Inflammatory myofibroblastic tumors are rare soft tissue neoplasms with an uncertain biological behavior, derived from fibroblastic and myofibroblastic cells. In rare cases, a peculiar epithelioid phenotypic variant of this tumor is encountered, named epithelioid inflammatory myofibroblastic sarcoma (EIMS). EIMS has overlapping features with inflammatory myofibroblastic tumor but has been correlated with a more aggressive clinical course, a characteristic nuclear membrane or perinuclear anaplastic lymphoma kinase (ALK) immunostaining pattern and a very specific RANBP2-ALK fusion. To date, EIMS has been reported almost exclusively in the abdominal and pelvic cavity, with the exception of some intrathoracic cases. Herein, we present the first case of primary cutaneous EIMS, confirmed by molecular analysis showing the diagnostic RANBP2-ALK fusion.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1097/DAD.0000000000002096 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
RAPO Attenuates Dermal and Pulmonary Fibrosis in a Mouse Model of Systemic Sclerosis through Macrophage Modulation and Growth of Short-Chain Fatty Acid Producers.
Immune Netw
December 2024
Division of Rheumatology, Department of Internal Medicine and Institute of Medical Science, Gyeongsang National University School of Medicine and Gyeongsang National University Hospital, Jinju 52727, Korea.
Systemic sclerosis (SSc) is a complex autoimmune disease with an unclear etiology and no effective treatments. Recent research has suggested involvement of the microbiome in SSc pathogenesis. This study aimed to identify specific microbial species associated with SSc and explore their therapeutic potential.
View Article and Find Full Text PDFPhysical Exercise: A Promising Treatment Against Organ Fibrosis.
Int J Mol Sci
January 2025
College of Physical Education, Shanghai University, Shanghai 200444, China.
Fibrosis represents a terminal pathological manifestation encountered in numerous chronic diseases. The process involves the persistent infiltration of inflammatory cells, the transdifferentiation of fibroblasts into myofibroblasts, and the excessive deposition of extracellular matrix (ECM) within damaged tissues, all of which are characteristic features of organ fibrosis. Extensive documentation exists on fibrosis occurrence in vital organs such as the liver, heart, lungs, kidneys, and skeletal muscles, elucidating its underlying pathological mechanisms.
View Article and Find Full Text PDFInflammatory Myofibroblastic Tumor of the Lung: An Incidental Finding of a Pediatric Case.
Ann Thorac Surg Short Rep
September 2024
Health Surveillance Centers, Ministry of Health, Jeddah Saudi Arabia.
Inflammatory myofibroblastic tumor is a rare occurring benign tumor composed of myofibroblastic spindle cells. Lung inflammatory myofibroblastic tumor is difficult to diagnose and may mimic lung cancer or infectious etiology. Surgical intervention with final histopathologic confirmation remains the mainstay of diagnosis.
View Article and Find Full Text PDFMetabolic Atlas of Human Eyelid Infiltrative Basal Cell Carcinoma.
Invest Ophthalmol Vis Sci
January 2025
State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-Sen University, Guangdong Provincial Key Laboratory of Ophthalmology and Visual Science, Guangzhou, China.
Purpose: Eyelid infiltrative basal cell carcinoma (iBCC) is the most common malignant tumor affecting the ocular adnexa, but studies on metabolic changes within its microenvironment and heterogeneity at the tumor invasive area are limited. This study aims to analyze metabolic differences among iBCC cell types using single-cell and spatial metabolomics analysis and to examine metabolic environment at the tumor invasive area.
Methods: Single-cell transcriptomic data of human basal cell carcinoma (BCC) were clustered and visualized using Uniform Manifold Approximation and Projection.
Introduction: 5-methoxytryptophan (5-MTP) is an anti-inflammatory metabolite. Several recent reports indicate that 5-MTP protects against post-injury tissue fibrosis. It was unclear how 5-MTP controls tissue fibrosis.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!