The receptor for advanced glycation end products (RAGE) is a non-specific multi-ligand pattern recognition receptor capable of binding to a range of structurally diverse ligands, expressed on a variety of cell types, and performing different functions. The ligand-RAGE axis can trigger a range of signaling events that are associated with diabetes and its complications, neurological disorders, cancer, inflammation and other diseases. Since RAGE is involved in the pathophysiological processes of many diseases, targeting RAGE may be an effective strategy to block RAGE signaling.

/pdf/pathophysiology-of-rage-in-inflammatory-diseases-2tmrjqpy.pdf

Pathophysiology of RAGE in inflammatory diseases

C34 Introduction: Gastrointestinal stromal tumors (GIST) involving the duodenum are rarely reported. The present surgical opinions is divided regarding the optimal method for the resection of duodenal GIST with some investigators supporting the selective use of limited resection (LR) versus others who prefer pancreaticoduodenectomy (PD).  Methods: A retrospective review of 22 patients who underwent surgery for a GIST or suspected GIST involving the duodenum at a single institution was performed. Comparison was made between GIST versus other non-GIST benign submucosal tumors and between results of LR versus PD.  Results: There were 15 GISTs, 1 leiomyosarcoma and 6 other non-GIST benign submucosal tumors. 20 patients underwent curative resection including 7 PDs and 13 LRs. Surgery with palliative intent was performed in 2 patients with advanced duodenal leiomyosarcoma and GIST respectively. All 7 PDs were performed for GIST and all 6 patients with benign non-GIST submucosal tumors underwent LR. Comparison between the clinicopathological features of GIST and other benign tumors demonstrated that patient demographics and clinical presentation were similar and that size was the only statistically significant distinguishing factor. Comparison between LR versus PD demonstrated that LR was associated with a significantly shorter operation time but both had similar morbidity and mortality rates. Seven patients with GIST underwent LR and 7 underwent PD. The median follow-up of the 14 patients was 42 (range, 2-174) months and all were alive at follow-up. The long-term outcomes of patients who underwent LR versus PD were similar in terms of disease-specific specific survival and recurrence rates. Two patients (1 moderate risk and 1 high risk GIST) who underwent PD and 1 patient (high risk GIST) who underwent LR developed recurrences and all 3 recurrences occurred at distant sites.  Conclusion: Benign non-GIST submucosal tumors should always be considered in the differential diagnosis of small duodenal submucosal tumors. LR is a viable treatment option in patients with suspected GIST involving the duodenum provided that complete resection with clear margins can be obtained. PD should be reserved for patients where LR is not technically feasible.

Outcome after surgical treatment of suspected gastrointestinal stromal tumors involving the duodenum: Is limited resection appropriate?

// Fan Feng 1,* , Yangzi Tian 2,* , Zhen Liu 1,* , Guanghui Xu 1 , Shushang Liu 1 , Man Guo 1 , Xiao Lian 1 , Daiming Fan 1 and Hongwei Zhang 1 1 Department of Digestive Surgery, Xijing Hospital, Fourth Military Medical University, Xi’an, Shaanxi, China 2 Department of Dermatology, Xijing Hospital, Fourth Military Medical University, Xi’an, Shaanxi, China * These authors have contributed equally to this work Correspondence to: Hongwei Zhang, email: // Keywords : gastrointestinal stromal tumor, colon, feature, prognosis Received : November 30, 2015 Accepted : April 16, 2016 Published : May 05, 2016 Abstract Background: Due to the extremely rare incidence, data about colonic GISTs are limited. Therefore, aim of the present study was to explore clinicopathological characteristics and prognosis of colonic GISTs. Patients and Methods: Colonic GISTs cases were obtained from our center and from case report and clinical studies extracted from MEDLINE. Clinicopathological features and survivals were analyzed. Results: There were 79 colonic GISTs patients with a female predominance. The median age was 66 years (range 0.17-84). The median tumor size was 5.8 cm (range 0.5-29). The most common location was sigmoid colon (45.8%), followed by transverse colon (19.5%). The majority of colonic GISTs were high risk (70.8%). Mitotic index was correlated with gender ( P = 0.002) and tumor size ( P = 0.005), and tumor location was correlated with age ( P = 0.017). The five year DFS and DSS were 57.4% and 61.6%, respectively. Mitotic index and NIH risk classification were associated with prognosis of colonic GISTs. However, mitotic index was the only independent risk factor. The distribution of tumor size and NIH risk classification were significantly different between colonic and gastric GISTs (both P = 0.000). The DFS and DSS of colonic GISTs were significantly lower than that of gastric GISTs ( P = 0.012 and P = 0.002, respectively). Conclusions: The most common location for colonic GISTs was sigmoid colon. Most tumors were high risk. Mitotic index was the only independent risk factor for prognosis of colonic GISTs. Colonic GISTs differ significantly from gastric GISTs in respect to clinicopathological features. The prognosis of colonic GISTs was worse than that of gastric GISTs.

/pdf/clinicopathological-features-and-prognosis-of-mesenteric-4jy276v0yf.pdf

Clinicopathological features and prognosis of mesenteric gastrointestinal stromal tumor: evaluation of a pooled case series

Mounting evidence demonstrates the presence of extragastrointestinal stromal tumor (EGIST) which originates from tissues outside the gastrointestinal (GI) tract and shares overlapping immunohistological features with gastrointestinal stromal tumor (GIST). GIST emanating from prostate is extremely rare. To our knowledge, there are only 3 definitely reported cases of primary prostatic EGIST. Herein, we report a case of prostatic EGIST in 31-year-old man with low urinary tract symptoms who was initially misdiagnosed as sarcoma of prostate. Imaging studies assist in determining the origin and location of EGIST. Immunohistochemical assessment (DOG-1, CD117, and CD34) helps in differentiating such lesion from other stromal tumors and in addressing an appropriate and optimal therapeutic strategy.

A young man with primary prostatic extra-gastrointestinal stromal tumor: a rare case report and review of the literature

An extramural gastrointestinal stromal tumor of the duodenum mimicking a pancreatic head tumor

Primary omental gastrointestinal stromal tumors

Backround
Duodenal gastrointestinal stromal tumors are rare tumors. When these tumors arise from the second part of the duodenum they can easily be misdiagnosed as a pancreatic head cancer.

A duodenal stromal tumor mimicking a pancreatic head tumor: one case report and literature review

OBJECTIVES
The receptor for advanced glycation end-products (RAGE) is a membranous immunoglobulin involved in the pathogenesis of numerous autoimmune diseases and tumors. The aim of this study was to investigate the possible involvement of RAGE in the pathogenesis of myasthenia gravis.


MATERIAL AND METHODS
This prospective study included 41 cases of myasthenia gravis treated at our institution between 2010 and 2015. There were 18 men and 23 women, with an average age of 36.44±14.47 years. The majority of patients (24.4%) were classified as IIb, according to MGFA scoring, and 21 of them required corticosteroid and/or immunosuppressive treatment. Assessment of RAGE in thymus specimens was done by immunohistochemistry using RAGE antibody (C-term). RAGE expression was assessed according to various clinical, paraclinical and pathological parameters.


RESULTS
Histopathological studies found 18 thymomas, 17 hyperplasias and six other types of pathology. Expression of RAGE was negative/weak in 19 cases and moderate/strong in 22 cases. It was more important in thymoma type B2 (P<0.001) and when the duration of myasthenia was short (P=0.04), and was not significantly related to either myasthenia clinical severity or preoperative treatment.


CONCLUSION
Our results suggest that the RAGE pathway is involved in myasthenia gravis pathophysiology, especially at disease onset, and in forms with thymomas. Further studies would be indispensable to explore other aspects of this signaling pathway, especially the potential role of different ligands and soluble forms of RAGE.

Iconographies supplémentaires de l'article : Expression of receptor for advanced glycation end-products (RAGE) in thymus from myasthenia patients

Expression of receptor for advanced glycation end-products (RAGE) in thymus from myasthenia patients.

L’endometriose se definit par la presence de tissu endometrial en dehors de l’uterus. C’est une cause rare d’occlusion aigue. Son diagnostic est anatomopathologique vu l’absence de signes pathognomoniques permettant de faire le diagnostic au stade preoperatoire. A partir d’un cas clinique nous allons montrer la possibilite d’expression de l’endometriose intestinale par un tableau d’occlusion aigue faisant evoquer un diagnostic de neoplasie au stade radiologique et operatoire.

Une cause rare d’occlusion intestinale: endométriose intestinale. A propos d’un cas

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