A5027358780- Vascular Malformations and Hemangiomas
- Vascular Malformations Diagnosis and Treatment
- Vascular Tumors and Angiosarcomas
- Tumors and Oncological Cases
- CNS Lymphoma Diagnosis and Treatment
- Tuberous Sclerosis Complex Research
- Platelet Disorders and Treatments
- Glioma Diagnosis and Treatment
- Teratomas and Epidermoid Cysts
- Neuroblastoma Research and Treatments
- Sarcoma Diagnosis and Treatment
- Acute Myeloid Leukemia Research
- Chromatin Remodeling and Cancer
- Central Venous Catheters and Hemodialysis
- Vascular anomalies and interventions
- Vascular Anomalies and Treatments
- Full-Duplex Wireless Communications
- Renal Diseases and Glomerulopathies
- Tracheal and airway disorders
- Immunodeficiency and Autoimmune Disorders
- Lymphatic Disorders and Treatments
- Parvovirus B19 Infection Studies
- Neurofibromatosis and Schwannoma Cases
- Autoimmune and Inflammatory Disorders Research
- Hemophilia Treatment and Research
Gifu University
2016-2025
National Hospital Organization
2019-2024
Nagoya Medical Center
2019-2024
Gifu Prefectural General Medical Center
2023
Pediatrics and Genetics
2016-2019
Gifu University Hospital
2018-2019
Institut Curie
2014
Barwon Health
2014
Medical University of Graz
2014
German Cancer Research Center
2014
Background Complex lymphatic anomalies are intractable disorders, including generalized anomaly (GLA), Gorham–Stout disease (GSD), and kaposiform lymphangiomatosis (KLA). The etiology of these diseases remains unknown diagnosis is confused by their similar clinical findings. This study aimed to clarify the differences in features prognosis among GLA, KLA, GSD, Japanese patients. Procedure Clinical features, radiological pathological findings, treatment, patients were obtained from a...
Lymphatic anomalies (LAs) include several disorders in which abnormal lymphatic tissue invades the neck, chest, and various organs. Progressive cases may result lethal outcomes have proven difficult to treat. Sirolimus is showing promising results management of vascular anomalies. We examined efficacy safety sirolimus treatment patients with progressive LAs. All LAs treated from May 2015 September 2018 were included. They received oral once a day dose was adjusted so that trough...
Introduction Intractable lymphatic anomalies (LAs) include cystic malformation (LM; macrocystic, microcystic, or mixed), generalized anomaly, and Gorham–Stout disease. LAs can present with severe symptoms poor prognosis. Thus, prospective studies for treatments are warranted. We conducted a clinical trial of sirolimus intractable LAs. Methods This was an open-label, single-arm, multicenter, involving five institutions in Japan. All patients received oral once daily, the dose adjusted to...
Lymphatic malformation (LM), which was previously termed lymphangioma, is a rare congenital of the lymphatic system and its treatment still challenging. Propranolol (beta blocker) has been recently developed as first-line infantile hemangioma. Our study aimed to assess effect propranolol on pediatric LM relationship between effectiveness vascular endothelial growth factor (VEGF) family members (VEGF-A, C D). Six Japanese patients with (age range: 10 months-19 years old; 2 macrocystic,...
Abstract Background Kaposiform lymphangiomatosis (KLA) has recently been distinguished as a novel subtype of generalized lymphatic anomaly (GLA) with foci spindle endothelial cells. All cases KLA involve multiple organs and have an unfavorable prognosis. However, the molecular pathogenesis is unknown, there are no useful biomarkers. In present study, we performed genetic analysis to elucidate cause this disease detect biomarkers for it. Methods We whole-exome sequencing DNA samples from...
Abstract To identify patients with infantile hemangioma (IH) in need of early‐stage treatment this multicenter, prospective, observational study, we investigated the potential plasma cytokines as a clinically useful marker. Plasma samples were collected at three time points from 41 hemangioma: baseline (days 14–60 after delivery), visit 1 61–150, proliferative phase), and 2 151–395, involuting phase). With twofold or more increase tumor volume during baseline–visit period regarded...
Background/Objectives: The therapeutic efficacy of percutaneous sclerotherapy (PS) for venous malformations (VMs) based on volumetric magnetic resonance imaging (MRI) measurements and its association with early post-treatment coagulation markers remains unexplored. This study evaluates the 5% monoethanolamine oleate (EO)-based PS in treating difficult-to-resect VMs using MRI investigates changes markers. Methods: post-hoc analysis utilized data from a prospective, open-label, multicenter...
To evaluate the effect and safety of sclerotherapy in patients with difficult-to-resect venous malformations treated ethanolamine oleate. This investigator-initiated clinical trial employed a multicenter, single-arm design was conducted Japan. Overall, 44 were categorized into two cohorts: 22 cystic-type diffuse-type malformations, including children (<15 years old). Adult received injections 5% oleate solution, double diluted contrast or normal saline, maximum dose 0.4 mL/kg. The same...
The efficacy, safety, and optimal concentration of topical sirolimus gel for treatment cutaneous lesions vascular anomalies requires evaluation. This study was a multicenter, double-blind, placebo-controlled, parallel-group, phase II randomized clinical trial. We enrolled patients with venous malformation (n = 27), lymphatic 14), tufted angioma 8), or kaposiform hemangioendothelioma 1). Patients applied either placebo 0.2% 0.4% to the target lesion twice per day 12 weeks. primary endpoint...
Schwannomas arise from Schwann cells of the peripheral nerve sheath. are usually benign, and most them located in head, neck, or distal extremities. The retroperitoneal region is an unusual location for schwannomas. seen adults very rare pediatric population. A 6-year-old boy was referred to our institution with a right abdominal mass. His main complaint intermittent myoclonus his lower limb. Abdominal computed tomography (CT) magnetic resonance imaging scans revealed round-shaped solid mass...
Pseudomyogenic hemangioendothelioma (PMH) is a recently described vascular neoplasm that occurs most commonly in the soft tissue of distal extremities young adults. Metastatic PMH can be fatal and there are no effective medications. We describe case 15-year-old boy with metastatic PMH, who responded to treatment everolimus, mammalian target rapamycin inhibitor. Immunohistochemistry showed was expressed biopsy specimens, which may explain reduction tumor size following treatment.
Lymphatic anomalies (LAs) refer to a group of diseases involving systemic dysplasia lymphatic vessels. These lesions are classified as cystic malformation (macrocystic, microcystic or mixed), generalized anomaly, and Gorham-Stout disease. LAs occur mainly in childhood, present with various symptoms including chronic airway problems, recurrent infection, organ disorders. Individuals often experience progressively worsening deteriorating quality life. Although limited treatment options...
Venous malformations (VMs) are the most common vascular malformations. TEK and PIK3CA causal genes of VMs, may be involved in PI3K/AKT pathway. However, downstream mechanisms underlying or mutations VMs not completely understood. This study aimed to identify a possible association between genetic clinicopathological features. A retrospective clinical, pathological, 114 patients with was performed. TEK, PIK3CA, combined TEK/PIK3CA were identified 49 (43%), 13 (11.4%), 2 (1.75%) patients,...
Gorham–Stout disease (GSD) is a rare disorder of unknown etiology. We present 6-year-old male with GSD involving the skull base who presented recurrent cerebrospinal fluid (CSF) rhinorrhea, severe hearing loss, and facial palsy secondary to cerebellar herniation into internal auditory canal. After 2 months treatment pegylated interferon (IFN) α-2b (50 μg/week), his recovered dramatically. Two years later, new bone formation appeared radiologically IFN was switched sirolimus. One year after...
Propranolol has recently been shown to be highly effective for infantile hemangioma (IH), but the mechanism of action propranolol and usefulness measurement vascular endothelial growth factor (VEGF) remain poorly understood. The aim this study was therefore determine efficacy treatment evaluate changes in plasma VEGF IH patients who underwent treatment.The group consisted 35 children with IH. Oral give at a dose 2.0 mg/kg/day divided three doses. Outcome assessed using visual analog scale...
Primary intestinal lymphangiectasia (PIL), also known as Waldmann's disease, is an exudative enteropathy resulting from morphologic abnormalities in the lymphatics. In this article, we describe a 12-year-old boy with PIL that led to protein-losing characterized by diarrhea, hypoalbuminemia associated edema (serum albumin level: 1.0 g/dL), and hypogammaglobulinemia IgG 144 mg/dL). Severe hypoalbuminemia, electrolyte abnormalities, tetany persisted despite low-fat diet propranolol. Everolimus...
Abstract Background Kaposiform lymphangiomatosis (KLA) has recently been distinguished as a novel subtype of generalized lymphatic anomaly (GLA), and is characterized by foci spindle endothelial cells amid background malformed channels. The etiology these diseases remains unknown diagnosis confounded their similar clinical findings. This study aimed to clarify differences in the findings plasma cytokine profiles GLA KLA patients. Procedure Clinical features data patients were obtained from...