Myasthenia gravis is an autoimmune disease that is characterised by muscle weakness and fatigue, is B-cell mediated, and is associated with antibodies directed against the acetylcholine receptor, muscle-specific kinase (MUSK), lipoprotein-related protein 4 (LRP4), or agrin in the postsynaptic membrane at the neuromuscular junction. Patients with myasthenia gravis should be classified into subgroups to help with therapeutic decisions and prognosis. Subgroups based on serum antibodies and clinical features include early-onset, late-onset, thymoma, MUSK, LRP4, antibody-negative, and ocular forms of myasthenia gravis. Agrin-associated myasthenia gravis might emerge as a new entity. The prognosis is good with optimum symptomatic, immunosuppressive, and supportive treatment. Pyridostigmine is the preferred symptomatic treatment, and for patients who do not adequately respond to symptomatic therapy, corticosteroids, azathioprine, and thymectomy are first-line immunosuppressive treatments. Additional immunomodulatory drugs are emerging, but therapeutic decisions are hampered by the scarcity of controlled studies. Long-term drug treatment is essential for most patients and must be tailored to the particular form of myasthenia gravis.

https://thelancet.com/pdfs/journals/laneur/PIIS1474-4422(15)00145-3.pdf

Myasthenia gravis: subgroup classification and therapeutic strategies.

Myasthenia gravis (MG) is an autoimmune disorder caused by autoantibodies that target the neuromuscular junction, leading to muscle weakness and fatigability. Currently available treatments for the disease include symptomatic pharmacological treatment, immunomodulatory drugs, plasma exchange, thymectomy and supportive therapies. Different autoantibody patterns and clinical manifestations characterize different subgroups of the disease: early-onset MG, late-onset MG, thymoma MG, muscle-specific kinase MG, low-density lipoprotein receptor-related protein 4 MG, seronegative MG, and ocular MG. These subtypes differ in terms of clinical characteristics, disease pathogenesis, prognosis and response to therapies. Patients would, therefore, benefit from treatment that is tailored to their disease subgroup, as well as other possible disease biomarkers, such as antibodies against cytoplasmic muscle proteins. Here, we discuss the different MG subtypes, the sensitivity and specificity of the various antibodies involved in MG for distinguishing between these subtypes, and the value of antibody assays in guiding optimal therapy. An understanding of these elements should be useful in determining how to adapt existing therapies to the requirements of each patient.

Myasthenia gravis — autoantibody characteristics and their implications for therapy

Thymomas (Ts) and thymic carcinomas (TCs) are rare tumours of the mediastinum with an incidence rate of 1.7/million per year in Europe. Histological classification is based on rate of non-malignant-appearing thymic epithelial cells and proportions of lymphocytes (A, AB, B1, B2, B3, and C), while staging system concerns localisation of the involved areas. Surgery is the mainstay of treatment with a 10-year survival of 80%, 78%, 75%, and 42% for stages I, II, III and IV, respectively, with an R0 resection. Radiotherapy has a role in selected cases (stage III patients or R1-2 residual) and platinum-based chemotherapy remains the standard of care for patients with advanced disease. A multimodality approach would be advisable when surgery is not recommended. Since molecular aberrations are poorly understood and few responses are reported, targeted therapies are yet being studied. In this review, we describe key aspects of clinical management for Ts and TCs.

/pdf/thymoma-and-thymic-carcinomas-36180d7uxi.pdf

Thymoma and thymic carcinomas

https://www.jto.org/article/S1556-0864(15)00010-6/pdf

Minimally Invasive versus Open Thymectomy for Thymic Malignancies: Systematic Review and Meta-Analysis

Masses in the anterior mediastinum can be neoplasms (eg, thymomas, thymic carcinomas, or lung metastases) or non-neoplastic conditions (eg, intrathoracic goiter). Thymomas are the most common primary tumor in the anterior mediastinum, although they are rare. Thymic carcinomas are very rare. Thymomas and thymic carcinomas originate in the thymus. Although thymomas can spread locally, they are much less invasive than thymic carcinomas. Patients with thymomas have 5-year survival rates of approximately 78%. However, 5-year survival rates for thymic carcinomas are only approximately 40%. These guidelines outline the evaluation, treatment, and management of these mediastinal tumors.

/pdf/thymomas-and-thymic-carcinomas-34sxd32067.pdf

Thymomas and thymic carcinomas

Surgical techniques for early-stage thymoma: video-assisted thoracoscopic thymectomy versus transsternal thymectomy.

Predictive Factors for Lymph Node Metastasis in Clinical Stage IA Lung Adenocarcinoma

Background: The purpose of this study was to compare perioperative outcomes in patients who underwent videoassisted thoracoscopic surgery or robot-assisted thoracoscopic surgery and assess the feasibility of robotic-assisted thymectomy for the treatment of Masaoka stage I. Methods: We evaluated the short-term outcomes of 46 patients who underwent surgery for Masaoka stage I thymoma without myasthenia gravis between January 2009 and June 2012. Of these patients, 25 received unilateral video-assisted thoracoscopic surgery (VATS group) and the rest 21 recieved unilateral robotic-assisted thoracoscopic surgery (RATS group). We evaluated the duration of surgery, amount of intraoperative blood loss, duration of chest drainage, duration of postoperative hospital stay, hospitalization costs, postoperative complications and oncological outcomes. Results: The duration of surgery was not significantly different between the two groups. Intraoperative blood loss volumes did not differ significantly between the VATS and RATS groups (86.8 mL and 58.6 mL, respectively; P=0.168). The postoperative hospital stay was significantly shorter in the RATS group (3.7 days vs. 6.7 days; P <0.01), and the postoperative pleural drainage volume of the RATS group was significantly less than VATS group (1.1 days vs. 3.6 days; P <0.01). No patients in the RATS group needed conversion to open surgery. However, in the VATS series, one patient had conversion to an open procedure. No surgical complications were observed except that one case had pulmonary atelectasis in the RATS group and one case developed pneumonia after surgery. Use of robot is much more expensive than video. No early recurrence was observed in both groups. Conclusions: Robotic thymectomy is feasible and safe for Masaoka stage I thymoma. RATS is equally minimally invasive as VATS and results in a shorter drainage period and reduced hospital stay compared with the VATS approach.

/pdf/video-assisted-thoracoscopic-surgery-versus-robotic-assisted-5dvpbgxg9u.pdf

Video-assisted thoracoscopic surgery versus robotic-assisted thoracoscopic surgery in the surgical treatment of Masaoka stage I thymoma

Background
This study aimed to compare the perioperative outcomes for patients who underwent transsternal or robot-assisted thymectomy and to determine the feasibility of robot-assisted thymectomy for the treatment of Masaoka stages 1 and 2 thymomas.

Surgical treatment of early-stage thymomas: robot-assisted thoracoscopic surgery versus transsternal thymectomy

No standard therapy for pulmonary epithelioid hemangioendothelioma (PEH) has yet been established due to the rarity of the disease, the lack of clear standards for treatment and the partial-to-complete spontaneous regression. This report describes three cases of PHE manifested as bilateral intrapulmonary masses with an initial diagnosis conducted by thoracoscopic lung biopsy. These patients demonstrated a partial response to combination chemotherapy with carboplatin, paclitaxel, bevacizumab or endostar, and an improvement in clinical status. Furthermore, we reviewed the literature regarding such patients who received chemotherapy and immunotherapy; this indicated that patients with PEH demonstrated a good partial response to chemotherapy with carboplatin, paclitaxel, bevacizumab, thalidomide and α-interferon. Overall, combination chemotherapy regimens may hold therapeutic potential for the treatment of this rare disease.

/pdf/treatment-of-pulmonary-epithelioid-hemangioendothelioma-with-2f6esyllg3.pdf

Wang Li

Papers

Surgical techniques for early-stage thymoma: video-assisted thoracoscopic thymectomy versus transsternal thymectomy.

Predictive Factors for Lymph Node Metastasis in Clinical Stage IA Lung Adenocarcinoma

Video-assisted thoracoscopic surgery versus robotic-assisted thoracoscopic surgery in the surgical treatment of Masaoka stage I thymoma

Surgical treatment of early-stage thymomas: robot-assisted thoracoscopic surgery versus transsternal thymectomy

Treatment of pulmonary epithelioid hemangioendothelioma with combination chemotherapy: Report of three cases and review of the literature