Tumor cell-derived complement components C1r and C1s promote growth of cutaneous squamous cell carcinoma.

CONCLUSIONS: These results provide evidence for the role of tumor cell-derived C1r and C1s in the progression of cSCC and identify them as biomarkers and putative therapeutic targets in cSCC. This article is protected by copyright. All rights reserved. PMID: 31049937 [PubMed - as supplied by publisher]
Source: The British Journal of Dermatology - Category: Dermatology Authors: Tags: Br J Dermatol Source Type: research

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Squamous cell carcinoma (SCC) develops in more than 80% of individuals with the skin blistering disorder recessive dystrophic epidermolysis bullosa (RDEB). In contrast with UV-induced SCC, RDEB SCC has a high proliferative and metastatic rate, with five-year survival near zero. RDEB dermal fibroblasts were found with higher expression of the myofibroblast markers α-smooth muscle actin (6/8 pairs), Fibronectin (7/8 pairs), and periostin (7/8 pairs) compared to matched donors. RDEB fibroblasts were also found to have higher expression of MCP-1 (3/4 pairs) and TGFβ1 (3/4 pairs), indicative of increased TGFβ1 secretion.
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Carcinogenesis and Cancer Genetics Source Type: research
Recessive Dystrophic Epidermolysis Bullosa (RDEB) is a devastating skin blistering disease for which there is still no available cure. Although RDEB is characterized by blisters caused by minimal trauma, non-healing wounds, recurrent infections, pseudosyndactyly, squamous cell carcinoma and others, one of the most challenging symptoms to treat in these patients is fibrosis. Healing takes place with an obvious scarring phenotype and RDEB can be viewed as a fibrotic disorder characterized by excessive ECM deposition, accumulation of collagen fibers, increase tissue stiffness and TGF β signaling.
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Genetic Disease, Gene Regulation, and Gene Therapy Source Type: research
Dystrophic epidermolysis bullosa (DEB) is a one of the most severe form of EB caused by mutations in COL7A1 (coding type VII collagen). DEB patients, without functional type VII collagen, suffer from the repetitive blistering and have high risk of early-onset aggressive squamous cell carcinoma. Recently, we found that a fragment of HMGB1 activates an endogenous tissue regeneration mechanism and ameliorates the DEB related manifestations in a DEB model mouse. This HMGB1 treatment model in the DEB mouse serves as a unique opportunity to investigate on how the skin can lose the integrity by loss of a single protein and how th...
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Tissue Regeneration and Wound Healing Source Type: research
The high frequency and extremely aggressive behavior of squamous cell carcinoma from recessive dystrophic epidermolysis bullosa patients (RDEB-cSCC), as well as the lack of an appropriate preclinical mouse model recapitulating the RDEB-cSCC phenotype for testing effective therapies/drugs, creates an urgent need for generating such a model. Here, we established a novel mouse model for RDEB-cSCC, in which we generated 3D cSCC skin constructs (SCs) using RDEB-cSCC and RDEB fibroblasts (FB), which were then grafted onto immunocompromised mice.
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Translational Studies Source Type: research
ConclusionscSCC is a life-threatening complication of RDEB patients. Although tumors are usually well differentiated, they tend to relapse. This is the first Spanish report of cSCC arising in RDEB patients.
Source: Clinical and Translational Oncology - Category: Cancer & Oncology Source Type: research
CONCLUSIONS: These data support a "first in RDEB" phase II clinical trial of rigosertib to assess tumor targeting in patients with late stage, metastatic and/ or unresectable SCC. PMID: 30846478 [PubMed - as supplied by publisher]
Source: Clinical Cancer Research - Category: Cancer & Oncology Authors: Tags: Clin Cancer Res Source Type: research
Patients with epidermolysis bullosa (EB) require care of wounds that are often colonized with bacteria. A subset of EB patients are at risk for squamous cell carcinoma (SCC) and certain bacterial-host interactions have been implicated in this risk. The EB Clinical Characterization and Outcomes Database serves as a repository of information from EB patients at multiple centers in the US and Canada. Using this resource, we conducted a retrospective analysis of 739 wound culture results reported from 159 patients between 2001 and 2017 in the Research Electronic Data Capture Research Electronic Data Capture registry.
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Genetic Disease, Gene Regulation and Gene Therapy Source Type: research
Recessive dystrophic epidermolysis bullosa (RDEB) is caused by loss-of-function mutations in COL7A1 encoding type VII collagen (C7) that forms anchoring fibrils (AFs), structures essential for dermal-epidermal adherence (Uitto et  al., 2017). Patients with RDEB suffer from skin and mucosal blistering and develop severe complications including invasive squamous cell carcinoma, resulting in a poor prognosis (Guerra et al., 2017). Different therapeutic strategies have been explored, including gene-, protein-, cell-based, and pharmacological therapies that have shown promising preclinical or transitory clinical ...
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Letter to the Editor Source Type: research
Recessive dystrophic epidermolysis bullosa (RDEB) is a severe inherited skin disease characterized by epidermal fragility leading to chronic wounds (Fine et  al., 2014). RDEB is complicated by other pathologies such as esophageal strictures, anemia, mitten deformity, and corneal abrasion, but poor wound healing has arguably the greatest impact on quality of life and ultimately leads to the development of highly aggressive squamous cell carcinoma (Fine et al., 2009). One of the defining features of RDEB wounds is that when they do heal, they do so with evident scarring.
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Letter to the Editor Source Type: research
Recessive dystrophic epidermolysis bullosa (RDEB) is a skin blistering disease caused by mutations in the COL7A1 gene. RDEB patients experience severe blistering and fragility of the skin and mucous membranes, leading to pseudosyndactyly, susceptibility to infections, esophageal strictures, and aggressive cutaneous squamous cell carcinoma (SCC), which accounts for more than two thirds of RDEB patient deaths. RDEB patients can have multiple primary tumors and these tumors develop and metastasize much more quickly than in the general population.
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Carcinogenesis and Cancer Genetics Source Type: research
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