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European Journal of Surgical Oncology :... May 2024Inflammatory myofibroblastic tumor (IMT) is a soft tissue neoplasm which can be locally invasive, recur, or in rare cases metastasize. Often originating from the abdomen... (Review)
Review
Inflammatory myofibroblastic tumor (IMT) is a soft tissue neoplasm which can be locally invasive, recur, or in rare cases metastasize. Often originating from the abdomen or thorax, IMT most commonly affects children and young adults. Due to its rarity comprehensive reports detailing clinical management and outcome(s) are sparse and often based on limited index case numbers. This study systematically analyzes outcome metrics of pediatric IMT and identifies risk factors for mortality. Medline/Embase databases were searched in accordance with PRISMA guidelines. Final analysis included 57 studies with 673 IMT patients (355 males, 53 %). Individual patient data was available for 405 cases with a median follow-up period of 36 months. Tumor sites included abdomen/pelvis (n = 233, 58 %), thorax (n = 125, 31 %), head/neck (n = 34, 8 %), and extremities (n = 13, 3 %). Surgical tumor resection was the mainstay of treatment, while only 20 patients (5 %) were treated non-operatively. Recurrence(s) were reported in 80 patients (20 %) with 34 (12 %) requiring reoperation. Positive tumor margins were a significant risk factor for tumor recurrence (p < 0.0001). Chemo/radiotherapy was reported in 98 patients (25 %). Most patients (94 %) survived; 81 % (n = 237) with no evidence of recurrent disease, 14 % (n = 41) were alive with disease, and 25 (6 %) died of disease. Positive margins at primary operation, and metastatic disease were associated with mortality (p < 0.0001 for both). IMT is a rare tumor with favorable outcome for the majority of patients. Whilst most patients will present with benign tumors, complete surgical resection (R0) is crucial, as positive surgical margins are a significant risk factor for tumor recurrence and mortality.
PubMed: 38713995
DOI: 10.1016/j.ejso.2024.108388 -
Diagnostics (Basel, Switzerland) Jan 2023Dermatofibromas (DFs) are benign fibrohistiocytic lesions that usually do not express CD34 protein. This study aimed to analyze the literature concerning the... (Review)
Review
BACKGROUND
Dermatofibromas (DFs) are benign fibrohistiocytic lesions that usually do not express CD34 protein. This study aimed to analyze the literature concerning the immunohistological and ultrastructural features of DFs. It also related these features to the histogenesis of these lesions.
METHODS
This study included a PubMed literature search for studies addressing the clinicopathological, ultrastructural, and immunohistochemical features of DFs. It also presented some current cases of CD34-negative DFs and a subset of these lesions with aberrant expression of this protein.
RESULTS
Analysis of the PubMed literature revealed that DFs with an aberrant expression of CD34 are rare tumors that commonly affect the extremities of adult females. Separating these tumors from dermatofibrosarcoma protuberans (DFSP, CD34-positive tumors) requires using a large panel of immunostains. Ultrastructurally, DFs are composed of diverse cell types, including cells with histiocytic, myofibroblastic, and fibroblastic features. An analysis of the DFs described by this study revealed that cases with an aberrant expression of CD34 protein had slightly high mean age and male sex predominance when compared to CD34-negative cases. The former commonly affected the extremities. There was no evidence of local recurrence or distant metastasis on follow-up.
CONCLUSIONS
DFs have the potential to express CD34 protein, defining a rare aberrant phenotype, which was not associated with any differences in the outcome as compared to CD34-negative DFs.
PubMed: 36672995
DOI: 10.3390/diagnostics13020185 -
Pharmacology & Therapeutics Jan 2024The treatment of interstitial lung diseases, including idiopathic pulmonary fibrosis (IPF), remains challenging as current available antifibrotic agents are not... (Review)
Review
The treatment of interstitial lung diseases, including idiopathic pulmonary fibrosis (IPF), remains challenging as current available antifibrotic agents are not effective in halting disease progression. Connective tissue growth factor (CTGF), also known as cellular communication factor 2 (CCN2), is a member of the CCN family of proteins that regulates cell signaling through cell surface receptors such as integrins, the activity of cytokines/growth factors, and the turnover of extracellular matrix (ECM) proteins. Accumulating evidence indicates that CTGF plays a crucial role in promoting lung fibrosis through multiple processes, including inducing transdifferentiation of fibroblasts to myofibroblasts, epithelial-mesenchymal transition (EMT), and cooperating with other fibrotic mediators such as TGF-β. Increased expression of CTGF has been observed in fibrotic lungs and inhibiting CTGF signaling has been shown to suppress lung fibrosis in several animal models. Thus, the CTGF signaling pathway is emerging as a potential therapeutic target in IPF and other pulmonary fibrotic conditions. This review provides a comprehensive overview of the current evidence on the pathogenic role of CTGF in pulmonary fibrosis and discusses the current therapeutic agents targeting CTGF using a systematic review approach.
Topics: Animals; Connective Tissue Growth Factor; Fibrosis; Fibroblasts; Transforming Growth Factor beta; Idiopathic Pulmonary Fibrosis; Transforming Growth Factor beta1; Lung
PubMed: 38103794
DOI: 10.1016/j.pharmthera.2023.108578 -
The British Journal of Surgery Jul 2016Radiation-induced fibrosis (RIF) is a late complication of radiotherapy that results in progressive functional and cosmetic impairment. Autologous fat has emerged as an... (Review)
Review
BACKGROUND
Radiation-induced fibrosis (RIF) is a late complication of radiotherapy that results in progressive functional and cosmetic impairment. Autologous fat has emerged as an option for soft tissue reconstruction. There are also sporadic reports suggesting regression of fibrosis following regional lipotransfer. This systematic review aimed to identify cellular mechanisms driving RIF, and the potential role of lipotransfer in attenuating these processes.
METHODS
PubMed, OVID and Google Scholar databases were searched to identify all original articles regarding lipotransfer for RIF. All articles describing irradiated fibroblast or myofibroblast behaviour were included. Data elucidating the mechanisms of RIF, role of lipotransfer in RIF and methods to quantify fibrosis were extracted.
RESULTS
Ninety-eight studies met the inclusion criteria. A single, definitive model of RIF is yet to be established, but four cellular mechanisms were identified through in vitro studies. Twenty-one studies identified connective tissue growth factor and transforming growth factor β1 cytokines as drivers of fibrotic cascades. Hypoxia was demonstrated to propagate fibrogenesis in three studies. Oxidative stress from the release of reactive oxygen species and free radicals was also linked to RIF in 11 studies. Purified autologous fat grafts contain cellular and non-cellular properties that potentially interact with these processes. Six methods for quantifying fibrotic changes were evaluated including durometry, ultrasound shear wave elastography, thermography, dark field imaging, and laser Doppler and laser speckle flowmetry.
CONCLUSION
Understanding how lipotransfer causes regression of RIF remains unclear; there are a number of new hypotheses for future research.
Topics: Adipose Tissue; Biomechanical Phenomena; Fibrosis; Humans; Hypoxia; Oxidative Stress; Platelet-Derived Growth Factor; Radiotherapy; Skin; Transforming Growth Factor beta1; Transplantation, Autologous; Tumor Necrosis Factor-alpha
PubMed: 27169866
DOI: 10.1002/bjs.10180 -
Cureus Jan 2024Dupuytren's disease (DD) is a fibroproliferative disorder that manifests as an abnormal growth of myofibroblasts, causing nodule formation and contractures and affecting... (Review)
Review
Comparing Complications and Patient Satisfaction Following Injectable Collagenase Versus Limited Fasciectomy for Dupuytren's Disease: A Systematic Review and Meta-Analysis.
Dupuytren's disease (DD) is a fibroproliferative disorder that manifests as an abnormal growth of myofibroblasts, causing nodule formation and contractures and affecting digit function. If left untreated, these contractures can lead to a loss of mobility and potentially impact hand function. This systematic review critically compares and evaluates the existing literature on the complications and patient satisfaction following injectable collagenase (CCH) versus limited fasciectomy (LF) for DD. We performed a comprehensive search of the PubMed, Medical Literature Analysis and Retrieval System Online (MEDLINE), The Cochrane Library, and Excerpta Medica database (EMBASE) databases from 2006 to August 2023. This research targeted all clinical studies involving adults who underwent injectable collagenase and/or limited fasciectomy in the management of DD. Out of the 437 identified studies, only 53 were considered eligible for our analysis, and merely 14 met our inclusion criteria. These selected studies encompassed a total of 967 patients with 1,344 treated joints, with an average follow-up duration of 19.22 (ranging from one to 84.06) months. Within this cohort, 498 joints from 385 patients underwent LF, while 846 joints from 491 patients received CCH injections. Notably, among the 491 patients treated with CCH, 1,060 complications were reported, averaging 2.15 complications per patient, with the most common being contusion/bruising/hematoma/ecchymosis (22.54%), and edema/swelling (18.96%). In contrast, among the 385 patients treated with LF, only 97 complications were reported, translating to 0.25 complications per patient, with the most frequent being paraesthesia or numbness (23.7%), scar sequelae like skin laceration, tear, fissure, or hypertrophic scar (23.7%), and neuropraxia or nerve injury (22.6%). Our meta-analysis indicates that paraesthesia or numbness is more frequently observed in LF than CCH injections, although without statistical significance, with a risk ratio (RR) of 0.39 (95% confidence interval (CI) 0.13-1.18, p-value 0.1). However, scar sequelae (hypertrophic scar, skin laceration, tear, or fissure) show a contrasting pattern, being more commonly associated with CCH injections than LF, with an RR of 1.98 (95% CI 0.26-14.85, p-value 0.51), which, upon eliminating the source of heterogeneity, becomes statistically significant, with an RR of 4.98 (95% CI 1.40-17.72, p-value 0.01). Our data revealed a higher frequency of complications with CCH compared to LF, although more severe adverse effects were observed in the LF group, such as neuropraxia or nerve injury. Scar sequelae were more common with CCH injections. Despite both treatments showing increased patient satisfaction at the final follow-up, CCH injection resulted in earlier improvements in satisfaction.
PubMed: 38420076
DOI: 10.7759/cureus.53147 -
Indian Journal of Urology : IJU :... 2024Inflammatory myofibroblastic tumors (IMTs) are intermediate-grade lesions that frequently recur and rarely metastasize. There are currently no guidelines on the... (Review)
Review
INTRODUCTION
Inflammatory myofibroblastic tumors (IMTs) are intermediate-grade lesions that frequently recur and rarely metastasize. There are currently no guidelines on the management of bladder IMTs. This systematic review aims to describe the clinical presentation and compare the management options for bladder IMTs.
METHODS
A PubMed/Medline search was conducted, according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, using the following Mesh terms: ("inflammatory myofibroblastic") AND ("tumor") OR ("tumor") AND ("bladder") AND ("case report"). A total of 75 case reports were included in the analysis.
RESULTS
The mean age of the patients was 36 years. 65% of the cases initially presented with hematuria. 68% of the tumors stained positive for anaplastic lymphoma kinase, and 20% invaded the muscularis. Patients underwent either transurethral resection of the bladder tumor (TURBT) only (34%), TURBT followed by complementary partial cystectomy (16%), or TURBT followed by radical cystectomy (4%). 36% and 9% of the cases underwent partial and radical cystectomy after the initial diagnosis, respectively. Cystectomies were performed using an open (74%), laparoscopic (14%), robotic-assisted (10%), or unknown (2%) approach. At a mean follow-up of 14 months, the recurrence and metastasis rates were about 9% and 4%, respectively. In addition, we present the case of a 49-year-old woman with a bladder IMT who underwent TURBT followed by laparoscopic partial cystectomy. The patient remains tumor free postoperatively (follow-up period of 12 months).
CONCLUSION
A complete surgical excision of the bladder IMT is crucial for the optimal management of these cases. Proper differentiation of this tumor from sarcoma or leiomyosarcoma leads to the best outcomes.
PubMed: 38725889
DOI: 10.4103/iju.iju_50_24 -
Oral Diseases Mar 2018In oral cancer, acquisition of α-smooth muscle actin (α-SMA)-positive fibroblasts, known as myofibroblasts or carcinoma-associated fibroblasts (CAF), is an important... (Review)
Review
In oral cancer, acquisition of α-smooth muscle actin (α-SMA)-positive fibroblasts, known as myofibroblasts or carcinoma-associated fibroblasts (CAF), is an important event for progression and metastasis. However, the contribution of myofibroblasts in oral potentially malignant disorders (OPMD) remains controversial. This systematic review provides evidence that immunodetection of myofibroblasts may identify oral submucous fibrosis (OSMF) with high risk of malignant transformation, but does not represent an auxiliary tool to predict the malignant potential of leukoplakia and erythroplakia, the most common OPMD.
Topics: Actins; Carcinoma, Squamous Cell; Cell Transformation, Neoplastic; Humans; Mouth Neoplasms; Myofibroblasts; Oral Submucous Fibrosis; Precancerous Conditions
PubMed: 29480603
DOI: 10.1111/odi.12694 -
Pulmonary Medicine 2012Interstitial lung diseases (ILDs) represent a heterogeneous group of more than two hundred diseases of either known or unknown etiology with different pathogenesis and...
Interstitial lung diseases (ILDs) represent a heterogeneous group of more than two hundred diseases of either known or unknown etiology with different pathogenesis and prognosis. Lung cancer, which is the major cause of cancer death in the developed countries, is mainly attributed to cigarette smoking and exposure to inhaled carcinogens. Different studies suggest a link between ILDs and lung cancer, through different pathogenetic mechanisms, such as inflammation, coagulation, dysregulated apoptosis, focal hypoxia, activation, and accumulation of myofibroblasts as well as extracellular matrix accumulation. This paper reviews current evidence on the association between lung cancer and interstitial lung diseases such as idiopathic pulmonary fibrosis, sarcoidosis, systemic sclerosis, dermatomyositis/polymyositis, rheumatoid arthritis, systemic lupus erythematosus, and pneumoconiosis.
PubMed: 22900168
DOI: 10.1155/2012/315918 -
Advances in Medical Sciences Mar 2022Inflammatory myofibroblastic tumors (IMTs) are rare intermediate-grade neoplasms that have a high recurrence rate after excision and exhibit low metastatic potential.... (Review)
Review
BACKGROUND
Inflammatory myofibroblastic tumors (IMTs) are rare intermediate-grade neoplasms that have a high recurrence rate after excision and exhibit low metastatic potential. These tumors contain proliferating neoplastic, fibroblastic and myofibroblastic cells, and are also characterized by chronic inflammatory infiltration by lymphocytes, plasma cells, eosinophils, and histiocytes. They belong to the group of inflammatory spindle cell lesions. Some reactive lesions, such as inflammatory pseudotumors, may appear to be IMTs, which makes their differential diagnosis extremely difficult. The aim of this article is to compile the recent information on IMTs to aid in their diagnosis and treatment.
METHODS
We reviewed articles published between 2017 and 2021, which were selected from online medical databases. In addition, some earlier articles and latest scientific monographies were analyzed.
RESULTS
The terminology used for inflammatory spindle cell lesions seems to be confusing. The terms "inflammatory myofibroblastic tumors" and "inflammatory pseudotumors" are interchangeably used by many scientists. However, a detailed analysis of the development of terminology suggests that the term "inflammatory myofibroblastic tumors" should be used to refer to a neoplastic lesion.
CONCLUSIONS
IMTs are rare neoplasms, which have not been investigated in detail due to the difficulty in collecting a large number of cases. Thus, our knowledge about this disease remains unsatisfactory. Recently developed techniques such as next-generation sequencing and computer-aided histopathological diagnosis may be useful in understanding the etiopathology of IMTs, which will help in the selection of the most appropriate therapy for patients.
Topics: Diagnosis, Differential; Granuloma, Plasma Cell; Humans; Inflammation; Myofibroblasts
PubMed: 35219201
DOI: 10.1016/j.advms.2022.02.002 -
The Laryngoscope Nov 2015Inflammatory pseudotumor (IPT) of the lateral skull base is a rare nonneoplastic inflammatory process of unknown cause often mistaken for malignancy or infection. This... (Review)
Review
OBJECTIVE
Inflammatory pseudotumor (IPT) of the lateral skull base is a rare nonneoplastic inflammatory process of unknown cause often mistaken for malignancy or infection. This systematic review aims to analyze all reported cases of lateral skull base IPT to date in order to provide insight into the management of this uncommon lesion.
DATA SOURCES
MEDLINE/PubMed database.
REVIEW METHODS
The MEDLINE/PubMed databases were searched for articles related to lateral skull base IPT. A bibliography review of the search results was then performed for additional articles. Demographics, presentation, radiographic findings, treatment, follow-up, and outcome were analyzed.
RESULTS
Thirty articles describing 39 patients were reviewed. The most common presenting symptom was hearing loss (53.8%). The mastoid bone had the highest incidence of IPT (61.5%). Computed tomography (CT) was the most utilized imaging modality. The lesion appeared isointense on T1-weighted magnetic resonance imaging (MRI) (75.0%) and hypointense on T2-weighted MRI (62.5%). Histopathological analysis showed fibrosis (96.9%) with inflammatory cell infiltration (100.0%). Surgical resection (alone or in combination with other treatments) was the most common treatment modality (92.3%), producing the greatest number of disease-free patients overall (96.0%). The mean follow-up time was 21.6 months, at which point 34.4% of patients showed improvement and 31.3% were completely disease-free.
CONCLUSION
This review is the most comprehensive analysis of lateral skull base IPT to date. A thorough workup including clinical exam, imaging, and biopsy is essential for diagnosis. Surgical excision is the most common and most successful treatment modality, followed by surgery with corticosteroids.
Topics: Bone Diseases; Earache; Granuloma, Plasma Cell; Hearing Loss; Humans; Magnetic Resonance Imaging; Skull Base; Tomography, X-Ray Computed
PubMed: 25892586
DOI: 10.1002/lary.25308