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Biomedicines ◽  
2021 ◽  
Vol 9 (11) ◽  
pp. 1545
Stephanie Arndt ◽  
Petra Unger ◽  
Anja-Katrin Bosserhoff ◽  
Mark Berneburg ◽  
Sigrid Karrer

Cold Atmospheric Plasma (CAP) has shown promising results in the treatment of various skin diseases. The therapeutic effect of CAP on localized scleroderma (LS), however, has not yet been evaluated. We investigated the effects of CAP on LS by comparing human normal fibroblasts (hNF), human TGF-β-activated fibroblasts (hAF), and human localized scleroderma-derived fibroblasts (hLSF) after direct CAP treatment, co-cultured with plasma-treated human epidermal keratinocytes (hEK) and with an experimental murine model of scleroderma. In hAF and hLSF, 2 min CAP treatment with the MicroPlaSterβ® plasma torch did not affect pro-fibrotic gene expression of alpha smooth muscle actin, fibroblast activating protein, and collagen type I, however, it promoted re-expression of matrix metalloproteinase 1. Functionally, CAP treatment reduced cell migration and stress fiber formation in hAF and hLSF. The relevance of CAP treatment was confirmed in an in vivo model of bleomycin-induced dermal fibrosis. In this model, CAP-treated mice showed significantly reduced dermal thickness and collagen deposition as well as a decrease in both alpha smooth muscle actin-positive myofibroblasts and CD68-positive macrophages in the affected skin in comparison to untreated fibrotic tissue. In conclusion, this study provides the first evidence for the successful use of CAP for treating LS and may be the basis for clinical trials including patients with LS.

PLoS ONE ◽  
2021 ◽  
Vol 16 (10) ◽  
pp. e0258802
Mohammad Alabduljabbar ◽  
Diego Strianese ◽  
Osama Al-Sheikh ◽  
Hind M. Alkatan ◽  
Hailah Al-Hussain ◽  

To evaluate and compare the clinical and histopathological profile of primary and recurrent orbital-periorbital plexiform neurofibromas (OPPN) in patients with neurofibromatosis type 1. We retrospectively evaluated 43 primary or recurrent neurofibroma (NF) specimens from 26 patients (2002 to 2018) at the King Khaled Eye Specialist Hospital, Saudi Arabia. Demographics, clinical presentation, and surgical intervention data were collected. Histopathological specimens were studied with hematoxylin-eosin, Alcian blue, and immunohistochemical markers; S-100, CD44, CD117, smooth muscle actin (SMA), neurofilament, and Ki-67. Of the 43 NFs specimens, 20 were primary and 23 recurrent tumors. For primary NF, the ratio of plexiform to the diffuse type was 13:7, however in recurrent tumors was 3:8 after the first recurrence, and 1:5 after multiple recurrences. Of the 17 patients with primary tumors that had paired recurrent tumors, 12/17 (70.6%) primary NFs were plexiform and 5/17 (29.4%) were diffuse. However, when tumors recurred, 13/17 tumors (76.5%) were diffuse and only 4/17 tumors (23.5%) had a plexiform pattern. The odds of a tumor having a diffuse pattern in recurrent NF was significantly higher than the plexiform pattern [OR = 7.8 (95% confidence interval 1.69:36.1) P = 0.008]. Primary plexiform NFs underwent an excision at a significantly younger age than the diffuse type. Recurrent NFs had significantly higher CD44, CD117, and neurofilament labeling (P = 0.02, P = 0.01 and P<0.001 respectively) but had significantly decreased Alcian blue, and S-100 labeling (P = 0.03, and P = 0.02 respectively) compared to primary tumors. SMA and Ki-67 proliferation index were not different between primary and recurrent NFs (P = 0.86, and P = 0.3 respectively). There appears to be a high risk for primary plexiform NFs to develop a diffuse histologic pattern when they recur. Immunohistochemical staining suggests a role of mast cells (CD117) and expression of infiltration makers (CD44) in the transformation of plexiform tumors to the diffuse phenotype.

Biomolecules ◽  
2021 ◽  
Vol 11 (10) ◽  
pp. 1547
Doris H. Rosero Salazar ◽  
René E. M. van Rheden ◽  
Manon van Hulzen ◽  
Paola L. Carvajal Monroy ◽  
Frank A. D. T. G. Wagener ◽  

This study aimed to analyze the effects of fibrin constructs enhanced with laminin-nidogen, implanted in the wounded rat soft palate. Fibrin constructs with and without laminin-nidogen were implanted in 1 mm excisional wounds in the soft palate of 9-week-old rats and compared with the wounded soft palate without implantation. Collagen deposition and myofiber formation were analyzed at days 3, 7, 28 and 56 after wounding by histochemistry. In addition, immune staining was performed for a-smooth muscle actin (a-SMA), myosin heavy chain (MyHC) and paired homeobox protein 7 (Pax7). At day 56, collagen areas were smaller in both implant groups (31.25 ± 7.73% fibrin only and 21.11 ± 6.06% fibrin with laminin-nidogen)) compared to the empty wounds (38.25 ± 8.89%, p < 0.05). Moreover, the collagen area in the fibrin with laminin-nidogen group was smaller than in the fibrin only group (p ˂ 0.05). The areas of myofiber formation in the fibrin only group (31.77 ± 10.81%) and fibrin with laminin-nidogen group (43.13 ± 10.39%) were larger than in the empty wounds (28.10 ± 11.68%, p ˂ 0.05). Fibrin-based constructs with laminin-nidogen reduce fibrosis and improve muscle regeneration in the wounded soft palate. This is a promising strategy to enhance cleft soft palate repair and other severe muscle injuries.

2021 ◽  
Vol 15 (1) ◽  
pp. 36-42
S. V. Lukyanov ◽  
K. M. Blikyan ◽  
S. S. Todorov ◽  
V. Y. Deribas ◽  
N. S. Lukyanov

Adrenal leiomyosarcoma — rare mesenchymal tumor, which is diagnosed most often after it reaches a large size. Primary adrenal leiomyosarcoma is extremely rare: in the English-language literature, only 45 cases were previously reported. We report the case of a 46-year-old man with the formation of the right adrenal gland measuring 12x8x7,5 cm. The patient underwent right-sided adrenalectomy, nephrectomy, and plastic of the inferior vena cava. The phenotype was confirmed by light microscopy and immunohistochemistry. Microscopic examination of the tumor is represented by fusiform and polymorphic cells that form multidirectional bundle structures with a high rate of mitosis. Immunohistochemically, tumor cells were positive for smooth muscle actin, CD-34, CD-117.

2021 ◽  
Xiao Wei ◽  
Attigah S. D. Kwasi ◽  
Qi Li ◽  
Hongyan Wu ◽  
Jun Chen ◽  

Abstract Background: Plexiform fibromyxoma (PF) is a very rare mesenchymal tumor of the stomach. Here we report one case of this unusual gastric tumor that was pathologically confirmed after endoscopic resection. Its clinical and pathologic features were observed while the relevant literature was reviewed. Case presentation: A 1.0 cm round elevated submucosal mass was discovered by gastroscopy in a 44-year-old Chinese woman due to recurrent abdominal pain. The tumor was characterized by a multinodular plexiform pattern, bland-looking oval to spindle cells, and a myxoid stroma with thin arborizing capillaries. Immunohistochemistry analysis revealed that the tumor cells were positive for smooth muscle actin (SMA) and negative for CD117, DOG-1, CD34, Desmin, progesterone receptor (PR), CD10, S100 and SOX10. A diagnosis of PF was rendered.Conclusion: Gastric PF is a benign tumor without evidence of local recurrence and distant metastasis. This case emphasizes the unique histological appearance and immunophenotype of PF to promote early diagnosis, while endoscopic resection can be used as an alternative treatment for small and superficial PF.

2021 ◽  
Vol 11 (1) ◽  
Giuseppina Abignano ◽  
Heidi Hermes ◽  
Sonsoles Piera-Velazquez ◽  
Sankar Addya ◽  
Francesco Del Galdo ◽  

AbstractMyofibroblasts are the key effector cells responsible for the exaggerated tissue fibrosis in Systemic Sclerosis (SSc). Despite their importance to SSc pathogenesis, the specific transcriptome of SSc myofibroblasts has not been described. The purpose of this study was to identify transcriptome differences between SSc myofibroblasts and non-myofibroblastic cells. Alpha smooth muscle actin (α-SMA) expressing myofibroblasts and α-SMA negative cells were isolated employing laser capture microdissection from dermal cell cultures from four patients with diffuse SSc of recent onset. Total mRNA was extracted from both cell populations, amplified and analyzed employing microarrays. Results for specific genes were validated by Western blots and by immunohistochemistry. Transcriptome analysis revealed 97 differentially expressed transcripts in SSc myofibroblasts compared with non-myofibroblasts. Annotation clustering of the SSc myofibroblast-specific transcripts failed to show a TGF-β signature. The most represented transcripts corresponded to several different genes from the Neuroblastoma Breakpoint Family (NBPF) of genes. NBPF genes are highly expanded in humans but are not present in murine or rat genomes. In vitro studies employing cultured SSc dermal fibroblasts and immunohistochemistry of affected SSc skin confirmed increased NBPF expression in SSc. These results indicate that SSc myofibroblasts represent a unique cell lineage expressing a specific transcriptome that includes very high levels of transcripts corresponding to numerous NBPF genes. Elevated expression of NBPF genes in SSc myofibroblasts suggests that NBPF gene products may play a role in SSc pathogenesis and may represent a novel therapeutic target.

2021 ◽  
pp. 109352662110469
Paola X. De la Iglesia Niveyro ◽  
J. Pandolfi ◽  
F. Jauk ◽  
T. Kreindel ◽  
P. Lobos

We present a 29-month-old male patient in follow-up due to pyelocaliceal dilation with a prostatic nodule incidentally found during ultrasound evaluation. Cysto video endoscopy was performed and a prostate biopsy, obtained. Microscopic evaluation showed a haphazardly distributed population of muscular cells with cross striations without evidence of mitosis or necrosis. Immunohistochemistry was positive for myogenin and desmin and negative for smooth muscle actin. Next generation sequencing was performed without finding any pathogenic variant or fusion in the tumor RNA. The patient received no further treatment, remained asymptomatic and continues in follow up, 3 years after initial diagnosis. We report a case of prostate rhabdomyoma in a toddler, an exceptional location that raises concern about differential diagnosis with its malignant counterpart, rhabdomyosarcoma, especially at this age.

2021 ◽  
Vol 11 (1) ◽  
Karoline B. Rypdal ◽  
Pugazendhi M. Erusappan ◽  
A. Olav Melleby ◽  
Deborah E. Seifert ◽  
Sheryl Palmero ◽  

AbstractFibrosis accompanies most heart diseases and is associated with adverse patient outcomes. Transforming growth factor (TGF)β drives extracellular matrix remodelling and fibrosis in the failing heart. Some members of the ADAMTSL (a disintegrin-like and metalloproteinase domain with thrombospondin type 1 motifs-like) family of secreted glycoproteins bind to matrix microfibrils, and although their function in the heart remains largely unknown, they are suggested to regulate TGFβ activity. The aims of this study were to determine ADAMTSL2 levels in failing hearts, and to elucidate the role of ADAMTSL2 in fibrosis using cultured human cardiac fibroblasts (CFBs). Cardiac ADAMTSL2 mRNA was robustly increased in human and experimental heart failure, and mainly expressed by fibroblasts. Over-expression and treatment with extracellular ADAMTSL2 in human CFBs led to reduced TGFβ production and signalling. Increased ADAMTSL2 attenuated myofibroblast differentiation, with reduced expression of the signature molecules α-smooth muscle actin and osteopontin. Finally, ADAMTSL2 mitigated the pro-fibrotic CFB phenotypes, proliferation, migration and contractility. In conclusion, the extracellular matrix-localized glycoprotein ADAMTSL2 was upregulated in fibrotic and failing hearts of patients and mice. We identified ADAMTSL2 as a negative regulator of TGFβ in human cardiac fibroblasts, inhibiting myofibroblast differentiation and pro-fibrotic properties.

2021 ◽  
Vol 14 (10) ◽  
pp. e244800
Ruchi Goel ◽  
Ayushi Agarwal ◽  
Shweta Raghav ◽  
Ravindra Kumar Saran

A 16-year-old woman presented with a painless, progressive, hard swelling in the left inferolateral orbital wall for the past 1 year. There was no diminution of vision or limitation of ocular motility. Imaging revealed an intraosseous, well-defined, expansile, soft tissue lesion in the inferolateral wall of the left orbit. A left anterior orbitotomy with complete surgical excision was performed. Histopathological evaluation of the specimen revealed fascicular pattern of spindle cells with a rich network of slit-like, branching blood vessels. Tumour cells exhibited smooth muscle actin and vimentin positivity but were negative for CD-34 and STAT-6. In absence of any systemic manifestation, a diagnosis of intraosseous solitary orbital myofibroma was made. The case highlights the importance of integrating clinical, radiological and histopathological features in overcoming the diagnostic challenge of differentiating myofibroma from other mesenchymal neoplasms. It also brings forth the importance of complete resection and curettage to prevent recurrence.

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