[Chest Wall Reconstruction with Polypropylene Mesh After Resection of Primary Sternal Chondrosarcoma].

Although rare, primary sternal chondrosarcoma (PSC) is the most common primary malignant chest wall tumor. Here, we report a rare case of PSC treated with partial sternal resection and reconstruction. A 47-year-old man presented with subcutaneous anterior chest wall mass and chest pain.

Thymoma Exhibiting Spontaneous Regression With Cystic Change Due to Acute Infarction: A Case Report and Literature Review.

Spontaneous regression (SR) of thymoma is rare. We report a case of a surgically resected thymoma due to cystic changes owing to acute ischemic infarction with an increased anti-acetylcholine receptor antibody level. A 61-year-old male underwent a computed tomography (CT) scan, which showed a 4.

[Surgically Resected Cases of Mediastinal Ganglioneuroma Detected in Adults].

A ganglioneuroma is a rare, benign, neurogenic tumor originating from the sympathetic ganglion. Mediastinal ganglioneuroma are mostly detected in children, typically around 10 years of age, and are rarely identified in adults. Herein, we report two surgically resected cases of mediastinal ganglioneuroma in adults.

Efficacy and Safety of the Natural Killer T Cell-Stimulatory Glycolipid OCH-NCNP1 for Patients With Relapsing Multiple Sclerosis: Protocol for a Randomized Placebo-Controlled Clinical Trial.

Background: Multiple sclerosis (MS) is an autoimmune inflammatory disease of the central nervous system that causes myelin sheath damage and axonal degeneration. The glycolipid (2S, 3S, 4R)-1-O-(α-d-galactosyl)-2-tetracosanoylamino-1,3,4-nonaetriol (OCH-NCNP1 or OCH) exerts an immunoregulatory action that suppresses T helper (Th)1 cell-mediated immune responses through natural killer T cell activation, selective interleukin-4 production, and Th2 bias induction in human CD4-positive natural killer T cells.

Objective: This trial aims to investigate the efficacy and safety of the immunomodulator OCH in patients with relapsing MS through 24-week repeated administration.

Atypical type A thymoma component identified by pulmonary metastasectomy 11 years after surgery of type AB thymoma.

Atypical type A thymomas exhibit more aggressive features than conventional type A thymomas. Type AB thymomas rarely have atypical type A components. We report a rare case of type AB thymoma with an atypical type A component, that was identified after pulmonary metastasectomy 11 years after the primary surgery and long-term follow-up after recurrence.

Mediastinal aberrant goiter characterized by high radiodensity on non-enhanced computed tomography: a case report.

Background: A mediastinal aberrant goiter is extremely rare, characterized by independence from the thyroid gland. This tumor most commonly develops in the anterior mediastinum and should be differentiated from a thymoma. Moreover, as mediastinal aberrant goiters frequently have a blood supply arising from the thoracic great vessels, preoperative diagnosis and evaluation of the feeding vessel are vital for safe surgery.

Pulmonary metastasis of matrix-producing carcinoma mimicking small cell lung cancer.

Matrix-producing carcinoma (MPC) of the breast is an extremely rare subtype of invasive breast cancer. MPC is characterized by the production of a cartilaginous or osseous matrix without spindle cells. It is an aggressive carcinoma, often presenting as triple-negative breast cancer.

Successful Video-Assisted Thoracoscopic Removal of a Chest Tube Protruding Deep Into the Pulmonary Hilum.

Tube thoracostomy is an invasive procedure frequently used to drain pleural fluid collections or to manage pneumothorax, wherein the lungs commonly sustain trauma. In some cases, deep pulmonary hilar injuries are managed by anatomical lung resections. A deep hilar injury with the chest tube protruding into the lung parenchyma is a rare complication of tube thoracostomy.

Thymoma exhibiting spontaneous regression with developing myasthenia gravis: A case report.

Spontaneous regression (SR) of thymoma is rare. A 44-year-old man with right chest pain underwent computed tomography (CT), which showed an 11.0 cm mass in the anterior mediastinum and right pleural effusion.

Extra-muscular manifestations in GNE myopathy patients: A nationwide repository questionnaire survey in Japan.

Objective: GNE myopathy is a rare autosomal recessive adult-onset distal myopathy caused by biallelic pathogenic variants in GNE. Although some extra-muscular manifestations associated with GNE myopathy have been reported, little is known about whether they are disease-specific and how often they present. This study aimed to characterize extra-muscular manifestations of GNE myopathy.

Multifocal Cholesterol Granulomas of the Thymus: A Case Report of Positron Emission Tomography- Positive Benign Tumor of the Anterior Mediastinum.

Cholesterol granulomas of the thymus are extremely rare benign entities. Herein, we describe a case of cholesterol granuloma in a 45-year-old man who presented with multifocal anterior mediastinal lesions on computed tomography (CT). Positron emission tomography (PET)-CT revealed increased fluorodeoxyglucose uptake, with a maximum standardized uptake value of 8.

Patient characteristics and burden of disease in Japanese patients with generalized pustular psoriasis: Results from the Medical Data Vision claims database.

Generalized pustular psoriasis (GPP) is a rare and severe systemic, neutrophilic skin disease. To date, accurate clinical profiling of patients with GPP remains poorly understood. In this study, we present the characteristics and estimate the burden of disease in patients with GPP compared with those with plaque psoriasis, in Japan.

Feasibility of intraoperative diagnosis of lung adenocarcinoma in situ to avoid excessive resection.

Background: Limited lung resection is generally believed to be available for lung adenocarcinoma in situ (AIS). At our institute, intraoperative hematoxylin-eosin staining of frozen-section slides is routinely performed for evaluating tumor invasiveness after partial resection to avoid excessive lung resection. This study aimed to evaluate the feasibility and usefulness of intraoperative frozen-section diagnosis of AIS.

Lymphatic permeation and vascular invasion should not be integrated as lymphovascular invasion in lung adenocarcinoma.

Objective: Lymphatic permeation (LY) and vascular invasion (VI) are well-known as postoperative prognostic factors in non-small cell lung cancer (NSCLC). Some reports use the term "lymphovascular invasion (LVI)" in reference to the integration of LY and VI. The purpose in this study is to elucidate whether NSCLC with LY or VI can be labeled as LVI-positive NSCLC or it should be treated as an LY- or VI-positive tumor, respectively.

How long is cessation of preoperative smoking required to improve postoperative survival of patients with pathological stage I non-small cell lung cancer?

Background: Smoking can cause non-small cell lung cancer (NSCLC). However, the effects of preoperative smoking on tumor progression are not well-known. In addition, the duration of smoking cessation that can provide NSCLC patients with smoking history similar postoperative prognosis as that of nonsmokers remains unknown.

Pregnancy in GNE myopathy patients: a nationwide repository survey in Japan.

Background: GNE myopathy is an autosomal recessive adult-onset distal myopathy. While a few case reports have described the progression of GNE myopathy during pregnancy, to our knowledge, none have examined disease progression after delivery or obstetric complications.

Objective: This study aimed to reveal maternal complications, newborn complications, and the impact of pregnancy on disease progression in GNE myopathy patients.

Clinicopathological Factors Related to Recurrence Patterns of Resected Non-Small Cell Lung Cancer.

Even after complete resection, non-small cell lung cancer (NSCLC) shows preferential recurrence in the mediastinal lymph nodes, lungs, brain, bone, liver, and adrenal gland. However, the relationship between clinicopathological factors and recurrence patterns after resection has not been well-evaluated. Among 688 NSCLC cases with complete resection between 2004 and 2016, 233 cases recurred at our institute.

Expectations and anxieties of Duchenne muscular dystrophy patients and their families during the first-in-human clinical trial of NS-065/NCNP-01.

Duchenne muscular dystrophy (DMD) is a recessive X-linked genetic disease caused by a mutation in the dystrophin gene. The new drug NS-065/NCNP-01 utilizing exon-skipping therapy targeting specific deletions has been used in a first-in-human trial for the treatment of DMD. We surveyed 10 pairs of DMD participants and their parents within this clinical trial via an iPad survey form and through interviews regarding their understanding of the trial, expectations, anxieties, and reasons for participating in the trial.

Quantitative computed tomography for predicting cardiopulmonary complications after lobectomy for lung cancer in patients with chronic obstructive pulmonary disease.

Objectives: In lung cancer resection, chronic obstructive pulmonary disease is a risk factor for post-operative complications. Few studies on post-operative complications of lung cancer resection have considered radiographic emphysematous change as an index. Here, we have examined the relationship between the regional ratio of the emphysematous area in pre-operative computed tomography images and cardiopulmonary complications in patients with chronic obstructive pulmonary disease who underwent lung cancer resection.

[Thoracic Paravertebral Block in Patients Ineligible for Epidural Block].

We retrospectively assessed the effectiveness and the safety of thoracic paravertebral block(PVB) in patients ineligible for epidural block (EP). Eleven PVB patients and 33 EP patients were enrolled. Postoperative pain was evaluated using a numerical rating scale (NRS).

Clinical trial network for the promotion of clinical research for rare diseases in Japan: muscular dystrophy clinical trial network.

Background: Duchenne muscular dystrophy (DMD) is the most commonly inherited neuromuscular disease. Therapeutic agents for the treatment of rare disease, namely "orphan drugs", have recently drawn the attention of researchers and pharmaceutical companies. To ensure the successful conduction of clinical trials to evaluate novel treatments for patients with rare diseases, an appropriate infrastructure is needed.

High-Grade Neuroendocrine Carcinoma with Bronchial Intraepithelial Tumor Spread: Possibly a New Histologic Feature of Large-Cell Neuroendocrine Carcinoma.

Introduction: During surgical resection of a peripherally located high-grade neuroendocrine carcinoma (HGNEC), we unexpectedly discovered prominent bronchial intraepithelial tumor spread up to the surgical end of the bronchus. Because bronchial intraepithelial tumor spread of peripherally located HGNEC has been rarely reported, we conducted a retrospective analysis at our hospital.

Methods: We histologically reviewed surgically resected HGNEC cases to assess bronchial intraepithelial spread of tumor cells.

[Infrastructure for the clinical research of muscular dystrophies: remudy and MDCTN].

Remudy, operated by the NCNP, runs two national registries for Dystrophinopathy and GNE myopathy in Japan under the collaboration with the TREAT-NMD alliance. The aim is to construct the clinical research infrastructure and accelerate the clinical development research for these rare diseases. We successfully provide the data sets for the feasibility studies, send out the appropriate information of the clinical trials for the candidates to speed up the recruitment for trials, collaboration with the Muscular Dystrophy Clinical Trial Network: MDCTN, as well as present the natural history and epidemiological data of the rare diseases with a new 'registry based' research style.

Cooperative International Neuromuscular Research Group Duchenne Natural History Study demonstrates insufficient diagnosis and treatment of cardiomyopathy in Duchenne muscular dystrophy.

Introduction: Cardiomyopathy is a common cause of morbidity and death in patients with Duchenne muscular dystrophy (DMD).

Methods: This investigation was a cross-sectional cross-sectional analysis of clinical data from the multi-institutional Cooperative International Neuromuscular Research Group (CINRG) DMD Natural History Study of 340 DMD patients aged 2-28 years. Cardiomyopathy was defined as shortening fraction (SF) <28% or ejection fraction (EF) <55%.