Management of advanced thymoma presenting with myasthenia gravis in a resource-limited setting: A case report

@#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.

Timectomía transesternal extendida en pacientes con miastenia grave / Extended Transsternal Thymectomy in Patients with Myasthenia Gravis

Introducción: La miastenia grave adquirida es un trastorno de la transmisión neuromuscular, causado por la unión de autoanticuerpos con los componentes de la placa neuromuscular, más frecuentemente con el receptor de acetilcolina. Objetivo: Evaluar los resultados quirúrgicos inmediatos, obtenidos con la timectomía extendida en pacientes con miastenia grave timomatosa y no timomatosa. Métodos: Se realizó un estudio observacional, prospectivo y descriptivo para evaluar los resultados postoperatorios inmediatos de 21 pacientes intervenidos por miastenia grave entre junio de 2015 y mayo de 2020. Las variables estudiadas fueron edad, sexo, lesiones tímicas asociadas y resultados inmediatos: complicaciones y mortalidad ocurridas hasta 30 días de la intervención. Los datos fueron obtenidos de una base en Microsoft Access. Resultados: Dieciséis (76,2 por ciento) pertenecían al sexo femenino y 5 (23, 8 por ciento) al masculino. El mayor número correspondió a la MG con timoma, seguido por la MG e hiperplasia tímica (8) y uno con MG y restos tímicos. Se complicaron tres pacientes (14,3 por ciento). Uno presentó dos complicaciones: enfisema subcutáneo y septicemia, el siguiente, fiebre sin causa aparente y, el último, infección superficial de la herida. No hubo fallecidos. Conclusiones: La timectomía transesternal extendida permite extirpar el timo y la mayor parte de los tejidos adiposo y tímico aberrante, en el cuello y el mediastino. En pacientes tratados por equipos multidisciplinarios, con experiencia en esta cirugía, las complicaciones y la mortalidad tempranas usualmente son favorables(AU)

Introduction: Acquired myasthenia gravis (MG) is a neuromuscular transmission disorder caused by the union of autoantibodies and the components of the neuromuscular plaque, most frequently with the acetylcholine receptor. Objective: To assess the immediate surgical outcomes of extended thymectomy in patients with thymomatous and nonthymomatous myasthenia gravis. Methods: An observational, prospective and descriptive study was carried out to assess the immediate postoperative outcomes of 21 patients operated on for myasthenia gravis between June 2015 and May 2020. The variables studied were age, sex, associated thymic lesions, and immediate outcomes: complications and mortality that occurred up to thirty days after the intervention. The data were obtained from a Microsoft Access database. Results: Sixteen (76.2 percent) belonged to the female sex and five (23.8 percent) to the male sex. The highest number corresponded to MG with thymoma, followed by MG and thymic hyperplasia (8) and one with MG and thymic remains. Three patients (14.3 percent) were complicated. One had two complications: subcutaneous emphysema and septicemia; the next had unexplained fever; and the last one had superficial wound infection. There were no deaths. Conclusions: Extended transsternal thymectomy allows removal of the thymus and most of the adipose and aberrant thymic tissues in the neck and mediastinum. In patients treated by multidisciplinary teams, with experience in this surgery, outcomes regarding early complications and mortality are usually favorable(AU)

Protocolo Clínico e Diretrizes Terapêuticas da miastenia gravis / Clinical Protocol and Therapeutic Guidelines for myasthenia gravis

CONTEXTO: Os PCDT são documentos que visam garantir o melhor cuidado de saúde diante do contexto brasileiro e dos recursos disponíveis no SUS. Podem ser utilizados como materiais educativos aos profissionais de saúde, auxílio administrativo aos gestores, regulamentação da conduta assistencial perante o Poder Judiciário e explicitação de direitos aos usuários do SUS. Os PCDT são os documentos oficiais do SUS que estabelecem critérios para o diagnóstico de uma doença ou agravo à saúde; tratamento preconizado, com os medicamentos e demais produtos apropriados, quando couber; posologias recomendadas; mecanismos de controle clínico; e acompanhamento e verificação dos resultados terapêuticos a serem seguidos pelos gestores do SUS. Os PCDT devem incluir recomendações de condutas, medicamentos ou produtos para as diferentes fases evolutivas da doença ou do agravo à saúde de que se tratam, bem como aqueles indicados em casos de perda de eficácia e de surgimento de intolerância ou reação adversa relevante, provocadas pelo medicamento, produto ou procedimento de primeira escolha. A lei reforçou a análise baseada em evidências científicas para a elaboração dos protocolos, destacando os critérios de eficácia, segurança, efetividade e custo-efetividade para a formulação das recomendações sobre intervenções em saúde. Para a constituição ou alteração dos PCDT, a Portaria GM n° 2.009 de 2012 instituiu na Conitec uma Subcomissão Técnica de Avaliação de PCDT, com as competências de definir os temas para novos protocolos, acompanhar sua elaboração, avaliar as recomendações propostas e as evidências científicas apresentadas, além da revisão periódica dos PCDT vigentes, em até dois anos. A Subcomissão Técnica de Avaliação de PCDT é composta por representantes de Secretarias do Ministério da Saúde interessadas na elaboração de diretrizes clínicas: Secretaria de Atenção Primária à Saúde, Secretaria de Atenção Especializada à Saúde, Secretaria de Vigilância em Saúde, Secretaria Especial de Saúde Indígena e Secretaria de Ciência, Tecnologia, Inovação e Insumos Estratégicos em Saúde. Após concluídas as etapas de definição do tema e escopo do PCDT, de busca, seleção e análise de evidências científicas e consequente definição das recomendações, a aprovação do texto é submetida à apreciação do Plenário da Conitec, com posterior disponibilização deste documento para contribuição de sociedade, por meio de consulta pública (CP) pelo prazo de 20 dias, antes da deliberação final e publicação. A consulta pública é uma importante etapa de revisão externa dos PCDT. O Plenário da Conitec é o fórum responsável pelas recomendações sobre a constituição ou alteração de PCDT, além dos assuntos relativos à incorporação, exclusão ou alteração das tecnologias no âmbito do SUS, bem como sobre a atualização da Relação Nacional de Medicamentos Essenciais (RENAME). É composto por treze membros, um representante de cada Secretaria do Ministério da Saúde ­ sendo o indicado pela Secretaria de Ciência, Tecnologia, Inovação e Insumos Estratégicos em Saúde (SCTIE) o presidente do Plenário ­ e um representante de cada uma das seguintes instituições: ANVISA, Agência Nacional de Saúde Suplementar - ANS, Conselho Nacional de Saúde - CNS, Conselho Nacional de Secretários de Saúde - CONASS, Conselho Nacional de Secretarias Municipais de Saúde - CONASEMS e Conselho Federal de Medicina - CFM. Cabe à Secretaria-Executiva, exercida pelo Departamento de Gestão e Incorporação de Tecnologias e Inovação em Saúde (DGITIS/SCTIE), a gestão e a coordenação das atividades da Conitec. Conforme o Decreto n° 7.646 de 2011, o Secretário de Ciência, Tecnologia, Inovação e Insumos Estratégicos em Saúde deverá submeter o PCDT à manifestação do titular da Secretaria responsável pelo programa ou ação a ele relacionado antes da sua publicação e disponibilização à sociedade. APRESENTAÇÃO: A proposta de atualização do PCDT de Miastenia Gravis é uma demanda que cumpre o Decreto nº 7.508 de 28 de junho de 2011 e as orientações previstas no artigo 26º e o parágrafo único, sobre a responsabilidade do Ministério da Saúde de atualizar os Protocolos Clínicos e Diretrizes Terapêuticas. Este PCDT apresenta a atualização da versão publicada em 2015, com inclusão do exame complementar de diagnóstico dosagem sérica de anticorpos de acetilcolina (anti-AChR). DELIBERAÇÃO INICIAL: Os membros da Conitec presentes na 88ª Reunião do Plenário, realizada nos dias 07, 08 e 09 de julho de 2020, deliberaram para que o tema fosse submetido à consulta pública com recomendação preliminar favorável à publicação deste Protocolo. CONSULTA PÚBLICA: A Consulta Pública nº 27/2020 foi realizada entre os dias 21 de julho a 10 de agosto de 2020. A seguir é apresentado o resumo da análise das contribuições recebidas, ressaltando-se que foram consideradas apenas as encaminhadas no período estipulado e por meio do sítio eletrônico da Conitec. Os dadosforam avaliados quantitativa e qualitativamente, considerando asseguintes etapas: a) leitura de todas as contribuições, b) identificação e categorização das ideias centrais, e c) discussão acerca das contribuições. Foram recebidas ao todo 34 contribuições. A grande maioria dos participantes (n= 33; 97%) classificou a proposta de PCDT como boa ou muito boa na avaliação geral.

VATS Uniportal, masterclass Guatemala / VATS Uniportal, masterclass Guatemala

En las dos últimas décadas la evolución de la cirugía mínimamente invasiva del tórax ha transmutado de un abordaje de tres puertos, siguiendo dos puertos hasta llegar a puerto único, conocido también como VATS Uniportal, procurando un confort mucho mejor para el paciente y resultados quirúrgicos similares. Objetivos. Presentar la técnica quirúrgica de VATS Uniportal en un hospital nacional, efectuadas por un experto internacional en este campo. Pacientes y Métodos. Se presentan dos casos clínicos quirúrgicos: El de una paciente con Miastenia Gravis a quien se le realizó timectomía por abordaje sub-xifoideo y otra paciente, a quien se le completó una lobectomía inferior derecha por hallazgos de patología posterior a la resección de un nódulo pulmonar solitario, reportado como cáncer primario de pulmón. Conclusiones. La técnica de cirugía mínimamente invasiva, VATS Uniportal, ofrece grandes beneficios para el paciente, tanto estéticos como funcionales y su aprendizaje es posible con la transmisión de conocimientos y experiencias directa con la presencia del experto o indirectas a través de la información publicada. (AU)

In the last two decades, the evolution of minimally invasive chest surgery has transmuted from a three-port approach, following two ports until reaching a single port, also known as VATS Uniportal, seeking much better comfort for the patient and similar surgical results. Objective. Present the VATS Uniportal surgical technique in a national hospital, performed by an international expert in this field. Patients and Methods. Two surgical clinical cases are presented: that of a patient with Myasthenia Gravis who underwent thymectomy through the sub-xiphoid approach and another patient, who underwent a right lower lobectomy due to findings of pathology after the resection of a pulmonary nodule. solitary, reported as primary lung cancer. Conclusions. The minimally invasive surgery technique, VATS Uniportal, offers great benefits for the patient, both aesthetic and functional and its learning is possible with the transmission of knowledge and experiences directly with the presence of the expert or indirectly through published information. (AU)

Perioperative challenges and neuromuscular blockade concerns in robotic thymectomy for myasthenia gravis / Desafios perioperatórios e pontos de atenção no bloqueio neuromuscular durante timectomia robótica para miastenia gravis

Abstract Myasthenia Gravis (MG) is an autoimmune disease characterized by weakness and fatigability of skeletal muscles, with improvement following rest. It is a disease of great significance to the anesthesiologist because it affects the neuromuscular junction. Robotic thymectomy has come up in recent times due to the minimally invasive nature and its advantages. This presents a new set of challenges for the anesthesia team, and here we present the various anesthesia considerations and perioperative management in a series of 20 patients who underwent robotic thymectomy. As it is a recent upcoming procedure, there is a paucity of literature on this topic, and most of the available literature talks about One-Lung Ventilation (OLV) and thoracic epidurals. To our notice, this is the first literature without the use of OLV and thoracic epidural for the management of robotic thymectomy.

Resumo Miastenia Gravis (MG) é uma doença autoimune que se caracteriza por fraqueza e fadiga da musculatura esquelética, com melhora após o repouso. É uma doença de grande interesse para o anestesiologista, pois compromete a junção neuromuscular. Recentemente, a timectomia robótica tem sido empregada por apresentar as vantagens da abordagem minimamente invasiva. O procedimento introduz uma série de novos desafios para a equipe de anestesia. Relatamos aqui as várias considerações anestésicas e o cuidado perioperatório em uma série de 20 pacientes submetidos a timectomia robótica. Sendo um procedimento recente, há limitada literatura discutindo esse tópico e, além disso, a maior parte da literatura disponível concentra a atenção na Ventilação Monopulmonar (VMP) e na peridural torácica. A nosso ver, este é a primeiro relato na literatura sem o emprego de VMP e peridural torácica para o manejo da timectomia robótica.

Descripción de las características clínicas y la respuesta a tratamiento en pacientes con miastenia grave sin timoma sometidos a timectomía en una institución de alta complejidad de Cali, Colombia, 2010-2017 / Description of the clinical characteristics and response to treatment in patients with myasthenia gravis without thymoma undergoing thymectomy in a tertiary level care institution in Cali, Colombia, 2010-2017

Introducción. La miastenia grave es una enfermedad autoinmunitaria con una prevalencia mundial de 150 a 250 casos por 1 ́000.000 de habitantes. El tratamiento recomendado para la miastenia grave sin timoma es la timectomía total, la cual es la única alternativa de curación. Métodos. Se llevó a cabo un estudio descriptivo y retrospectivo de una serie de casos de pacientes adultos con miastenia grave sin timoma sometidos a timectomía, durante el periodo de 2010 a 2017. En el análisis estadístico descriptivo, se utilizaron frecuencias absolutas y porcentajes para las variables cualitativas y, para las variables cuantitativas, se utilizaron la mediana y el rango intercuartílico. Resultados. Veintiocho pacientes con miastenia grave sin timoma se sometieron a timectomía desde el año 2010 hasta el 2017. Se categorizaron según la clasificación del estado posterior a la intervención de la Myasthenia Gravis Foundation of America y se evidenció que 4 (14,3 %) pacientes presentaban remisión completa y el grado 3 de manifestaciones clínicas mínimas fue el más frecuente en 19 (67,9 %); 26 (92,9 %) tuvieron mejoría con respecto al cambio del estado clínico, en 2 (7,1 %) no se documentaron cambios y en ningún paciente hubo empeoramiento, exacerbación o muerte secundaria a la enfermedad. Conclusiones. A lo largo de siete años se practicó timectomía a 28 pacientes con diagnóstico de miastenia grave sin timoma, aproximadamente, en el 15 % de los pacientes hubo remisión completa, el grado 3 de manifestaciones mínimas fue el más frecuente y el 93 % presentó mejoría de su estatus clínico.

Miastenia Gravis: reporte de dos casos y revisión de la literatura / Myasthenia Gravis: two case reports and review

Myasthenia gravis is an acquired autoimmune disorder of the neuromuscular junction characterized by fluctuating weakness and fatigability of skeletal muscles. The diagnosis can be established by clinical and serologic testing, with predominance of autoantibodies against the acetylcholine receptor, and Muscle-specific kinase antibodies. We report two cases of Myasthenia gravis, the first one is a 31 year old patient with a debut of the disease, mainly with bulbar symptoms, and the second one is a 29 year old patient diagnosed with generalized Miasthenia Gravis also mainly with bulbar symptoms with worsening of symptomatology. In this report treatments alternatives and management approaches are discused

Tratamiento de la miastenia gravis juvenil / Treatment of juvenile myasthenia gravis

La miastenia gravis juvenil es una enfermedad autoimmune poco frecuente, por lo que ha sido difícil recopilar datos de estudios controlados aleatorizados prospectivos para evaluar la eficacia y los resultados de distintos tratamientos. Si bien hay diferencias entre la miastenia gravis juvenil y la del adulto, se han utilizado los datos aportados por algunas investigaciones en adultos en el tratamiento de la miastenia gravis juvenil. Se evaluarán las distintas opciones terapéuticas, con las distintas evidencias que lo sostienen y se elaborará un algoritmo de tratamiento teniendo siempre presente que cada paciente nos ofrece distintos desafíos.

Juvenile myasthenia gravis is a rare autoimmune disease, which has made it difficult to collect data from prospective randomized controlled trials to evaluate the efficacy and results of different treatments. Although there are differences between the juvenile myasthenia gravis and that of the adult, the data provided by some researches in adults in the treatment of juvenile myasthenia gravis have been used. The different therapeutic options will be evaluated, with the different evidences that sustain it and a treatment algorithm will be elaborated keeping always in mind that each patient offers us different challenges.

A Case of Asymptomatic Multiple Endocrine Neoplasia Type I with Thymic Carcinoid

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.

Carcinoma tímico en un adulto / Thymic carcinoma in an adult

Se presenta el caso clínico de un paciente de 50 años de edad, quien acudió a la consulta de Oncología del Hospital General Docente Dr Juan Bruno Zayas Alfonso de Santiago de Cuba, por presentar un tumor tímico de 4 meses de evolución. Se le realizó timectomía y linfadenectomía mediastinal, así como una biopsia que indicó la presencia de carcinoma tímico de tipo linfoepitelioma (II) de alto grado de malignidad, estadio IVA; por tal razón se decidió efectuar tratamiento con quimioterapia y radioterapia. Debido a la persistencia del tumor se planificó la segunda línea de quimioterapia, pero el paciente falleció.

The case report of a 50 year-old patient is presented who visited the Oncology department of Dr Juan Bruno Zayas Alfonso Teaching General Hospital in Santiago de Cuba, due to a 4 months thymic tumor. A thymectomy and mediastinal lymphadenectomy were carried out , as well as a biopsy which indicated the presence of thymic carcinoma, type II lymphoepithelioma of high degree of malignancy, stage IV A; for such a reason it was decided to carry out a treatment with chemotherapy and radiotherapy. Due to the tumor persistence the second chemotherapy line was planned, but the patient died.

Miastenia gravis: resultados inmediatos y alejados de la timectomía transesternal extendida / Results of extended transsternal thymectomy for Myasthenia gravis: experience with 58 patients

Background: Thymectomy improves clinical outcomes and decreases the need for medical treatment in patients with myasthenia gravis (MG). Aim: To describe the immediate and long-term results of extended transsternal thymectomy (ETT) in patients with MG. Material and Methods: A review of databases, surgical protocols, clinical records and interviews of patients subjected to extended transsternal thymectomy for MG between 1990 and 2016. Perioperative clinical characteristics, anticholinesterase treatment, immediate and remote surgical results were analyzed and patients were followed from one to 10years. Results: We studied 58 patients aged 35 ± 14years (72%) women. In the preoperative period, according to Osserman classification, nine patients (15,5%) were in grade I, eight (13,8%) in grade IIA 8 and 40 (69%) in grade IIB. The pathological study of the surgical piece showed thymic hyperplasia in 39 cases (67,2%). Four patients had postoperative complications but none died. In the Follow-up at 1, 3, 5, 8 and 10years the Masaoka palliation rate was 71.7, 77.5, 67.7, 70.0 and 70,6% respectively. The figures for remission rate were 13.0, 15.0,19.4, 35.0 and 35,3% respectively. The figures for Zielinski positive results were 79.6, 87.5, 87.1, 90.0 and 82,4% respectively. The DeFilippi score improved by 80.4, 87.5, 87.1, 90.0 and 82.4% respectively. The Myasthenia Gravis Foundation of America Post-Intervention State improved by 67.4, 77.5, 77.5, 75.0 and 70,6% respectively. Mean Myasthenia Gravis Activities of daily living (MGADL) and Myasthenia Gravis Quality of life scale 15 (MGQOL 15) were 1.65 and 6.31 respectively. Conclusions: In selected patients with MG, extended transsternal thymectomy in MG has good immediate and long-term results.

Value of Adjuvant Radiotherapy for Thymoma with Myasthenia Gravis after Extended Thymectomy / 中华医学杂志(英文版)

<p><b>Background</b>The co-existence of myasthenia gravis (MG) and thymoma makes the surgical treatment more complicated and adjuvant radiation more controversial. The aim of this study was to investigate adjuvant radiotherapy for thymoma with MG after extended thymectomy.</p><p><b>Methods</b>A total of 181 patients with both MG and thymoma were recruited between 2003 and 2014 at Tongren Hospital, China. Among all the patients, 157 patients received radiation therapy after surgery (Group A); whereas the other 24 patients did not receive radiation therapy (Group B). According to the time that patients started mediastinal radiation therapy, we subdivided the 157 patients in Group A into subgroups (1-month subgroup, n = 98; 2-month subgroup, n = 7; and 3-month subgroup, n = 52). We then compared the effect of the mediastinal radiation therapy across these different groups using the survival rate, the rate of postoperative myasthenic crisis, and the complete stable remission (CSR) rate as the primary endpoints.</p><p><b>Results</b>There was a significant difference in the occurrence of postoperative myasthenic crisis between 1-month subgroup and Group B (χ = 4.631, P = 0.031). The rates of reaching CSR were 32.6% in 1-month subgroup, 25% in 3-month subgroup, and 22.7% in Group B, respectively. The overall survival rates of 1-month subgroup, 3-month subgroup, and Group B were 88.8%, 83.3%, and 77.3%, respectively. Analysis on the Kaplan-Meier survival curves demonstrated that within 8 years after surgery, there was no significant difference in aspects of overall survival and disease-free survival between 1-month subgroup and Group B, and between 3-month subgroup and Group B; over 8 years after surgery, the disease-free survival rates in 1-month subgroup, 3-month subgroup and Group B were 79.4%, 70.6%, and 55.3%, respectively.</p><p><b>Conclusions</b>Adjuvant radiation within 1 month after extended thymectomy may be helpful in controlling postoperative MG, such as decreasing the possibility of postoperative myasthenic crisis, and raising cumulative probabilities of reaching CSR.</p>

Myasthenia Gravis with Thymoma Recurrence 20 Years after Thymectomy: What Is the Optimal Follow-up Duration?

Thymoma occurs in about 20% of patients with myasthenia gravis (MG), but the recurrence of thymoma has rarely been reported. A 68-year-old man presented with ptosis and weakness of the neck muscles. He had undergone thymectomy for ocular MG with invasive thymoma 20 years previously. Chest computed tomography revealed recurrence of the thymoma. This 20-year period is, to our knowledge, the longest reported interval for recurrence of an MG-associated thymoma. The literature on the optimal follow-up duration after thymectomy is reviewed.

Pure Red Cell Aplasia Associated with Good Syndrome

Pure red cell aplasia (PRCA) and hypogammaglobulinemia are paraneoplastic syndromes that are rarer than myasthenia gravis in patients with thymoma. Good syndrome coexisting with PRCA is an extremely rare pathology. We report the case of a 50-year-old man with thymoma and PRCA associated with Good syndrome who achieved complete PRCA remission after thymectomy and postoperative immunosuppressive therapy, and provide a review of the pertinent literature.

Ectopic Cervical Thymoma: A Case Report and Review

In the embryo, the thymus originates from the third and fourth pharyngeal pouches and migrates from the superior neck to the mediastinum. Ectopic cervical thymoma (ECT) is an extremely rare tumor that originates from ectopic tissue, and is caused by the aberrant migration of the embryonic thymus. Our patient was a 30-year-old woman who had a nodular lesion in the neck for several years. Ultrasonography and computed tomography were performed. She underwent surgery, and a histological examination resulted in a diagnosis of type AB thymoma. Herein, we report a case of ECT that was resected through a transcervical approach.

Successful Lung Transplantation in a Patient with Myasthenia Gravis

A 47-year-old man with myasthenia gravis (MG) was admitted for a lung transplant. He had bronchiolitis obliterans after allogeneic hematopoietic stem cell transplantation due to acute myeloid leukemia. MG developed after stem cell transplantation. Bilateral sequential lung transplantations and a total thymectomy were performed. The patient underwent right diaphragmatic plication simultaneously due to preoperatively diagnosed right diaphragmatic paralysis. A tracheostomy was performed and bilevel positive airway pressure (BiPAP) was applied on postoperative days 8 and 9, respectively. The patient was transferred to the general ward on postoperative day 12, successfully weaned off BiPAP on postoperative day 18, and finally discharged on postoperative day 62.

Successful Lung Transplantation in a Patient with Myasthenia Gravis

A 47-year-old man with myasthenia gravis (MG) was admitted for a lung transplant. He had bronchiolitis obliterans after allogeneic hematopoietic stem cell transplantation due to acute myeloid leukemia. MG developed after stem cell transplantation. Bilateral sequential lung transplantations and a total thymectomy were performed. The patient underwent right diaphragmatic plication simultaneously due to preoperatively diagnosed right diaphragmatic paralysis. A tracheostomy was performed and bilevel positive airway pressure (BiPAP) was applied on postoperative days 8 and 9, respectively. The patient was transferred to the general ward on postoperative day 12, successfully weaned off BiPAP on postoperative day 18, and finally discharged on postoperative day 62.

Multiple Thymoma with Myasthenia Gravis

The actual incidence of multiple thymoma is unknown and rarely reported because it remains controversial whether the cases represent a disease of multicentric origin or a disease resulting from intrathymic metastasis. In this case, a patient underwent total thymectomy for multiple thymoma with myasthenia gravis via bilateral video-assisted thoracic surgery. A well-encapsulated multinodular cystic mass, measuring 57 mm×50 mm×22 mm in the right lobe of the thymus, and a well-encapsulated mass, measuring 32 mm×15 mm×14 mm in the left lobe, were found. Both tumors were type B2 thymoma. Few cases of multiple thymoma with myasthenia gravis have ever been reported in the literature. We report a case of synchronous multiple thymoma associated with myasthenia gravis.

Paraneoplastic Pemphigus Associated with a Malignant Thymoma: A Case of Persistent and Refractory Oral Ulcerations Following Thymectomy

Paraneoplastic pemphigus is a rare, life-threatening autoimmune mucocutaneous blistering disease associated with underlying neoplasia, commonly lymphoproliferative tumors. Herein we report a case of paraneoplastic pemphigus with a unique autoantibody profile associated with a malignant thymoma. A 56-year-old female patient presented with relapsing oral ulcerations accompanied by erythematous papules and patches on her extremities for 2 months. Skin and mucosal biopsies identified interface dermatitis with lichenoid lymphocytic infiltration in the upper dermis. Immunoblotting and enzyme-linked immunosorbent assays revealed that the patient had multiple autoantibodies against desmoglein 1, desmocollin 1, 2, 3, laminin gamma-1, envoplakin, and periplakin. The skin lesions completely healed following thymectomy and systemic corticosteroid therapy, but the oral ulcerations persisted through a follow-up period of over 2 years.

Subgroup Classification and Therapeutic Guidance for Myasthenia Gravis / 대한소아신경학회지

Myasthenia gravis (MG) is a chronic autoimmune disease of neuromuscular blockade, characterized by muscle weakness and fatigue, and is associated with the production of autoantibodies against the skeletal muscle acetylcholine receptor, muscle-specific kinase (MuSK), low-density lipoprotein receptor-related protein 4 (LRP4), and other muscle endplate proteins. In addition, MG may be classified according the location of the affected muscles (ocular vs. generalized), patient age at symptom onset, and thymic pathology. Subgroup classification based on serum antibodies and clinical features include early-onset, late-onset, thymoma-associated, MuSK and LRP4 antibody-negative, and ocular forms of MG, and can help with therapeutic decisions and prognosis. Pyridostigmine is the chosen symptomatic treatment. For patients who do not adequately respond to symptomatic therapy, corticosteroids, other immunomodulating agents, and thymectomy are the first-line immunosuppressive treatments. The treatment of MG is highly individualized and depends on the age of the patient, the type and severity of the disease, and the pace of progression.