioma , Humanos , Masculino , Femenino , Mioepitelioma/mortalidad , Mioepitelioma/terapia , Mioepitelioma/patología , Persona de Mediana Edad , Neoplasias de Cabeza y Cuello/mortalidad , Neoplasias de Cabeza y Cuello/terapia , Neoplasias de Cabeza y Cuello/patología , Anciano , Adulto , Programa de VERF , Tasa de Supervivencia , Terapia Combinada , Pronóstico , Estudios Retrospectivos
5.
Am J Surg Pathol ; 48(5): 551-561, 2024 May 01.
Artículo en Inglés | MEDLINE | ID: mdl-38497430

RESUMEN

Recurrent gene fusions are common in salivary gland tumors including benign tumors, such as pleomorphic adenoma (PA) and myoepithelioma (ME). In cases where chromosomal rearrangement is identified in the pleomorphic adenoma gene 1 (PLAG1) gene, different gene partners are found. Oncocytic metaplasia, characterized by oncocytes with abundant eosinophilic granular cytoplasm and hyperchromatic nuclei, is a well-known phenomenon in salivary gland neoplasms. However, the pure oncocytic variant of PA/ME showed PLAG1 gene rearrangements involving various gene partners at the molecular level, without any recurrent fusion being found. Our study includes 20 cases of PA/ME, with 11 females and 9 males. The age of patients ranged from 37 to 96 years, with a median age of 62.8 years. Most tumors originate from the parotid gland. The median size of the tumor was 26.5 mm (range: 13 to 60 mm). Among the 20 cases, 14 were a pure oncocytic variant of PA/ME, whereas 6 cases showed focal oncocytic or oncocytic-like aspects. Molecular studies on 20 cases of PA/ME were conducted. A novel recurrent ZBTB47-AS1::PLAG1 fusion was identified in 6 of 12 cases with pure oncocytic metaplasia, whereas the other cases had PLAG1 gene fusion with different gene partners. The transcriptomic analysis of the cases harboring ZBTB47-AS1::PLAG1 fusion demonstrated that these tumors have a distinct molecular profile from conventional PA/ME. This study reveals a unique subset in the oncocytic PA/ME spectrum characterized by pure oncocytic morphology with larger oncocytic cells and recurrent ZBTB47-AS1::PLAG1 fusion. It also highlights the transcriptomic distinctness of salivary gland adenomas with pure oncocytic metaplasia in the spectrum of salivary gland neoplasms. Further studies are needed to better understand the oncocytic variant of PA/ME and to determine the true nature of oncocytic cells in PA/ME.


Asunto(s)
Adenoma Oxifílico , Adenoma Pleomórfico , Mioepitelioma , Neoplasias de las Glándulas Salivales , Masculino , Femenino , Humanos , Persona de Mediana Edad , Adulto , Anciano , Anciano de 80 o más Años , Adenoma Pleomórfico/genética , Adenoma Pleomórfico/patología , Mioepitelioma/genética , Mioepitelioma/patología , Proteínas de Unión al ADN/genética , Neoplasias de las Glándulas Salivales/genética , Neoplasias de las Glándulas Salivales/patología , Fusión Génica , Metaplasia
9.
Indian J Pathol Microbiol ; 67(1): 223-225, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38358228

RESUMEN

Benign proliferative breast diseases are well recognized in young females. Benign biphasic proliferation of epithelial and myoepithelial cells has been observed, among which adeno-myoepithelial adenosis is one of the rare morphologies published in the literature with the tendency to recur and poses a risk for low-grade malignant transformation. Here, we report a case of a young female who had a history of recurrent breast lump mimicking phyllodes tumor and eventually diagnosed as adeno-myoepithelial adenosis on histopathological examination. Benign proliferative breast diseases are well recognized in young females. Benign biphasic proliferation of epithelial and myoepithelial cells has been observed, among which adeno-myoepithelial adenosis is one of the rare morphologies published in the literature with the tendency to recur and poses a risk for low-grade malignant transformation. Here, we report a case of a young female who had a history of recurrent breast lump mimicking phyllodes tumor and eventually diagnosed as adeno-myoepithelial adenosis on histopathological examination.


Asunto(s)
Neoplasias de la Mama , Enfermedad Fibroquística de la Mama , Mioepitelioma , Tumor Filoide , Femenino , Humanos , Tumor Filoide/diagnóstico , Tumor Filoide/patología , Recurrencia Local de Neoplasia/patología , Enfermedad Fibroquística de la Mama/diagnóstico , Enfermedad Fibroquística de la Mama/patología , Células Epiteliales/patología , Hiperplasia/patología , Transformación Celular Neoplásica/patología , Neoplasias de la Mama/diagnóstico , Neoplasias de la Mama/patología , Mioepitelioma/patología
10.
Mod Pathol ; 37(3): 100430, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38266920

RESUMEN

Cutaneous mixed tumors exhibit a wide morphologic diversity and are currently classified into apocrine and eccrine types based on their morphologic differentiation. Some cases of apocrine-type cutaneous mixed tumors (ACMT), namely, hyaline cell-rich apocrine cutaneous mixed tumors (HCR-ACMT) show a prominent or exclusive plasmacytoid myoepithelial component. Although recurrent fusions of PLAG1 have been observed in ACMT, the oncogenic driver of eccrine-type cutaneous mixed tumors (ECMT) is still unknown. The aim of the study was to provide a comprehensive morphologic, immunohistochemical, and molecular characterization of these tumors. Forty-one cases were included in this study: 28 cases of ACMT/HCR-ACMT and 13 cases of ECMT. After morphologic and immunohistochemical characterization, all specimens were analyzed by RNA sequencing. By immunohistochemistry, all cases showed expression of SOX10, but only ACMT/HCR-ACMT showed expression of PLAG1 and HMGA2. RNA sequencing confirmed the presence of recurrent fusion of PLAG1 or HMGA2 in all cases of ACMT/HCR-ACMT, with a perfect correlation with PLAG1/HMGA2 immunohistochemical status, and revealed internal tandem duplications of SOX10 (SOX10-ITD) in all cases of ECMT. Although TRPS1::PLAG1 was the most frequent fusion, HMGA2::WIF1 and HMGA2::NFIB were detected in ACMT cases. Clustering analysis based on gene expression profiling of 110 tumors, including numerous histotypes, showed that ECMT formed a distinct group compared with all other tumors. ACMT, HCR-ACMT, and salivary gland pleomorphic adenoma clustered together, whereas myoepithelioma with fusions of EWSR1, FUS, PBX1, PBX3, POU5F1, and KLF17 formed another cluster. Follow-up showed no evidence of disease in 23 cases across all 3 tumor types. In conclusion, our study demonstrated for the first time SOX10-ITD in ECMT and HMGA2 fusions in ACMT and further refined the prevalence of PLAG1 fusions in ACMT. Clustering analyses revealed the transcriptomic distance between these different tumors, especially in the heterogenous group of myoepitheliomas.


Asunto(s)
Adenoma Pleomórfico , Mioepitelioma , Neoplasias de las Glándulas Salivales , Neoplasias Cutáneas , Neoplasias de las Glándulas Sudoríparas , Humanos , Adenoma Pleomórfico/genética , Proteínas de Unión al ADN/genética , Proteínas de Unión al ADN/metabolismo , Mioepitelioma/genética , Mioepitelioma/patología , Proteínas Represoras , Neoplasias de las Glándulas Salivales/genética , Neoplasias Cutáneas/genética , Factores de Transcripción SOXE , Neoplasias de las Glándulas Sudoríparas/genética , Factores de Transcripción
11.
World J Surg Oncol ; 22(1): 28, 2024 Jan 24.
Artículo en Inglés | MEDLINE | ID: mdl-38268020

RESUMEN

BACKGROUND: A malignant myoepithelioma is a rare tumor, mostly arising from the salivary glands. Myoepitheliomas of the ear have rarely been reported. The manuscript reports myoepithelial carcinoma of the external auditory canal (EAC) spreading to the infratemporal fossa. A clinician must be aware of anatomical variation of the bony EAC wall, such as the foramen of Huschke. This rare defect may be a pathway for spreading pathologies between these two anatomical regions. CASE REPORT: We present a case of osteoma-like stenosis of the EAC, which turned out to be an extremely rare malignant tumor. The preoperative MRI and PET/CT revealed that two parts of the tumor communicated through a defect in the antero-inferior portion of the bony ear canal. No distant metastases were detected. Subsequently, the tumor was resected from the ear canal and the infratemporal fossa en bloc. Perioperatively the defect in the EAC wall was suspected of the foramen of Huschke. After the surgery, the older scans of the patient from the past showed no presence of a congenital EAC wall defect. Therefore, the authors concluded that the tumor aggressively grew through the bone due to its biological nature. CONCLUSION: Malignant myoepithelioma of the external auditory canal is an extremely rare condition and could be misdiagnosed as other benign lesions. In cases of suspicious lesions, it is advisable to do a probatory biopsy from the EAC. Surgery is the treatment of choice in malignant myoepitheliomas, and regular follow-ups are essential to monitor for recurrence or metastatic disease. Any mass located at the antero-inferior portion of the EAC wall warrants close evaluation due to its potential for expansion from the EAC.


Asunto(s)
Carcinoma , Mioepitelioma , Humanos , Conducto Auditivo Externo/cirugía , Mioepitelioma/cirugía , Relevancia Clínica , Tomografía Computarizada por Tomografía de Emisión de Positrones
12.
Int J Surg Pathol ; 32(3): 511-514, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-37487199

RESUMEN

Concurrent occurrence of schwannoma and meningiomas are rare, and are found especially in association with neurofibromatosis type 2 (NF2). Occurrence of mixed tumor without the aforementioned conditions is extremely rare. We present three cases of mixed tumor in different locations, including two with NF2 and one without NF2. We analyse the relationship of mixed tumor with NF2 and its clinical implications. Presence of mixed schwannoma-meningioma should prompt screening for NF2. Thus aids in early diagnosis of unsuspected NF2 cases. We observed that irrespective of different locations, cases with NF2 showed frequent recurrence of schwannoma as compared to case who did not fit in the existing clinical criteria for NF2. Collision tumor and thereby NF2 mutations indicates the prognosis and recurrence of the tumor, thereby guides in deciding the management.


Asunto(s)
Neoplasias Meníngeas , Meningioma , Mioepitelioma , Neurilemoma , Neurofibromatosis 2 , Humanos , Neurofibromatosis 2/complicaciones , Neurofibromatosis 2/diagnóstico , Neurofibromatosis 2/genética , Meningioma/complicaciones , Meningioma/diagnóstico , Meningioma/genética , Neoplasias Meníngeas/complicaciones , Neoplasias Meníngeas/diagnóstico , Neoplasias Meníngeas/genética , Neurilemoma/complicaciones , Neurilemoma/diagnóstico , Carcinogénesis , Transformación Celular Neoplásica
13.
Int J Oral Maxillofac Surg ; 53(4): 268-274, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-37591716

RESUMEN

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.


Asunto(s)
Carcinoma , Mioepitelioma , Neoplasias de las Glándulas Salivales , Humanos , Pronóstico , Estudios Retrospectivos , Recurrencia Local de Neoplasia/patología , Neoplasias de las Glándulas Salivales/patología , Carcinoma/patología , Mioepitelioma/cirugía , Mioepitelioma/patología , Glándulas Salivales , Estadificación de Neoplasias
14.
Jpn J Clin Oncol ; 54(3): 229-247, 2024 Mar 09.
Artículo en Inglés | MEDLINE | ID: mdl-38018262

RESUMEN

Salivary gland-type tumors of the lung are thought to originate from the submucosal exocrine glands of the large airways. Due to their rare occurrence, reports of their study are limited to small-scale or case reports. Therefore, daily clinical practices often require a search for previous reports. In the last 20 years, several genetic rearrangements have been identified, such as MYB::NF1B rearrangements in adenoid cystic carcinoma, CRTC1::MAML2 rearrangements in mucoepidermoid carcinoma, EWSR1::ATF1 rearrangements in hyalinizing clear cell carcinoma and rearrangements of the EWSR1 locus or FUS (TLS) locus in myoepithelioma and myoepithelial carcinoma. These molecular alterations have been useful in diagnosing these tumors, although they have not yet been linked to molecularly targeted therapies. The morphologic, immunophenotypic, and molecular characteristics of these tumors are similar to those of their counterparts of extrapulmonary origin, so clinical and radiologic differential diagnosis is required to distinguish between primary and metastatic disease of other primary sites. However, these molecular alterations can be useful in differentiating them from other primary lung cancer histologic types. The management of these tumors requires broad knowledge of the latest diagnostics, surgery, radiotherapy, bronchoscopic interventions, chemotherapy, immunotherapy as well as therapeutic agents in development, including molecularly targeted agents. This review provides a comprehensive overview of the current diagnosis and treatment of pulmonary salivary gland tumors, with a focus on adenoid cystic carcinoma and mucoepidermoid carcinoma, which are the two most common subtypes.


Asunto(s)
Carcinoma Adenoide Quístico , Carcinoma Mucoepidermoide , Carcinoma , Neoplasias Pulmonares , Mioepitelioma , Neoplasias de las Glándulas Salivales , Humanos , Carcinoma Adenoide Quístico/diagnóstico , Carcinoma Adenoide Quístico/genética , Carcinoma Adenoide Quístico/terapia , Carcinoma Mucoepidermoide/diagnóstico , Carcinoma Mucoepidermoide/genética , Carcinoma Mucoepidermoide/terapia , Neoplasias de las Glándulas Salivales/diagnóstico , Neoplasias de las Glándulas Salivales/genética , Neoplasias de las Glándulas Salivales/terapia , Carcinoma/patología , Mioepitelioma/patología , Glándulas Salivales/metabolismo , Glándulas Salivales/patología , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/genética , Neoplasias Pulmonares/terapia
15.
Rio de Janeiro; s.n; 2024.
Tesis en Portugués | Coleciona SUS | ID: biblio-1556071

RESUMEN

O carcinoma mioepitelial é uma doença incomum que tem origem majoritariamente nas glândulas salivares. A sua ocorrência no pulmão é um achado extremamente raro e acredita-se ter origem nas glândulas brônquicas submucosas do trato respiratório inferior. Marcado pela sua grande diversidade citológica e arquitetônica, que recapitula as características celulares e imunofenotípicas das células mioepiteliais, é uma neoplasia tipicamente de alto grau e comportamento agressivo, sendo facilmente confundida com outras neoplasias torácicas e representando um desafio diagnóstico. Não há diretrizes estabelecidas para o tratamento ­ a cirurgia parece ser a melhor alternativa, uma vez que o papel da quimioterapia e radioterapia é incerto. Este é um estudo observacional descritivo retrospectivo em modelo de relato de caso realizado através de avaliação do prontuário de um paciente com diagnóstico de carcinoma mioepitelial pulmonar matriculado no INCA no período de setembro a novembro de 2023. Para a discussão do tema, esse trabalho também contou com uma revisão da literatura médica utilizando os dados obtidos através das plataformas Pubmed, Scielo e Google acadêmico. Após extensa pesquisa, encontramos apenas 21 casos publicados de carcinoma mioepitelial pulmonar e acreditamos que a dificuldade técnica envolvida no diagnóstico, sobreposta as barreiras de tecnologia e conhecimento do passado, possam ter contribuído para subestimar o número de casos dessa neoplasia. Nesse contexto, o carcinoma mioepitelial pulmonar primário deve ser incluído no diagnóstico diferencial das neoplasias torácicas. Embora mais estudos sejam necessários para esclarecimento dos aspectos moleculares e estratégias terapêuticas, o tratamento individualizado é uma estratégia clínica promissora para neoplasias torácicas em geral e os eventos moleculares subjacentes devem ser investigados para encontrar os potenciais alvos terapêuticos


Myoepithelial carcinoma is an uncommon disease that originates mainly in the salivary glands. Its occurrence in the lung is an extremely rare finding and is believed to originate from the submucosal bronchial glands of the lower respiratory tract. Marked by its great histological and architectural diversity, which recapitulates the cellular and immunophenotypic characteristics of myoepithelial cells, it is a typically high-grade neoplasm with aggressive behavior, being easily confused with other thoracic neoplasms and representing a diagnostic challenge. There are no established guidelines for treatment ­ upfront surgery appears to be the best alternative, as the role of chemotherapy and radiotherapy is uncertain. This is a retrospective descriptive observational study using a case report model carried out by evaluating the medical records of a patient diagnosed with pulmonary myoepithelial carcinoma enrolled at INCA from september to november 2023. To discuss the topic, this work also included a review of the medical literature using data obtained from Pubmed, Scielo and Google Scholar. We found Only 21 published cases of pulmonary myoepithelial carcinoma on research platforms and we believe that the technical difficulty involved in diagnosis, combined with technology and knowledge barriers from the past, may have contributed to underestimating the number of cases of this neoplasm. In this context, primary pulmonary myoepithelial carcinoma should be included in the differential diagnosis of thoracic neoplasms. Although further studies are needed to clarify the molecular aspects and therapeutic strategies, individualized treatment is a promising clinical strategy for thoracic neoplasms in general and the underlying molecular events must be investigated to find potential therapeutic targets.


Asunto(s)
Humanos , Masculino , Carcinoma , Mioepitelioma , Neoplasias Pulmonares , Biología Molecular
16.
Pract Radiat Oncol ; 14(4): 334-337, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38161001

RESUMEN

Salivary gland cancers are rare in general and salivary duct carcinoma and epithelial myoepithelial carcinomas are rare subtypes. This topic discussion will review the characteristics of these uncommon cancers. Additionally, it will briefly discuss available guidelines for salivary cancers and summarize author opinions on the role of adjuvant radiation therapy for these cases.


Asunto(s)
Mioepitelioma , Neoplasias de las Glándulas Salivales , Humanos , Neoplasias de las Glándulas Salivales/radioterapia , Neoplasias de las Glándulas Salivales/patología , Radioterapia Adyuvante/métodos , Mioepitelioma/radioterapia , Mioepitelioma/patología , Conductos Salivales/patología
17.
J Vet Diagn Invest ; 35(6): 789-794, 2023 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-37786275

RESUMEN

A 9-y-old male Boxer dog developed a mandibular skin tumor, which histologically had a locally invasive growth pattern composed of bilayered structures of inner eosinophilic cuboidal tumor cells and outer clear polygonal tumor cells with cytoplasm containing glycogen granules. Both cell populations gradually changed from low-grade morphologic features to highly anaplastic ones. Immunohistochemically, the eosinophilic tumor cells were positive for cytokeratin 8, a useful marker for luminal epithelial cells. In contrast, the clear tumor cells expressed several myoepithelial markers, including α-smooth muscle actin, p63, and cytokeratin 14. Based on these histologic and immunohistochemical characteristics, we diagnosed this apocrine sweat gland tumor as a carcinoma-and-malignant myoepithelioma with high-grade transformation of both luminal and myoepithelial cells. Our case may be a helpful reference for the histogenesis of carcinoma-and-malignant myoepithelioma, in which both the luminal epithelial and myoepithelial components are malignant.


Asunto(s)
Neoplasias Óseas , Carcinoma , Enfermedades de los Perros , Mioepitelioma , Neoplasias de las Glándulas Sudoríparas , Animales , Perros , Masculino , Biomarcadores de Tumor , Neoplasias Óseas/patología , Neoplasias Óseas/veterinaria , Carcinoma/veterinaria , Carcinoma/patología , Enfermedades de los Perros/diagnóstico , Enfermedades de los Perros/patología , Células Epiteliales/patología , Epitelio/patología , Mioepitelioma/veterinaria , Mioepitelioma/química , Mioepitelioma/diagnóstico , Neoplasias de las Glándulas Sudoríparas/veterinaria , Neoplasias de las Glándulas Sudoríparas/patología
18.
Hand Surg Rehabil ; 42(6): 549-552, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-37714516

RESUMEN

Cutaneous myoepithelioma is a rare neoplasm of the skin that has become more widely recognized in recent years despite significant diagnostic pitfalls. It is a benign neoplasm with a high recurrence rate if not excised radically, and must be distinguished from its malignant counterpart. Few cases have been described so far and, to our knowledge, no cases in the finger of a child exist in the literature. We report the case of a 15 year-old boy affected by a rare form of locally aggressive spindle-cell myoepithelioma, and suggest a new multidisciplinary approach combining surgical excision and custom brachytherapy.


Asunto(s)
Mioepitelioma , Neoplasias Cutáneas , Masculino , Niño , Humanos , Adolescente , Mioepitelioma/cirugía , Mioepitelioma/diagnóstico , Mioepitelioma/patología , Neoplasias Cutáneas/cirugía , Neoplasias Cutáneas/patología , Dedos , Extremidad Superior/patología
19.
Int J Oral Sci ; 15(1): 38, 2023 09 07.
Artículo en Inglés | MEDLINE | ID: mdl-37679344

RESUMEN

Pleomorphic adenoma (PA) is the most common benign tumour in the salivary gland and has high morphological complexity. However, the origin and intratumoral heterogeneity of PA are largely unknown. Here, we constructed a comprehensive atlas of PA at single-cell resolution and showed that PA exhibited five tumour subpopulations, three recapitulating the epithelial states of the normal parotid gland, and two PA-specific epithelial cell (PASE) populations unique to tumours. Then, six subgroups of PASE cells were identified, which varied in epithelium, bone, immune, metabolism, stemness and cell cycle signatures. Moreover, we revealed that CD36+ myoepithelial cells were the tumour-initiating cells (TICs) in PA, and were dominated by the PI3K-AKT pathway. Targeting the PI3K-AKT pathway significantly inhibited CD36+ myoepithelial cell-derived tumour spheres and the growth of PA organoids. Our results provide new insights into the diversity and origin of PA, offering an important clinical implication for targeting the PI3K-AKT signalling pathway in PA treatment.


Asunto(s)
Adenoma Pleomórfico , Mioepitelioma , Humanos , Adenoma Pleomórfico/genética , Fosfatidilinositol 3-Quinasas , Proteínas Proto-Oncogénicas c-akt , Transcriptoma
20.
Virchows Arch ; 483(5): 665-675, 2023 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-37548750

RESUMEN

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.


Asunto(s)
Mioepitelioma , Neoplasias Glandulares y Epiteliales , Neoplasias Cutáneas , Humanos , Masculino , Femenino , Niño , Adolescente , Adulto Joven , Adulto , Persona de Mediana Edad , Anciano , Mioepitelioma/patología , Biomarcadores de Tumor/análisis , Neoplasias Cutáneas/patología , Queratinas
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