Clinical Impact of Connexin 43 Deregulation on Myocardial Infraction.

INTRODUCTION: Coronary artery disease (CAD) is a major and multifaceted health problem but also the first cause of death in modern Western societies. Furthermore, myocardial infarction (MI) constitutes a challenge for analysis in the field of molecular mechanisms, early diagnosis and therapeutic approaches, as its incidence increases every year worldwide. Concerning the histopathological diagnosis in the corresponding cases, a variety of immunohistochemistry (IHC) markers and methods are available to support conventional histology diagnosis. Immunohistochemistry techniques are effective for use in forensic pathology, expanding the limits of differential diagnoses in borderline cases, as they can be applied to tissue samples fixed in formalin and embedded in paraffin. OBJECTIVE: The purpose of the current review was to explore the role of connexin 43 (gene locus: 6q22.31) as a reliable biomarker of myocardial disease/infarction and its impact on MI pathology. MATERIAL AND METHOD: A systematic review of the literature was carried out based on the international database PubMed. The majority of medical data referred to articles published after the year 2020, whereas specific references of great importance and value were also included. The following keywords were used: coronary, artery, myocardial, infarction, connexin and immunohistochemistry. RESULTS: A pool of 38 significant articles focused on the mechanisms and novel experimental biomarkers was selected for the present study at the basis of combining molecular knowledge with new clinical features in CAD, and MI histodiagnosis. CONCLUSIONS: The role of connexin 43 - as a significant gap junction intermediate protein - in MI pathology, clinical symptoms and prognosis is critical because its dysfunction is involved in myocardial conduction and the onset of ventricular arrhythmias due to a crucial interruption of the intra-cardiomyocyte's conjunction.

The Impact of Expert Pathology Review and Molecular Diagnostics on the Management of Sarcoma Patients: A Prospective Study of the Hellenic Group of Sarcomas and Rare Cancers.

Precise classification of sarcomas is crucial to optimal clinical management. In this prospective, multicenter, observational study within the Hellenic Group of Sarcoma and Rare Cancers (HGSRC), we assessed the effect of expert pathology review, coupled with the application of molecular diagnostics, on the diagnosis and management of sarcoma patients. Newly diagnosed sarcoma patients were addressed by their physicians to one of the two sarcoma pathologists of HGSRC for histopathological diagnostic assessment. RNA next-generation sequencing was performed on all samples using a platform targeting 86 sarcoma gene fusions. Additional molecular methods were performed in the opinion of the expert pathologist. Therefore, the expert pathologist provided a final diagnosis based on the histopathological findings and, when necessary, molecular tests. In total, 128 specimens from 122 patients were assessed. Among the 119 cases in which there was a preliminary diagnosis by a non-sarcoma pathologist, there were 37 modifications in diagnosis (31.1%) by the sarcoma pathologist, resulting in 17 (14.2%) modifications in management. Among the 110 cases in which molecular tests were performed, there were 29 modifications in diagnosis (26.4%) through the genomic results, resulting in 12 (10.9%) modifications in management. Our study confirms that expert pathology review is of utmost importance for optimal sarcoma diagnosis and management and should be assisted by molecular methods in selected cases.

Anti-PEc: Development of a novel monoclonal antibody against prostate cancer.

BACKGROUND: The Ec peptide (PEc) that defines the IGF-1Ec isoform, is associated with prostate cancer progression by inducing proliferation, metastases, and tumour repair. On these grounds, an anti-PEc monoclonal antibody (MAb) was developed. Our objective is to examine the effects of this antibody on prostate cancer and its possible side effects. METHODS: The effects of the obtained MAb were examined in cancer and non-cancerous cell lines (unmodified and modified either to overexpress or silence PEc) and in tumours in SCID mice injected with unmodified prostate cancer cells. The investigation was obtained with respect to cellular proliferation, migration, invasion, toxicity to tumours, effects on the cell cycle, immune response activation, effects on mesenchymal stem cell mobilisation leading to tumour repair, tissue distribution, and toxicity to mice. RESULTS: Anti-PEc MAb treatment led to a significant decrease in cellular proliferation, migration, and invasion compared to the untreated cell lines (p < 0.0005 in every case). Mechanistically, these effects were associated with the downregulation of pERK1/2 and vimentin and the upregulation of E-Cadherin. In vivo, anti-PEc MAb treatment was associated with a significant decrease in tumour size and metastases rate (p < 0.0005 in every case) by reversing the tumours mesenchymal phenotype. It also inhibited host stem cell mobilisation towards the tumour, leading to apoptosis. Anti-PEc MAb assessment in respect to distribution and toxicity, indicated its tumour specificity and lack of toxicity. CONCLUSIONS: These data indicate that the therapeutic targeting of PEc with the anti-PEc MAb may have considerable clinical benefit for prostate cancer patients.

The Immunohistochemical Expression of REV-7 in Various Human Cancer Pathology Specimens: A Systematic Review.

The purpose of this systematic review is to summarize all existing evidence, regarding the immunohistochemical expression of REV-7 in different human cancer pathology specimens. Moreover, the association of REV-7 expression with disease severity (clinical course), patients' survival, prognosis, and response to various treatments, such as chemotherapy and irradiation, was investigated. Three databases (PubMed, Scopus, and Cochrane) were systematically screened, from inception to September 2, 2023, as suggested by the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. Only studies using immunohistochemical staining for REV-7 in paraffin-embedded cancer tissues were included. Nine studies met the inclusion criteria and were included in the final qualitative synthesis. All nine studies were retrospective and non-comparative ones. Selected studies reported immunohistochemical expression of REV-7 in different types of cancer, including testicular cancer, ovarian cancer, esophagus squamous cell carcinoma, prostate cancer, colorectal cancer, diffuse large B-cell lymphoma, breast cancer, lung cancer, and skin cancer. High REV-7 expression was associated with faster disease progression, resistance to available treatment options, and worse prognosis in the majority of included studies. These results indicate that immunohistochemical staining of REV-7 protein could potentially be used as a predictive tissue marker in certain cases. Promising results, arising from REV-7 inactivation experiments, render REV-7 targeting a potential therapeutic strategy for future cancer management, especially in the cases of chemoresistant or radioresistant disease.

Predictive tissue markers in testicular germ cell tumors: Immunohistochemical expression of MLH1 and REV-7 proteins.

PURPOSE: Testicular Germ Cell Tumors (TGCTs) are the most frequent solid malignancies in young adult men. Regardless of differences in their cell of origin, all TGCTs are considered highly curable malignancies. However, approximately 3-5% of all TGCTs do not respond to platinum-based chemotherapies. The purpose of our paper is to investigate whether immunohistochemical expression of MLH1 and REV-7 can be used as predictive tissue markers for TGCTs. MATERIAL AND METHODS: The main demographic and clinicopathological characteristics of 64 male patients with TGCTs who underwent orchiectomy from 2007 to 2022 were retrospectively obtained from two large Oncology Clinics in Greece. Both patients with chemosensitive and chemoresistant disease were included. Immunohistochemical staining for MLH1 and REV-7 proteins was applied in specimens of these patients. RESULTS: 31 seminomas and 33 non-seminomas were included. 48 patients had chemosensitive disease, while 16 had chemoresistant disease. 53 specimens showed preserved MLH1 expression, while 11 specimens had lost MLH1 expression. Expression of MLH1 was only significantly associated with patients' age. 16 specimens showed positive REV-7 expression, while 48 specimens were REV-7 negative. Interestingly, 50% of patients with chemoresistant disease and 16,7% of patients with chemosensitive disease were REV-7 positive. This difference was statistically significant. Moreover, REV-7 positivity was significantly associated with chemoresistance, various clinicopathological parameters and patients' prognosis and survival. CONCLUSION: Loss of MLH1 expression was only found to be significantly associated with lower patients' age. Positive immunohistochemical REV-7 expression was significantly associated with various clinicopathological parameters, while it was also associated with significantly lower survival and greater hazard. REV-7 positive percentages were significantly higher in patients with chemoresistant disease. Our findings imply that immunohistochemical staining for REV-7 could potentially be used as a predictive tissue marker for TGCT tumors. Moreover, targeting of REV-7 protein, could represent a potential therapeutic strategy for chemoresistant TGCT cases. The implementation of well-designed studies on a larger scale is of utmost importance, in order to draw safer conclusions. Additional studies are needed so as to draw safer conclusions.

Evaluation of Vitamin D-enriched Bone Graft in Surgically-induced Critical-sized Bone Defects: An Experimental Study.

BACKGROUND: Restoration of bone defects in the craniac vault may require the use of autografts, allografts, xenografts, or synthetic grafts. There are promising data that vitamin D may play a positive role in graft incorporation. The purpose of the present study is the evaluation of the impact of vitamin D addition to human-derived bone grafts in the healing of critical-sized bone defects in porcine skulls. MATERIALS AND METHODS: Four identical critical-sized defects were created in the calvaria of 8 adult Landrace Large White pigs. The first defect was left blank as control, the second defect was filled with human-derived bone graft, the third defect was filled with human-derived bone graft enriched with a low concentration of vitamin D (2 mg/mL), and the fourth defect was filled with human-derived bone graft enriched with a high concentration of vitamin D (10 mg/mL). The animals were sacrificed after 12 weeks. Harvested tissue specimens were qualitatively evaluated by histology. New bone formation (bone volume/tissue volume) was quantitatively measured by histomorphometry. RESULTS: Signs of bone formation were evident in all bone sockets. Mean values of the bone volume/tissue volume of the 4 defects were 10.91%, 11.05%, 10.40% and 10.87% respectively, at 12 weeks. In 5 animals, high concentration of vitamin D caused a significant improvement in bone formation in relation to controls. In 3 animals, a high concentration of vitamin D was associated with decreased bone formation compared with controls. No statistical difference was observed in the graft healing among the 4 graft sites ( P > 0.05). CONCLUSIONS: The results of this study have shown that the addition of vitamin D to human-derived bone grafts does not have a significant effect on bone formation and graft incorporation in critical-sized bone defects of the porcine calvaria. Further high-quality studies are needed to fully elucidate the role of vitamin D in bone formation and bone graft union.

Primary immature teratoma of the thigh: a review.

Teratomas are a subtype of germ cell tumors composed of a variety of somatic tissues derived from more than one of the three germinal layers (ectoderm, endoderm and mesoderm). They can be classified as mature tumors and immature tumors. Teratomas most commonly arise at the sacrococcygeal region and the gonads. The occurrence of a teratoma outside the common gonadal and midline locations is exceptional. This review article lists the reported primary and metastatic malignant teratomas in extragonadal locations and discusses the possible explanation for the atypical location, their treatment and prognosis.

Primary Synovial Sarcoma of the Kidney: Diagnostic Approach and Therapeutic Modalities for a Rare Nosological Entity.

Synovial sarcoma (SS) is a rare mesenchymal entity that represents 5-10% among soft tissue sarcomas (STS). Primary renal synovial sarcoma (PRSS) is an uncommon, rapidly growing tumor, with potential metastatic dissemination. The main prognostic factors of PRSS include tumor size and histologic grade, while translocation t (X; 18) (p11.2; q11.2) (fusion of SYT gene -chromosome 18- with SSX genes (1, 2 or 4)-chromosome X) is the most common pathognomonic sign. Aggressive surgical resection of the tumor along with concomitant regional lymphadenectomy is the treatment of choice for PRSS, while additional en bloc resection of the adjacent affected organs is often performed. To date, the role of preoperative or postoperative chemotherapy remains equivocal. The prognosis of patients with PRSS is poor, as the 5-year survival rate is only 20-30% and further deteriorates when a high mitotic activity is detected. Local recurrence even after complete R0 surgical excision remains the most frequent cause of death. The aim of this review was to meticulously discuss clinical features, histogenesis, and morphological and immunochemical findings of PRSS, while the role of current diagnostic and therapeutic management of this aggressive neoplasm was emphasized.

Evaluating e-Learning in the Pathology Course During the COVID-19 Pandemic.

Purpose: Synchronous and asynchronous e-learning is a promising and effective educational method for the delivery of medical lessons. Due to the public health measures implemented during the COVID-19 pandemic, our Pathology Department faced the challenge of a total online transition of the lessons. Therefore, the aim is to evaluate the aspects of the applied e-learning method they received. Materials and Methods: At the end of the semester when e-learning was applied, we designed a structured questionnaire consisting of 17 items via Google Forms, which took the students between 5 and 7 minutes to complete. Of the 257 students registered on the Pathology course in the fifth semester, 207 students (80.5%) returned completed valid questionnaires. Results: Fifteen of the seventeen components of the e-learning Pathology questionnaire were evaluated highly by the vast majority of the students. The two remaining items, the HIPON platform and the Microlabs e-lessons, were evaluated highly by almost half the students. Approximately 93% of medical students answered that e-learning could be integrated with real class lessons in the medical curriculum: 62.8% (N = 130) of students answered to a great extent, and 30.4% (N = 63) answered to a small extent. Statistically significant differences were found between the demographic characteristics of the participants (gender, permanent residence, working status) and their evaluation of the e-learning items. Conclusion: E-learning was successfully implemented for the delivery of the pathology lessons and was widely accepted by the students, providing evidence for its future integration into the medical curriculum. Our findings illuminate various aspects of the students' experience with e-learning, and we strongly recommend that the students' evaluation and perspective be taken into consideration by the faculty in the development of policies for higher-quality medical education.

Polycystin-1 regulates cell proliferation and migration through AKT/mTORC2 pathway in a human craniosynostosis cell model.

Craniosynostosis is the premature fusion of skull sutures and has a severe pathological impact on childrens' life. Mechanical forces are capable of triggering biological responses in bone cells and regulate osteoblastogenesis in cranial sutures, leading to premature closure. The mechanosensitive proteins polycystin-1 (PC1) and polycystin-2 (PC2) have been documented to play an important role in craniofacial proliferation and development. Herein, we investigated the contribution of PC1 to the pathogenesis of non-syndromic craniosynostosis and the associated molecular mechanisms. Protein expression of PC1 and PC2 was detected in bone fragments derived from craniosynostosis patients via immunohistochemistry. To explore the modulatory role of PC1 in primary cranial suture cells, we further abrogated the function of PC1 extracellular mechanosensing domain using a specific anti-PC1 IgPKD1 antibody. Effect of IgPKD1 treatment was evaluated with cell proliferation and migration assays. Activation of PI3K/AKT/mTOR pathway components was further detected via Western blot in primary cranial suture cells following IgPKD1 treatment. PC1 and PC2 are expressed in human tissues of craniosynostosis. PC1 functional inhibition resulted in elevated proliferation and migration of primary cranial suture cells. PC1 inhibition also induced activation of AKT, exhibiting elevated phospho (p)-AKT (Ser473) levels, but not 4EBP1 or p70S6K activation. Our findings indicate that PC1 may act as a mechanosensing molecule in cranial sutures by modulating osteoblastic cell proliferation and migration through the PC1/AKT/mTORC2 cascade with a potential impact on the development of non-syndromic craniosynostosis.

Giant Sclerosing Epithelioid Fibrosarcoma of the Thigh: A Case Report.

CASE: We present a case of a 35-year-old-man with a giant sclerosing epithelioid fibrosarcoma (SEF) of the thigh. The patient presented with a history of a painful thigh mass. Plain radiographs revealed a soft-tissue mass with extensive calcifications, whereas on magnetic resonance imaging, a lobulated mass between the adductors and the posterior muscles of the thigh was noted. A wide en block resection was undertaken, and the histopathology confirmed the diagnosis of SEF. Postoperative radiation therapy was followed. The patient had no signs of recurrence at the 4-year follow-up. CONCLUSION: The clinicopathological, imaging characteristics, and treatment options of this rare soft-tissue tumor are discussed.

c-Fos/ c-Jun transcription factors in non-small cell lung carcinoma.

During lung carcinoma development, progression and metastasis, a variety of gross (chromosome) and specific (gene) genomic alterations are detected in dysplastic, neoplastic, and progressively malignant transformed epithelia as early or late genetic events. Oncogenes' overactivation combined with suppressor genes silence are crucial genetic events in malignant and pre-malignant epithelia. Especially, deregulation of crucial signalling transduction pathways that interact with strong transcription factors - such as c-Fos and c-Jun - leads to an aberrant expression of other critical genes responsible for cell homeostasis. Upregulation of c-Fos and c-Jun leading to other oncogenes overactivation seems to be correlated with aggressive biological behaviour in non-small cell lung carcinomas (NSCLCs). In the current special molecular article we explored the role of c-Fos/c-Jun complex deregulation in NSCLC based on their interactions with other genes that demonstrate modified expression profiles.

Osseous Healing in Surgically Prepared Bone Defects Using Different Grafting Materials: An Experimental Study in Pigs.

Regeneration of large jaw bone defects still remains a clinical challenge. To avoid incomplete bone repair, bone grafts have been advocated to support the healing process. This study comparatively evaluated new bone formation among a synthetic graft substitute, a human bone derivative, and a bovine xenograft. Materials were placed in 3 out of the 4 bone cavities, while 1 deficit was left empty, serving as a control, in mono-cortical defects, surgically prepared in the porcine calvaria bone. Animals were randomized in 2 groups and euthanized at 8 and 12 weeks. Harvested tissue specimens were qualitatively evaluated by histology. New bone formation was quantitatively measured by histomorphometry. Maximum new bone formation was noticed in defects grafted with beta-tricalcium phosphate b-TCP compared to the other bone substitutes, at 8 and 12 weeks post-surgery. Bovine and human allograft induced less new bone formation compared to empty bone cavity. Histologic analysis revealed that b-TCP was absorbed and substituted significantly, while bovine and human allograft was maintained almost intact in close proximity with new bone. Based on our findings, higher new bone formation was detected in defects filled with b-TCP when compared to bovine and human graft substitutes.

The effect of experimental periodontitis, experimental diabetes and their combination on the serum levels of adiponectin, leptin, IL-6, IL-18, MCP-1, RANTES and sICAM-1 in rats.

AIM: Periodontitis is often associated with diabetes mellitus and may be considered one of the chronic complications of this disease. Increasing evidence indicates that periodontal disease (gingivitis and periodontitis) has an adverse effect on glycemic control and participates in the pathophysiology of complications related to type 2 diabetes mellitus. Thus, this study aimed to evaluate the influence of obesity on clinical periodontal parameters of patients with type 2 diabetes mellitus with stage II or III periodontitis grade C after conventional periodontal treatment. METHODS: For this study, 36 patients, aged 25 to 65 years, were evaluated; 20 patients with type 2 diabetes mellitus and moderate to severe periodontitis (Non-Obese Group) and 16 patients with type 2 diabetes mellitus with obesity and moderate to severe periodontitis (Obese Group). These patients underwent conventional periodontal treatment and were evaluated using plaque index, probing depth, clinical attachment level, bleeding on probing and gingival crevicular fluid analysis, as well as laboratory tests of glycated hemoglobin, fasting glycemia, total cholesterol, and fractions of triglycerides. Periodontal and laboratory parameters were evaluated at baselineand six months. RESULTS: The results showed improvements in periodontal and clinical laboratory parameters(p less than 0.05) in the evaluated periods; however, the non-obese group presented significantly better results when compared to the obese group. CONCLUSION: It can be concluded that the presence of obesity may hinder the improvement of periodontal clinical parameters after conventional periodontal treatment in patients with diabetes mellitus and periodontitis.

A Clinicopathological Analysis of S100A14 Expression in Colorectal Cancer.

BACKGROUND/AIM: The calcium-binding protein S100A14 is involved in processes related to tumorigenesis and tumor propagation, such as proliferation, apoptosis, motility and invasiveness. Our aim was to investigate its role in colorectal cancer. PATIENTS AND METHODS: One hundred and seven patients (65 men and 42 women) were included in this study. They had been diagnosed with colorectal cancer and undergone complete resection of their primary tumor. Tissue samples from archival blocks of their normal and malignant colorectal tissues were used for immunohistochemical assessment of S100A14 expression. S100A14 levels were evaluated using image analysis and associated with various clinicopathological parameters and prognosis. RESULTS: S100A14 expression was reduced in malignant tissues when compared to normal intestinal mucosa in cases of T3-T4 tumors (p=0.017). Moreover, as far as S100A14 levels in malignant tissues are concerned, they were lower in T3-T4 tumors (p=0.001), N2 disease (p=0.034) and M1 disease (p=0.019). Finally, very high S100A14 production (>75th percentile) was associated with shorter disease-specific (HR=3.584, p=0.045) and relapse-free survival (HR=4.527, p=0.007) in multivariate survival analysis. CONCLUSION: S100A14 expression is decreased in advanced colorectal cancer. However, cases with very high S100A14 levels have a worse survival.

CRH Promotes the Neurogenic Activity of Neural Stem Cells in the Adult Hippocampus.

Local cues in the adult neurogenic niches dynamically regulate homeostasis in neural stem cells, whereas their identity and associated molecular mechanisms remain poorly understood. Here, we show that corticotropin-releasing hormone (CRH), the major mediator of mammalian stress response and a key neuromodulator in the adult brain, is necessary for hippocampal neural stem cell (hiNSC) activity under physiological conditions. In particular, we demonstrate functionality of the CRH/CRH receptor (CRHR) system in mouse hiNSCs and conserved expression in humans. Most important, we show that genetic deficiency of CRH impairs hippocampal neurogenesis, affects spatial memory, and compromises hiNSCs' responsiveness to environmental stimuli. These deficits have been partially restored by virus-mediated CRH expression. Additionally, we provide evidence that local disruption of the CRH/CRHR system reduces neurogenesis, while exposure of adult hiNSCs to CRH promotes neurogenic activity via BMP4 suppression. Our findings suggest a critical role of CRH in adult neurogenesis, independently of its stress-related systemic function.

Lazaroid U-74389G in liver ischemia-reperfusion injury: A swine model.

Reactive oxygen species have a key role in liver ischemia-reperfusion (I/R) injury. In the present study, the effect of the anti-oxidant compound lazaroid U-74389G in preventing liver I/R injury was investigated in a swine model. Ischemia was produced by portal vein occlusion. Two sets of experiments were performed, each with two groups (n=7 per group). In the first group, the potential protective effect of an intracaval injection of U-74389G after a 30-min ischemia, followed by a 60-min reperfusion period was assessed (biopsies at 0, 15, 30 and 90 min experimental time). In the second set, the effect of intracaval U-74389G injection after 30 min of ischemia, followed by a longer reperfusion period of 120 min was determined (biopsies at 0, 15, 30 and 150 min experimental time). Liver malondialdehyde, hepatocyte vacuolation-degeneration, venous congestion, inflammatory cell infiltration, sinus congestion-dilation and Chiu score of intestinal damage were determined at up to 150 min of reperfusion. In the second set of experiments, the Chiu score of intestinal damage was improved by the administration of U-74389G (3.17±0.40 vs. 4.33±0.21; P=0.030). However, in the two sets of experiments, the liver inflammatory reaction was more pronounced in the U-74389G groups (P=0.017 for the first set, P=0.021 for the second set). No significant effect of U-74389G on any other parameters was detected. In conclusion, intestinal damage due to portal venous congestion and reflow appears to be mitigated by the lazaroid U-74389G; however, intracaval administration of U-74389G does not appear to exert any protective effects against liver I/R-induced inflammation.

Recurrent aggressive mesenteric desmoid tumor successfully treated with sorafenib: A case report and literature review.

BACKGROUND: Desmoid tumors (DT) are locally advanced but histologically benign monoclonal neoplasms that can occur from any musculoaponeurotic structure. The aim of this report is to analyze a rare clinical case of an aggressive intra-abdominal DT successfully treated with sorafenib. CASE SUMMARY: A 36-year-old man presented with increasing colicky abdominal pain and a self-palpable mass in his left abdomen. Fourteen years earlier he was diagnosed with a large intra-abdominal tumor, which adhered to the left colonic flexure, part of the major gastric curvature and the spleen. Subsequent exploratory laparotomy revealed a voluminous mass in the epigastrium, arising from the posterior surface of the stomach and invading the superior mesenteric vessels, transverse mesocolon and the small bowel mesentery. As the tumor was unresectable, a jejunojejunal bypass was performed. Traditional therapeutic interventions proved insufficient, and the patient was started on sorafenib with a subsequent full-disease response. CONCLUSION: DT's pathogenesis has been associated with mutations in the adenomatous polyposis coli (APC) gene or beta-catenin gene CTNNB1, sex steroids or previous surgical trauma. Local treatment modalities, such as surgery or radiotherapy, are implemented in aggressively progressing or symptomatic patients. Sorafenib is a hopeful therapeutic option against DTs, while several pharmacological agents have been successfully used.

Management of primary retroperitoneal synovial sarcoma: A case report and review of literature.

BACKGROUND: Synovial sarcoma (SS) is a rare type of soft tissue sarcoma that is usually developed from areas where synovial tissue exists, especially at the extremities. Nevertheless, several cases of retroperitoneal SS (RSS) have been described. We herein report a case of RSS presented in our institution. CASE SUMMARY: A 69-year-old female patient was admitted with a large, palpable, firm mass in the right abdominal space SS. Computerized tomography scan depicted a concentric, sharply marinated retro-peritoneal lesion which was displacing the right kidney and the lower edge of the liver. Subsequently, the patient underwent surgical excision of the mass with additional right nephrectomy and resection of the right adrenal gland and a part of the diaphragm. The final histological diagnosis of the tumour was grade II monophasic RSS. CONCLUSION: RSS is encountered in the biphasic type, the monophasic fibrous, and the monophasic epithelial category as well. Relevant clinical manifestations are not always documented at early stages. Therefore, the final diagnosis is posed after complete histological examination taking into consideration the results of immunochemistry and genetic analysis. Therapeutic approach happens often late when metastases at the lungs and the liver are apparent. Thus, 5-year survival rates remain low.