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Therapeutic options in inoperable ROS1-rearranged inflammatory myofibroblastic tumor of the tongue in a child: a case report and literature review.

Inflammatory myofibroblastic tumor (IMT) is a rare borderline malignancy, usually treated with surgery only. Exceedingly rare cases of inoperable, recurrent, or metastatic IMTs pose a therapeutic challenge. We report successful treatment of a 7-year-old girl with an inoperable anaplastic lymphoma kinase (ALK)-negative IMT of the tongue. The patient underwent various anti-inflammatory (steroids, nonsteroidal anti-inflammatory drugs, clarithromycin) and antiproliferative (chemotherapy) therapies to enable tumor regression and complete resection. Ultimately, next-generation sequencing of the tumo

Aortic Rupture due to Co-localization of Aortic Intimal Myofibroblastic Sarcoma and Urothelial Carcinoma: A Unique Case Report.

We herein report a unique case of aortic rupture due to co-localization of aortic intimal myofibroblastic sarcoma (IMFS) and urothelial carcinoma (UC). A 76-year-old man who was being followed up after surgery for UC 5 years earlier developed aortic rupture and underwent emergency surgery. Intraoperatively, a tumorous mass on the luminal side of the aortic arch was found near the rupture. A histopathological analysis of the mass revealed aortic IMFS. Furthermore, co-localization of IMFS and UC cells was found near the rupture. The fragility of the aortic wall due to co-localization of IMFS and

Adult Mid Ileo-Ileal Intussusception Secondary to Inflammatory Myofibroblastic Tumor (IMT): A Case Report and Literature Review.

The intestinal intussusception as a cause of bowel obstruction in adult is a rare finding and is a heralder for an underlying lesion. This is in stark contrast to intestinal intussusception in pediatric population where the etiology is always a primary or benign condition and thus bears different outcomes. We hereby present a case of a young female adult, without the presence of any significant comorbidity. She presented with non-specific symptoms of small bowel intussusception. She was diagnosed as a case of adult intussusception with abdominal ultrasonography. The patient underwent complete

Spontaneous pneumothorax caused by an inflammatory myofibroblastic tumor-like lesion in a 14-year-old girl: a case report.

BACKGROUND: Spontaneous pneumothorax occurs more often in younger, slim, and shallow-chested men. Although less common, differential diagnoses for secondary pneumothorax in children are asthma, emphysematous blebs, catamenial pneumothorax, and others. We report a patient who presented with pneumothorax and was found to have an inflammatory myofibroblastic tumor (IMT)-like lesion, and present a review of the related literature. CASE PRESENTATION: A 14-year-old girl visited her physician for chest pain that developed while exercising. Although chest drainage was performed, the symptoms associat

Over expression of CDK4 and MDM2 in a patient with recurrent ALK-negative mediastinal inflammatory myofibroblastic tumor: A case report.

RATIONALE: The diagnosis of anaplastic lymphoma kinase (ALK)-negative inflammatory myofibroblastic tumors (IMT) remains challenging because of their morphological resemblance with spindle cell sarcoma with myofibroblastic characteristics. PATIENT CONCERNS: A 69-year-old female patient presented with loco-regional recurrent IMT several times within 8 years after primary treatment and neck lymph node metastasis 3.5 years after last recurrence. DIAGNOSIS: The primary, recurrence, and lymph node metastasis lesions were diagnosed as ALK-negative IMTs based on the histopathological features. INTE

Positive Feedback Loop of SNAIL-IL-6 Mediates Myofibroblastic Differentiation Activity in Precancerous Oral Submucous Fibrosis.

Oral submucosal fibrosis (OSF) is a premalignant disorder of the oral cavity, and areca nut chewing is known to be a major etiological factor that could induce epithelial to mesenchymal transition (EMT) and activate buccal mucosal fibroblasts (BMFs). However, this detailed mechanism is not fully understood. In this study, we showed that the upregulation of Snail in OSF samples and fibrotic BMFs (fBMFs) may result from constant irritation by arecoline, a major alkaloid of the areca nut. The elevation of Snail triggered myofibroblast transdifferentiation and was crucial to the persistent activat

Radiological diagnostic dilemma in a child with small aggressive facial mass, time to increase the differentials: an inflammatory myofibroblastic tumour.

A 5-year-old girl with left facial swelling in the medial maxillary region close to the nasal ala was brought by her parents to our head and neck clinic. They have visited other doctors for similar presentation in the last 6 months, which started as redness and swelling, with occasional epiphora. The redness resolved after medical treatment, with slight regression of swelling, although it did not disappear. CT and MRI showed a locally aggressive, small enhancing soft tissue mass involving the left anteromedial maxillary wall, the nasal bone and the orbital floor. The mass involved the bony cou

Cardiomyocyte-derived exosomal microRNA-92a mediates post-ischemic myofibroblast activation both in vitro and ex vivo.

AIMS: We hypothesize that specific microRNAs (miRNAs) within cardiomyocyte-derived exosomes play a pivotal role in the phenoconversion of cardiac myofibroblasts following myocardial infarction (MI). METHODS AND RESULTS: We used an established murine model of MI, obtained in vivo via ligation of the left anterior descending coronary artery. We isolated adult cardiomyocytes and fibroblasts, and we assessed the functional role of cardiomyocyte-derived exosomes and their molecular cargo in the activation of cardiac fibroblasts. We identified and biologically validated miR-92a as a transcriptional…

Colonic inflammatory myofibroblastic tumour presenting as 'pyrexia of unknown origin': report of a rare disease and its unique presentation.

An intra-abdominal inflammatory myofibroblastic tumour (IMT) belongs to a rare group of diseases initially described as an inflammatory pseudotumour. Even though it is seen more often in children, its incidence in adults is even rarer. Clinical presentations can vary depending on its site and inherent tumour properties. The colon is an uncommon site for IMT and pyrexia of unknown origin (PUO) as its dominant clinical presentation is even rarer. A 27-year-old woman presented with PUO. She was evaluated under the department of internal medicine before undergoing an 18F-fluorodeoxyglucose positro

Follistatin Mitigates Myofibroblast Discrimination и Collagen System of Fibroblast из Scar Tise вокруг поврежденного Flexor Tendons.

ЦЕЛЬ: Цель этого исследования состояла в изучении воздействия гена FST на ингибирование фиброза в фиброблатических клетках от шрамных тканей вокруг отремонтированных сухожилий зоны II. МАТЕРИАЛЫ И МЕТОДЫ: Иммуногистохимия была проведена на фибробалластных клетках, передающихся с помощью аденовируса-Лакза (Ад-Лакза) в качестве маркерного гена (контроля), или с аденовирусом-ФСТ (АД-ФСТ) в качестве терапевтического гена. Культуры фибробласта без аденовального воздействия служат средством контроля. Результаты: Фибробластические клетки, передающиеся с помощью AD-FST, продемонстрировали значительное

Совпадение в аннотации оригинала
PURPOSE: The aim of this study was to investigate the effect of FST gene on the inhibition of fibrosis in fibroblastic cells from scar tissu … eventing scar adhesions around repaired tendons by inhibiting fibroblasts from differentiating into myofibroblasts, in addition to producing type I collagen and regulating extracellular matrix turnover via the downregulation of MMP-1 and TIMP-1. FST may also decrease contracture of the scar by inhibiting Ca2+-dependent cell contraction.

Vocal cord inflammatory myofibroblastic tumor with mucoid deposits harboring TIMP3-ALK fusion: A potential diagnostic pitfall.

A 35-year-old Japanese man who had experienced hoarseness for 10 years presented with a vocal cord lesion. A gross examination revealed a le … mentin, and negative for other smooth muscle markers by immunohistochemistry. Structures resembling myofibroblasts were not observed by electron microscopy, which confirmed abundant rough endoplasmic reticulum in the tumor cells and accumulated lipid droplets in some tumor cells. ALK gene rearrangement was detected by fluorescence in situ hybridization, and TIMP …

Double amplifications of CDK4 and MDM2 in a gastric inflammatory myofibroblastic tumor mimicking cancer with local invasion of the spleen and diaphragm.

Inflammatory myofibroblastic tumor (IMT) is currently recognized as an intermediate mesenchymal neoplasm. It can arise anywhere in the body, but it is particularly common in the lungs. Gastric IMT is very rare in adults. In this study, we report a case of a 68-year-old woman with IMT in the gastric cardia, with invasion into the spleen and diaphragm. Because of its location and aggressive clinical features, it was first mistaken for gastric cancer. However, pathology and immunohistochemistry were used to finally confirm the diagnosis of IMT after total resection of the tumor and spleen and par

Coordinate activities of BRD4 and CDK9 in the transcriptional elongation complex are required for TGFβ-induced Nox4 expression and myofibroblast transdifferentiation.

Transdifferentiation of quiescent dermal fibroblasts to secretory myofibroblasts has a central role in wound healing and pathological scar formation. This myofibroblast transdifferentiation process involves TGFβ-induced de novo synthesis of alpha smooth muscle cell actin (αSMA)+ fibers that enhance contractility as well as increased expression of extracellular matrix (ECM) proteins, including collagen and fibronectin. These processes are mediated upstream by the reactive oxygen species (ROS)-producing enzyme Nox4, whose induction by TGFβ is incompletely understood. In this study, we demonstrat…

Epithelioid inflammatory myofibroblastic sarcoma: a clinicopathological, immunohistochemical and molecular cytogenetic analysis of five additional cases and review of the literature.

BACKGROUND: To explore the clinical characteristics and pathological features of epithelioid inflammatory myofibroblastic sarcoma (EIMS) with emphasis on the diagnostic spectrum. METHODS: The clinical data and histological features in 5 additional cases of EIMS were retrospectively reviewed. Immunohistochemical study and interphase fluorescence in situ hybridization (FISH) analysis were carried out. RESULTS: There were 2 males and 3 females with age at presentation ranging from 15 to 58 years (mean, 37 years). All 5 tumors were intra-abdominal with 2 arising in the mesentery and 1 each in th

Chromoplectic TPM3-ALK rearrangement in a patient with inflammatory myofibroblastic tumor who responded to ceritinib after progression on crizotinib.

BACKGROUND: Inflammatory myofibroblastic tumors (IMTs) are rare sarcomas that can occur at any age. Surgical resection is the primary treatment for patients with localized disease; however, these tumors frequently recur. Less commonly, patients with IMTs develop or present with metastatic disease. There is no standard of care for these patients and traditional cytotoxic therapy is largely ineffective. Most IMTs are associated with oncogenic ALK, ROS1 or PDGFRβ fusions and may benefit from targeted therapy. PATIENT AND METHODS: We sought to understand the genomic abnormalities of a patient who

Intrinsic Deregulation of Vascular Smooth Muscle and Myofibroblast Differentiation in Mesenchymal Stromal Cells from Patients with Systemic Sclerosis.

INTRODUCTION: Obliterative vasculopathy and fibrosis are hallmarks of systemic sclerosis (SSc), a severe systemic autoimmune disease. Bone m … h cell types, yet with striking phenotypic and functional differences: In relation to H-MSC-derived myofibroblasts induced by TGF-β1, those obtained from SSc-MSCs expressed more contractile proteins, migrated towards TGF-β1, had low proliferative capacity, and secreted higher amounts of collagen paralleled by reduced MMP expression. Higher levels of TGF-β recept …

Perivascular Cells in Diffuse Cutaneous Systemic Sclerosis Overexpress Activated ADAM12 and Are Involved in Myofibroblast Transdifferentiation and Development of Fibrosis.

OBJECTIVE: Microvascular damage is pivotal in the pathogenesis of systemic sclerosis (SSc), preceding fibrosis, and whose trigger is not still fully understood. Perivascular progenitor cells, with profibrotic activity and function, are identified by the expression of the isoform 12 of ADAM (ADAM12) and this molecule may be upregulated by transforming growth factor-β (TGF-β). The goal of this work was to evaluate whether pericytes in the skin of patients with diffuse cutaneous SSc (dcSSc) expressed ADAM12, suggesting their potential contribution to the fibrotic process, and whether TGF-β might

Fever,Weight Loss and Early Satiety due to Gastric Inflammatory Myofibroblastic Tumor; Case Report and Literature Review.

Gastric inflammatory myofibroblastic tumor (IMT) is a rare tumor with and unpredictable prognosis usually find in young adults. We present an 18-yearold man with gastric IMT. He complained of epigastric pain, intermittent fever and night sweating associated with weight loss since two years ago. Physical examination showed anemic and cachestic features with mild abdominal tenderness in palpitation as well as an abdominal mass in epigastrium. Abdominal CT scan revealed a huge mass that was arising from the stomach. Upper endoscopy revealed a submucosal lesion in fundus of stomach of approximatel

Loss of IκB kinase β promotes myofibroblast transformation and senescence through activation of the ROS-TGFβ autocrine loop.

Using forward and reverse genetics and global gene expression analyses, we explored the crosstalk between the IκB kinase β (IKKβ) and the transforming growth factor β (TGFβ) signaling pathways. We show that in vitro ablation of Ikkβ in fibroblasts led to progressive ROS accumulation and TGFβ activation, and ultimately accelerated cell migration, fibroblast-myofibroblast transformation and senescence. Mechanistically, the basal IKKβ activity was required for anti-oxidant gene expression and redox homeostasis. Lacking this activity, IKKβ-null cells showed ROS accumulation and activation of stres