RESUMO
@#Thymomas are rare tumours which generally account for only 0.2 – 1.5% of mediastinal tumours in adults. Around 40% of patients present with systemic symptoms such as motor weakness due to myasthenia gravis (MG), pure red cell aplasia, and hypogammaglobulinemia. Based on recent guidelines, management of advanced thymoma uses a multimodal approach, which is thymectomy followed by radiotherapy, but not all health care centers have radiotherapy facilities. A 52-year-old woman presented with nasal voice and had difficulty swallowing food. Patient was diagnosed with myasthenia gravis (MG). CT scan with contrast of the thorax showed a heterogenous solid mass in anterior mediastinum. Histopathological examination showed thymoma type B2. Thymectomy followed by seven cycles of platinum-based chemotherapy were done on the patient. Evaluation afterward showed complete remission of thymoma. The patient’s motor weakness improved after the chemotherapy. Post-chemotherapy period was uneventful at six months on follow-up visit. The dosage of acetylcholinesterase inhibitor drug is reduced periodically due to improvement in motor weakness. The case emphasizes how to manage an advanced thymoma with MG with limited therapeutic options, and the importance of multidisciplinary management involving oncologists, surgeons, and neurologists.
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Timoma , Miastenia Gravis , Tratamento Farmacológico , TimectomiaRESUMO
Objective: To investigate the clinicopathological features, and molecular genetic alterations of metaplastic thymoma (MT). Methods: A total of ten MT cases, diagnosed from 2011 to 2021, were selected from the Department of Pathology of Jinling Hospital, Nanjing University Medical School, Nanjing, China for clinicopathological and immunohistochemical (IHC) examination and clinical follow-up. Fluorescence in situ hybridization (FISH), next-generation sequencing (NGS), and YAP1 C-terminus (YAP1-CT) IHC were performed to detect YAP1::MAML2 fusions. Results: There were four males and six females, ranging in age from 29 to 60 years (mean 50 years, median 54 years). Microscopically, all tumors showed a typical biphasic morphology consisting of epithelial components and gradually or abruptly transitioning spindle cell components. The two components were present in varying proportions in different cases. Immunophenotypically, the epithelial cells were diffusely positive for CKpan, CK5/6 and p63. The spindle cells were diffusely positive for vimentin and focally positive for EMA. TdT was negative in the background lymphocytes. Ki-67 proliferation index was less than 5%. YAP1 and MAML2 break-apart FISH analyses showed that all ten cases had narrow split signals with a distance of nearly 2 signal diameters and may be considered false-negative. Using YAP1::MAML2 fusion FISH assays, abnormal fusion signals were observed in all the ten cases. NGS demonstrated YAP1::MAML2 fusions in all eight cases with adequate nucleic acids; in two cases the fusions were detected by DNA sequencing and in eight cases by RNA sequencing. All ten cases of MT demonstrated loss of YAP1 C-terminal expression in epithelioid cells. Conclusions: MT is a rare and low-grade thymic tumor characterized by a biphasic pattern and YAP1::MAML2 fusions. Break-apart FISH assays may sometimes show false-negative results due to the proximity of YAP1 and MAML2, while YAP1 C-terminal IHC is a highly sensitive and specific marker for MT. Loss of YAP1 C-terminal expression can also be used to screen YAP1::MAML2 fusions for possible MT cases.
Assuntos
Masculino , Feminino , Humanos , Adulto , Pessoa de Meia-Idade , Timoma/genética , Hibridização in Situ Fluorescente , Fatores de Transcrição/genética , Mutação , Neoplasias do Timo/genéticaRESUMO
BACKGROUND@#Thymic carcinomas (TCs) and thymic neuroendocrine neoplasms (TNENs) are two aggressive subtypes of thymic malignancy. Traditional therapy for advanced TCs and TNENs has limited outcome. New genomic profiling of TCs and TNENs might provide insights that contribute to the development of new treatment approaches.@*METHODS@#We used gene panel sequencing technologies to investigate the genetic aberrations of 32 TC patients and 15 TNEN patients who underwent surgery at Shanghai Chest Hospital between 2015 and 2017. Patient samples were sequenced using a 324-gene platform with licensed technologies. In this study, we focused on clinically relevant genomic alterations (CRGAs), which are previously proven to be pathogenic alterations, to identify the pathology-specific mutational patterns, prognostic signatures of TCs and TNENs.@*RESULTS@#The mutational profiles between TCs and TNENs were diverse. The genetic alterations that ranked highest in TCs were in CDKN2A, TP53, ASXL1, CDKN2B, PIK3C2G, PTCH1, and ROS1 , while those in TNENs were in MEN1, MLL2, APC, RB1 , and TSC2 . Prognostic analysis showed that mutations of ROS1, CDKN2A, CDKN2B, BRAF, and BAP1 were significantly associated with worse outcomes in TC patients, and that mutation of ERBB2 indicated shortened disease-free survival (DFS) and overall survival (OS) in TNEN patients. Further investigation found that the prognosis-related genes were focused on signal pathways of cell cycle control, chromatin remodeling/DNA methylation, phosphoinositide 3-kinases (PI3K)/protein kinase B (AKT)/mammalian target of rapamycin (mTOR), and receptor tyrosine kinase (RTK)/RAS/mitogen-activated protein kinase (MAPK) signaling.@*CONCLUSION@#We profiled the mutational features of 47 Chinese patients with thymic malignancy of diverse pathologic phenotypes to uncover the integrated genomic landscape of these rare tumors, and identified the pathology-specific mutational patterns, prognostic signatures, and potential therapeutic targets for TCs and TNENs.
Assuntos
Humanos , Timoma , Proteínas Tirosina Quinases/genética , Proteínas Proto-Oncogênicas/genética , China , Neoplasias do Timo/patologia , Prognóstico , Tumores Neuroendócrinos/patologia , Mutação/genéticaRESUMO
Chronic diarrhea is a frequent cause of consultation in daily clinical practice. There are multiple diagnostic algorithms that allow a staggered approach to the most frequent pathologies, leaving out some lesser-known ones. This article reports the case of a 66-year-old female patient with a history of arterial hypertension, dyslipidemia and resected AB thymoma and a history of chronic diarrhea of 8 weeks of evolution. The etiological study ruled out infectious causes, celiac disease and negative viral serology. Due to a history of thymoma, immunoglobulin count was performed, showing severe pan-hypogammaglobulinemia. Good's Syndrome is the combination of thymoma and hypogammaglobulinemia, where patients may present with diarrhea secondary to immunodeficiency. Hypogammaglobulinemia associated with the presence of a thymoma is a rare cause but widely described in the literature as Good's Syndrome. Therefore, it seems relevant to describe a case, its approach and subsequent management.
La diarrea crónica constituye una causa frecuente de consulta en la práctica clínica diaria. Existen múltiples algoritmos diagnósticos que permiten realizar un abordaje escalonado de las patologías más frecuentes y permiten descartar algunas menos conocidas. En el presente artículo se reporta el caso de una paciente de género femenino de 66 años, antecedentes de hipertensión arterial, dislipidemia y timoma AB resecado con historia de diarrea crónica de 8 semanas de evolución. Dentro del estudio etiológico se descartan las causas infecciosas, enfermedad celíaca y serologías virales negativas. Por antecedente de timoma, se realizó recuento de inmunoglobulinas, evidenciando una severa pan-hipogammaglobulinemia. El Síndrome de Good es la combinación de timoma e hipogammaglobulinemia, donde los pacientes podrían presentar diarreas secundarias a inmunodeficiencia. La hipogammaglobulinemia asociada a la presencia de un timoma es una causa poco frecuente pero ampliamente descrita en la literatura como Síndrome de Good. Por lo antes señalado, nos parece relevante describir un caso, su abordaje y manejo posterior.
Assuntos
Humanos , Feminino , Idoso , Timoma/complicações , Neoplasias do Timo/complicações , Diarreia/etiologia , Doença Crônica , Hospedeiro ImunocomprometidoRESUMO
BACKGROUND@#In recent years, with the advancement of minimally invasive techniques, thoracoscopic thymoma resection has experienced a development process from three-port video-assisted thoracic surgery (VATS) to two-port (TP) and single-port (SP) variants. However, the feasibility and safety of SP-VATS have not been generally recognized. This study intends to explore the safety and feasibility of SP-VATS in thymoma resection, in order to provide a reference for clinical surgicalselection.@*METHODS@#The clinical data of 197 patients who underwent thoracoscopic thymoma resection in Beijing Tongren Hospital from January 2018 to September 2021 were retrospectively analyzed. The patients were divided into SP-VATS group (n=42) and TP-VATS group (n=155). After matching propensity scores, there is no statistically significant difference in preoperative baseline data between SP-VATS group and TP-VATS group. Among them, there were 17 males and 25 females with an average age of 28-72 (48.00±9.43) years in the SP-VATS group, and 20 males and 22 females with an average age of 30-75 (50.38±9.83) years in TP-VATS group. The clinical effects of the two groups were compared.@*RESULTS@#The operation was successfully completed in both groups, and there was no conversion to thoracotomy or increased surgical incisions. Compared with the TP-VATS group, the chest drainage time and hospital stay in the SP-VATS group were shorter [(2.95±0.76) d vs (3.33±0.85) d; (4.57±0.83) d vs (5.07±1.13) d], and the visual pain score at 24 h and 72 h after surgery were lower [(3.64±0.85) points vs (4.05±0.66) points; (2.33±0.75) points vs (3.07±0.68) points] (P<0.05). There was no statistically significant difference between the two groups in operation time [(130.00±26.23) min vs (135.24±27.03) min], intraoperative blood loss [(69.52±22.73) mL vs (82.38±49.23) mL] (P>0.05).@*CONCLUSIONS@#SP-VATS in thymoma is a safe, feasible, and less invasive procedure, with less postoperative pain and faster recovery than multi-port VATS.
Assuntos
Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Pulmonares/cirurgia , Estudos Retrospectivos , Cirurgia Torácica Vídeoassistida/métodos , Timoma/cirurgia , Neoplasias do Timo/cirurgiaRESUMO
Resumen La enfermedad COVID-19, causada por el virus SARS-CoV-2, surgió a fines de 2019 en Wuhan, China. La evolución clínica es variable, así como la tasa de mortalidad, que es mayor en pacientes mayores de 65 años y en quienes padecen enfermedades subyacentes. Las inmunodeficiencias son potenciales factores de riesgo para formas graves de COVID-19. Los pacientes con inmunodeficiencias tienen además mayor frecuencia de complicaciones no infecciosas, que podrían representar un riesgo adicional. Hasta el momento existen escasas publicaciones sobre asociación COVID-19 e inmunodeficiencias humorales. Considerando la importancia del estudio de esta nueva enfermedad viral y de su potencial repercusión en la salud de los pacien tes con inmunodeficiencias presentamos seis casos de COVID-19 en adultos con déficit de anticuerpos (tres mujeres y tres varones, edad promedio 48.5 años, rango 20-67). Cuatro tenían inmunodeficiencias primarias: inmunodeficiencia común variable (n: 3) y agammaglobulinemia ligada al cromosoma X (n: 1). Los otro dos tenían hipogammaglobulinemia secundaria, en un caso asociada a timoma (síndrome de Good), y en el otro a tratamiento con rituximab. La evolución fue favorable en todos menos en el paciente con síndrome de Good, quien presentaba un marcado deterioro del estado general antes de contraer COVID-19.
Abstract COVID-19, caused by SARS-CoV-2, emerged in late 2019 in Wuhan, China. Its clinical course is variable, as well as the mortality rate, which is higher among people over 65 years of age and persons with underlying conditions. Immunodeficiencies are po tential risk factors for severe forms of COVID-19. Furthermore, patients with immunodeficiencies often undergo non-infectious complications, which could bear additional risk. So far, few reports of patients with COVID-19 and humoral immunodeficiencies have been published. Considering the importance of the study of this new viral disease and its potential health impact on patients with immunodeficiency disorders, we present six cases of COVID-19 in patients with impaired humoral immunity. Three were women and three were men. The average age was 48.5 years (range 20-67). Four had been diagnosed with primary antibody deficiency: three had common variable immunodeficiency and one had X-linked agammaglobulinemia. The other two patients had secondary hypogammaglobulinemia, one was associated with thymoma (Good's syndrome), and the other was associated with rituximab treatment. The evolution was favorable in all except the patient with Good's syndrome, who pre sented a marked decline in clinical status before contracting COVID-19.
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Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Adulto Jovem , Timoma , Neoplasias do Timo , Agamaglobulinemia , Doenças da Imunodeficiência Primária , COVID-19 , SARS-CoV-2RESUMO
A patient with thymoma associated immunodeficiency syndrome (Good's syndrome) and bronchiectasis was retrospectively analyzed. Good's syndrome is a rare condition of immunodeficiency that is characterized by thymoma and hypogammaglobulinemia. It is important to bear in mind that Good's syndrome should be included in the differential diagnosis When patients repeatedly visited for bronchiectasis or infection, we should alert to their immune state and history of thymoma. Early screening of immunological status and aggressive correction of immune deficiency are beneficial to improving the prognosis to patients with Good's syndrome.
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Humanos , Agamaglobulinemia/complicações , Bronquiectasia/complicações , Estudos Retrospectivos , Timoma/complicações , Neoplasias do Timo/complicaçõesRESUMO
Thymic tumor is a relatively rare type of thoracic cancer, which is pathologically-classified as thymic epithelial tumors by World Health Organization (WHO), including thymoma and thymic carcinoma. The incidence of thymic epithelial tumors in China is about 4.09 per million, which is slightly higher than that in European and American countries. For surgically-resectable thymic epithelial tumors, complete resection with or without postoperative radiotherapy or chemotherapy is preferred. For advanced unresectable disease, conventional radiotherapy and chemotherapy is the mainstream treatment but lack of standard first-line and second-line regimens. In recent years, targeted drugs and the immune checkpoint inhibitors have gradually exhibited certain therapeutic prospect in this subset of tumor. Since there was no guideline regarding thymic epithelial tumor released in China, Multidisciplinary Committee on Oncology, Chinese Physicians Association analyze, summarize and discuss the relevant research data for the diagnosis, treatment and prognosis of thymic epithelial tumors, and formulate the "Chinese guidelines for clinical diagnosis and treatment of thymic epithelial tumors (2021 Edition)" for clinicians' reference.
Assuntos
Humanos , China , Estadiamento de Neoplasias , Neoplasias Epiteliais e Glandulares/terapia , Timoma/terapia , Neoplasias do Timo/terapiaRESUMO
Objective To investigate the clinicopathological features,immunohistochemical characteristics,diagnosis,treatment and prognosis of intrathyroid thymic carcinoma.Methods Clinical data of 7 patients with intrathyroid thymic carcinoma were retrospectively reviewed.Histological examination and immunohistochemical staining were performed on the surgically resected tumors.The infection of Epstein-Barr virus(EBV)was detected by EBER in situ hybridization.Results The 7 patients included 5 males and 2 females.The age ranged from 40 to 71 years,with a median of 54 years.The tumors were located in the thyroid gland,with the maximum diameter ranging from 2.2 cm to 6.0 cm and the average maximum diameter of(4.0±1.2)cm.All the patients underwent thyroid gland resection and local lymph node dissection.After operation,all the cases were treated with radiotherapy and five of them additionally received chemotherapy.Six patients were followed up for 10-163 months,all of whom were still alive,including 2 patients with recurrence in situ,1 patient with homolateral cervical lymph node metastasis and the rest with no recurrence or metastasis.CK-pan,P63,CD5 and CD117 were expressed in all the cases,while TTF-1,TG,CT and PAX8 were negative.One case of them expressed SYN and CgA.Ki-67 proliferation index ranged from 10% to 90%.EBER in situ hybridization showed negative results in all 7 cases.Conclusions Intrathyroid thymic carcinoma is a relatively low-grade malignant tumor.The combination of immunohistochemical CD5,CD117 and monoclonal PAX8 is helpful in the diagnosis and differential diagnosis of intrathyroid thymic carcinoma.EBV may not be involved in the development of intrathyroid thymic carcinoma.Thyroid gland resection plus central lymph node dissection is an important treatment measure for intrathyroid thymic carcinoma.For patients with regional lymph node metastasis and obvious peripheral tissue invasion,postoperative radiotherapy with/without chemotherapy can effectively delay the disease progression.
Assuntos
Pré-Escolar , Feminino , Humanos , Masculino , Infecções por Vírus Epstein-Barr , Herpesvirus Humano 4 , Recidiva Local de Neoplasia , Estudos Retrospectivos , Timoma , Neoplasias do Timo/terapiaRESUMO
Thymomas are a heterogeneous group of tumors arising from the epithelium of the thymus. They are categorized by the proportion of neoplastic epithelia to lymphocytes and by the degree of cytologic atypia. Thymomas constitute 0.2-1.5% of all malignancies and nearly all occur in patients over 20 years. We reviewed the available literature and found less than 50 cases of thymoma reported in children (<18 years of age), the youngest being 4 years old, and no cases in newborns. They represent less than 1% of all mediastinal tumors in children. Due to the limited number of cases in the pediatric population, the diagnosis and treatment in this population is extremely challenging. Thymomas in all age groups may be associated with paraneoplastic syndromes, being myasthenia gravis the most common, which is associated with a worse prognosis in the pediatric population. We present the first case of a newborn infant with congenital thymoma. This case demonstrates a rare tumor in an unusual age group and emphasizes the importance of multidisciplinary teamwork in the decision-making and management of this condition.
Assuntos
Humanos , Masculino , Recém-Nascido , Timoma/congênito , Neoplasias do Timo/congênito , Timo , Hiperplasia do Timo , Anormalidades Congênitas/patologia , Miastenia GravisRESUMO
INTRODUCCIÓN: Los tumores de mediastino constituyen una entidad clínica poco frecuente, generalmente asintomática. Se pueden desarrollar desde estructuras que se ubican en el compartimento anterior, medio y posterior, siendo la primera, la ubicación más usual. En los adultos, timomas y linfomas (Hodgkin y no Hodgkin) son las masas más comunes del mediastino anterior, junto al bocio endotorácico y los teratomas; todas en su conjunto son conocidas como las "4 T del Mediastino Anterior". Casos clínicos: Se exponen tres casos clínicos de tumores de mediastino anterior, intervenidos en nuestro centro. Discusión: Las masas mediastínicas constituyen una entidad clínica infrecuente. La tomografía computada de tórax con y sin contraste intravenoso es utilizada para evaluar estas anormalidades descubiertas radiográficamente. El diagnóstico definitivo generalmente requiere una muestra de tejido que se obtiene mediante biopsia antes del tratamiento o como parte de una resección terapéutica planificada de toda la masa. En Chile, existen pocos trabajos recientes que muestren las características clínicas de pacientes que presentan timomas o linfomas; es por esto que se hace importante la presentación de estos casos que constituyen un aporte al conocimiento nacional.
INTRODUCTION: Mediastinal tumours constitute a clinical entity infrequent, generally asymptomatic. It can develop from structures localized on anterior compartment, middle and posterior, where structure localized on anterior compartment is usual. Adults tend to have thymomas and lymphomas more common in anterior mediastinal within intrathoracic goiter and teratomas. They are known as a "4T of anterior mediastinal mass". Clinical cases: Three clinical cases of anterior mediastinal tumours are exposed in our centre. Discussion: Mediastinal mass constitutes a clinical entity infrequent. The computed Tomography of the thorax with intravenous contrast and without intravenous contrast are used to evaluate abnormalities discover by radiography. The final diagnosis requires a fabric sample which is obtained by a biopsy before the treatment or as a part of a planned therapeutic recession. In Chile exist a few recently studies that shows clinical characteristics of patients which presents thymus and lymphomas as a result is important to present this kind of cases due to these cases constitute to the national knowledge.
Assuntos
Humanos , Masculino , Adulto , Pessoa de Meia-Idade , Timoma/diagnóstico por imagem , Linfoma/diagnóstico por imagem , Neoplasias do Mediastino/diagnóstico por imagem , Doença de Hodgkin , Toracotomia , Tomografia Computadorizada por Raios X , Hospitais Públicos , Linfoma/terapia , Neoplasias do Mediastino/cirurgiaRESUMO
RESUMO: Relatamos o caso de um paciente do sexo feminino, 76 anos, com tosse há seis meses. Os exames laboratoriais estavam normais. Na radiografia do tórax observou-se alargamento do mediastino no terço médio do tórax. Posteriormente, foram realizadas tomografia computadorizada e ressonância magnética do tórax que caracterizaram uma massa localizada no mediastino anterior, envolvendo a aorta ascendente e comprimindo a veia cava superior. O diagnóstico histológico pós-biópsia e ressecção parcial da lesão foi de um carcinoma tímico de células escamosas. (AU)
ABSTRACT: We report the case of a female patient, 76 years old, with a cough for six months. Laboratory tests were normal. Chest X-ray revealed enlargement of the mediastinum in the middle third of the thorax. Later, computed tomography and magnetic resonance imaging of the thorax were performed, characterizing a mass located in the anterior mediastinum, involving the ascending aorta and compressing the superior vena cava. Histological diagnosis after biopsy and partial resection of the lesion was a thymic squamous cell carcinoma. (AU)
Assuntos
Humanos , Feminino , Idoso , Timoma/diagnóstico por imagem , Biópsia , Imageamento por Ressonância Magnética , Neoplasias do MediastinoRESUMO
To investigate the differences in energy spectrum CT findings between anterior mediastinal lymphoma and thymic carcinoma. Twenty-two cases of anterior mediastinal lymphoma and 28 cases of thymic carcinoma confirmed by biopsy in Tangshan People's Hospital were selected.The CT values and changes of iodine content and water content in lesion sites were measured by energy spectrum analysis software.The differences between anterior mediastinal lymphoma and thymic carcinoma were compared. The single-energy CT value of 40-80 keV in thymus carcinoma was higher than that in anterior mediastinal lymphoma(=0.001,=0.037,=0.042,=0.034,=0.002;=0.016,=0.013,=0.018,=0.024,=0.012).The difference in the single-energy CT value of 90-110 keV between anterior mediastinal lymphoma and thymic carcinoma showed no statistical significance(all >0.05).The concentrations of water in the arterial and venous stages of thymic carcinoma were significantly lower than those in the anterior mediastinal lymphoma(=0.030,=0.037),whereas the iodine concentrations were significantly higher(=0.026,=0.000). Anterior mediastinal lymphoma and thymic carcinoma have remarkably different 40-80 keV single energy CT value and iodine concentration in arterial and venous phases,which may be helpful for the differential diagnosis of these two malignancies.
Assuntos
Humanos , Linfoma , Diagnóstico por Imagem , Neoplasias do Mediastino , Diagnóstico por Imagem , Timoma , Diagnóstico por Imagem , Neoplasias do Timo , Diagnóstico por Imagem , Tomografia Computadorizada por Raios XRESUMO
ABSTRACT Objective: To evaluate the results of resection of tumors of the thymus by robotic thoracic surgery, analyzing the extent of resection, postoperative complications, time of surgery, and length of stay. Methods: Retrospective study from a database involving patients diagnosed with a tumor of the thymus and undergoing robotic thoracic surgery at one of seven hospitals in Brazil between October of 2015 and June of 2018. Results: During the study period, there were 18 cases of resection of tumors of the thymus: thymoma, in 12; carcinoma, in 2; and carcinoid tumor, in 1; high-grade sarcoma, in 1; teratoma, in 1; and thymolipoma, in 1. The mean lesion size was 60.1 ± 32.0 mm. Tumors of the thymus were resected with tumor-free margins in 17 cases. The median (interquartile range) for pleural drain time and hospital stay, in days, was 1 (1-3) and 2 (2-4), respectively. There was no need for surgical conversion, and there were no major complications. Conclusions: Robotic thoracic surgery for resection of tumors of the thymus has been shown to be feasible and safe, with a low risk of complications and with postoperative outcomes comparable to those of other techniques.
RESUMO Objetivo: Avaliar os resultados da ressecção de tumores tímicos por cirurgia torácica robótica, verificando a radicalidade da ressecção, complicações pós-operatórias, tempo de cirurgia e tempo de internação. Métodos: Estudo retrospectivo a partir de um banco de dados envolvendo pacientes com diagnóstico de tumor tímico e submetidos à cirurgia torácica robótica em sete hospitais no Brasil entre outubro de 2015 e junho de 2018. Resultados: Durante o período estudado, houve 18 casos de ressecção de tumores tímicos (timomas, em 12; carcinoma tímico, em 2; e tumor carcinoide tímico, sarcoma tímico de alto grau, teratoma tímico e timolipoma, em 1 cada). A média do tamanho das lesões foi de 60,1 ± 32,0 mm. Tumores tímicos foram ressecados com margens livres em 17 casos. As medianas (intervalos interquartis) de tempo de dreno pleural e de internação, em dias, foram 1 (1-3) e 2 (2-4), respectivamente. Não houve necessidade de conversão cirúrgica nem complicações maiores. Conclusões: A cirurgia torácica robótica para a ressecção de tumores tímicos demonstrou ser factível e segura, com baixo risco de complicações e desfechos pós-operatórios comparáveis aos de outras técnicas.
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Timoma/cirurgia , Neoplasias do Timo/cirurgia , Procedimentos Cirúrgicos Torácicos/métodos , Procedimentos Cirúrgicos Robóticos/métodos , Tomografia Computadorizada por Raios X , Estudos Retrospectivos , Resultado do TratamentoRESUMO
RESUMEN Los timomas son las neoplasias mediastínicas más comunes en adultos. Los pacientes con timoma son frecuentemente diagnosticados con síndromes paraneoplásicos y generalmente están asociados con varias condiciones autoinmunes. El síndrome de Sjögren (SS) es un trastorno inflamatorio autoinmune, que se caracteriza por la destrucción mediada por linfocitos de las glándulas exocrinas, que conduce a la ausencia de secreción glandular, en particular las salivales y las lagrimales; la hiposalivación puede variar desde una ligera reducción en el flujo de saliva con molestias transitorias, a un grave deterioro de la salud oral y malestar psicológico. El penfigoide de membranas mucosas (PMM) es una enfermedad autoinmune crónica, muestra predominante afectación de la mucosa, afectando con mayor frecuencia la cavidad oral, seguida de la conjuntiva, la cavidad nasal y el esófago; intraoralmente incluye gingivitis descamativa, vesículas, erosiones cubiertas por pseudomembranas y úlceras. El objetivo de este trabajo es presentar el primer caso de una paciente con antecedente de timoma, la cual, conjuntamente presentó SS y PMM; además evidenciar la importancia del manejo multidiciplinario para un diagnóstico preciso y temprano, ya que así, aumenta la eficiencia y la eficacia de la estrategia de tratamiento.
ABSTRACT Thymomas are the most common mediastinal neoplasms in adults. Patients with thymoma are frequently diagnosed with paraneoplastic syndromes and are generally associated with several autoimmune conditions. Sjögren's syndrome (SS) is an autoimmune inflammatory disorder, characterized by the lymphocyte-mediated destruction of the exocrine glands, which leads to the absence of glandular secretion, particularly salivary and lacrimal; hyposalivation can vary from a slight reduction in the flow of saliva with transient discomfort, to a serious deterioration of oral health and psychological distress. Mucous membrane pemphigoid (MMP) is a chronic, autoimmune disease that shows predominant mucosal involvement, most commonly affecting the oral cavity, followed by the conjunctiva, the nasal cavity and the esophagus; intraorally includes scaly gingivitis, vesicles, erosions covered by pseudomembranes and ulcers. The objective of this work is to present the first case of a patient with a history of thymoma, which, jointly presented SS and MMP; also highlight the importance of multidisciplinary management for an accurate and early diagnosis, as this increases the efficiency and efficacy of the treatment strategy.
Assuntos
Humanos , Feminino , Adulto , Timoma/diagnóstico , Xerostomia , Síndrome de Sjogren/complicações , Penfigoide Mucomembranoso Benigno/diagnóstico , CandidíaseRESUMO
Klinefelter syndrome (47, XXY in most cases) is a frequently underdiagnosed chromosomal anomaly associated with multiple comorbidities in adult life. Patients with Klinefelter syndrome have a higher risk of cancer. Specifically, these patients have a higher risk for mediastinal germ cell tumors. It is estimated that 8% of male patients with mediastinal tumors have Klinefelter. We report a 42-years-old male who suffered recurrent respiratory infections. During the study, a mediastinal mass was found, whose pathological study disclosed a type B thymoma. The patient had a history of infertility, high stature, gynecomastia, obesity with gynecoid distribution of body fat and testicular atrophy. A karyotype was requested (47, XXY), confirming the diagnosis of Klinefelter syndrome.
Assuntos
Humanos , Masculino , Adulto , Timoma/patologia , Neoplasias do Timo/patologia , Síndrome de Klinefelter/patologia , Timoma/diagnóstico por imagem , Neoplasias do Timo/diagnóstico , Radiografia Torácica , Tomografia Computadorizada por Raios X , Síndrome de Klinefelter/diagnóstico , Síndrome de Klinefelter/genética , Neoplasias do Mediastino/diagnóstico , Neoplasias do Mediastino/patologiaRESUMO
PURPOSE: To explore the role of salvage radiotherapy (RT) for recurrent thymoma as an alternative to surgery. MATERIALS AND METHODS: Between 2007 and 2015, 47 patients who received salvage RT for recurrent thymoma at Yonsei Cancer Center were included in this study. Recurrent sites included initial tumor bed (n = 4), pleura (n = 19), lung parenchyma (n = 10), distant (n = 9), and multiple regions (n = 5). Three-dimensional conformal and intensity-modulated RT were used in 29 and 18 patients, respectively. Median prescribed dose to gross tumor was 52 Gy (range, 30 to 70 Gy), with equivalent doses in 2-Gy fractions (EQD₂). We investigated overall survival (OS), progression-free survival (PFS), and patterns of failure. Local failure after salvage RT was defined as recurrence at the target volume receiving >50% of the prescription dose. RESULTS: Median follow-up time was 83 months (range, 8 to 299 months). Five-year OS and PFS were 70% and 22%, respectively. The overall response rate was 97.9%; complete response, 34%; partial response, 44.7%; and stable disease, 19.1%. In multivariate analysis, histologic type and salvage RT dose (≥52 Gy, EQD₂) were significantly associated with OS. The high dose group (≥52 Gy, EQD₂) had significantly better outcomes than the low dose group (5-year OS: 80% vs. 59%, p = 0.046; 5-year PFS: 30% vs. 14%, p=0.002). Treatment failure occurred in 34 patients; out-of-field failure was dominant (intra-thoracic recurrence 35.3%; extrathoracic recurrence 11.8%), while local failure rate was 5.8%. CONCLUSION: Salvage RT for recurrent thymoma using high doses and advanced precision techniques produced favorable outcomes, providing evidence that recurrent thymoma is radiosensitive.
Assuntos
Humanos , Intervalo Livre de Doença , Seguimentos , Pulmão , Análise Multivariada , Pleura , Prescrições , Radioterapia , Recidiva , Timoma , Falha de TratamentoRESUMO
Multiple endocrine neoplasia type 1 (MEN 1) is an autosomal dominant hereditary disorder caused by germline mutation of the MEN1 gene. It is characterized by tumors of the anterior pituitary gland, parathyroid glands, and endocrine pancreas. Thymic carcinoid tumor is uncommon and associated with a high mortality, but its natural history has not been investigated yet. We report a case of asymptomatic MEN 1 with a thymic carcinoid tumor. A 37-year-old man underwent a routine medical checkup and upper gastrointestinal endoscopy revealed a duodenal neuroendocrine tumor (NET). Further studies showed the coexistence of pancreatic tumor, parathyroid hyperplasia, pituitary adenoma, and thymoma. The patient underwent duodenal endoscopic mucosal resection, distal pancreatectomy, subtotal parathyroidectomy, and thymectomy. The pathological test revealed a duodenal NET, pancreatic NET, parathyroid hyperplasia, and thymic carcinoid tumor. He was treated for MEN 1. We report this asymptomatic case of MEN 1 with a literature review.