Synovial sarcoma of the viscera (lung and jejunum): a case report.

We report the case of a woman nearing 70 years old who was admitted to the hospital with a complaint of "epigastric distension for 1 month". Her main signs and symptoms were progressive abdominal distension and occasional abdominal pain. Computed tomography suggested an abdominal mass. She had a surgical history of synovial sarcoma (SS) of the lungs. After admission, she was diagnosed with jejunal SS following a puncture biopsy and laparoscopic surgery. This disease usually occurs in the soft tissues of the limbs, and it is extremely rare for SS to originate in the jejunum. The morphologic heterogeneity of SS overlaps with other tumors and makes the diagnosis particularly difficult. Imaging studies usually lack specificity; however, measuring multiple immunohistochemical markers can greatly assist in the diagnosis and differential diagnosis of SS. This case not only enriches our understanding of SS and describes a rare site of origin, but also emphasizes the importance and challenges of achieving an accurate diagnosis. Immunohistochemical and molecular biological testing have important roles in the definitive diagnosis, highlighting the need for precise and innovative diagnostic and therapeutic approaches in SS.

Exploring the predictive power of jejunal microbiome composition in clinical and subclinical necrotic enteritis caused by Clostridium perfringens: insights from a broiler chicken model.

BACKGROUND: Necrotic enteritis (NE) is a severe intestinal infection that affects both humans and poultry. It is caused by the bacterium Clostridium perfringens (CP), but the precise mechanisms underlying the disease pathogenesis remain elusive. This study aims to develop an NE broiler chicken model, explore the impact of the microbiome on NE pathogenesis, and study the virulence of CP isolates with different toxin gene combinations. METHODS: This study established an animal disease model for NE in broiler chickens. The methodology encompassed inducing abrupt protein changes and immunosuppression in the first experiment, and in the second, challenging chickens with CP isolates containing various toxin genes. NE was evaluated through gross and histopathological scoring of the jejunum. Subsequently, jejunal contents were collected from these birds for microbiome analysis via 16S rRNA amplicon sequencing, followed by sequence analysis to investigate microbial diversity and abundance, employing different bioinformatic approaches. RESULTS: Our findings reveal that CP infection, combined with an abrupt increase in dietary protein concentration and/or infection with the immunosuppressive variant infectious bursal disease virus (vIBDV), predisposed birds to NE development. We observed a significant decrease (p < 0.0001) in the abundance of Lactobacillus and Romboutsia genera in the jejunum, accompanied by a notable increase (p < 0.0001) in Clostridium and Escherichia. Jejunal microbial dysbiosis and severe NE lesions were particularly evident in birds infected with CP isolates containing cpa, netB, tpeL, and cpb2 toxin genes, compared to CP isolates with other toxin gene combinations. Notably, birds that did not develop clinical or subclinical NE following CP infection exhibited a significantly higher (p < 0.0001) level of Romboutsia. These findings shed light on the complex interplay between CP infection, the gut microbiome, and NE pathogenesis in broiler chickens. CONCLUSION: Our study establishes that dysbiosis within the jejunal microbiome serves as a reliable biomarker for detecting subclinical and clinical NE in broiler chicken models. Additionally, we identify the potential of the genera Romboutsia and Lactobacillus as promising candidates for probiotic development, offering effective alternatives to antibiotics in NE prevention and control.

Multiple enteric muco-submucosal elongated polyps causing intussusception.

A 20-year-old woman presented to our hospital with abdominal pain. Abdominal computed tomography revealed multiple masses in the upper jejunum, which were suspected as lipomas. Partial resection of the small intestine, including the masses, was performed on the same day due to intussusception secondary to the masses. Pathological examination revealed that the masses consisted of mucosa and edematous submucosa with multiple dilated blood vessels and lymphatic ducts without muscularis propria. The masses were diagnosed as multiple muco-submucosal elongated polyps (MSEP), a type of non-neoplastic polyp. MSEP was originally named colonic MSEP, but with the development of endoscopic techniques and imaging tests, similar polyps have been reported to occur not only in the colon but also in the entire intestinal tract. In this case, multiple MSEPs in the upper jejunum caused intussusception. As reported cases of multiple lesions causing intussusception are few, our case may help to clarify the pathogenesis of this disease.

Superiority of Intestinal Adaptation by Hepatocyte Growth Factor in the Jejunum: An Experimental Study in a Short-Bowel Rat Model.

BACKGROUND: We evaluated the effect of recombinant human hepatocyte growth factor (rh-HGF) on intestinal adaptation in a rat model of short-bowel syndrome (SBS). METHODS: Sprague-Dawley rats underwent jugular vein catheterization for continuous total parenteral nutrition (TPN) and 90 % small bowel resection. The animals were divided into 3 groups: TPN/SBS (control group, n = 7), TPN/SBS/intravenous recombinant human hepatocyte growth factor (HGF) (0.3 mg/kg/day) (HGF group, n = 7), and TPN/SBS/intravenous c-Met inhibitor (0.3 mg/kg/day) (anti-HGF group, n = 5). On day 7, rats were euthanized and histologically evaluated. Serum diamine oxidase (S-DAO) levels were evaluated using an enzyme-linked immunosorbent assay. The nutrient transporter and glucagon-like peptide-2 (GLP-2) receptor expression were evaluated using real-time polymerase chain reaction. RESULTS: The jejunal and ileal villus heights were higher and the S-DAO concentrations significantly higher (p = 0.04) in the HGF group than in the control and anti-HGF groups. The sodium-dependent glucose transporter 1 expression in the HGF group was significantly higher than in the control group and significantly suppressed in the anti-HGF group (p < 0.01). The peptide transporter 1 expression in the jejunum was higher in the HGF group than in the other groups and significantly suppressed in the anti-HGF group (p < 0.01). The GLP-2 receptor expression in the jejunum was higher in the HGF group than the other groups, and it was significantly suppressed in the anti-HGF group (p < 0.01). These jejunal results regarding nutrient transporter an GLP-2 receptor were not found in the ileum. CONCLUSIONS: The administration of rh-HGF appears to be more effective in the jejunum than in the ileum. TYPE OF STUDY: Experimental Research. LEVEL OF EVIDENCE: N/A.

Gastrointestinal stromal tumour in a jejunal diverticulum: The eighth reported case worldwide with a brief review of the literature.

Jejunal diverticulosis is uncommon and so are gastrointestinal stromal tumours (GIST) arising in the jejunum. GIST arising in a jejunal diverticulum is a rarity and to date there are only 7 cases in the English literature. Our case of GIST occurring in a jejunal diverticulum of a 48-year-old lady would be the first reported in Malaysia and the 8th in the world. As in most cases, the clinical presentation and radiological findings of this patient were non-specific. With a history of acute abdominal pain, vomiting and fever, the patient was provisionally diagnosed as a case of twisted ovarian cyst and subjected to laparotomy. An intact roundish jejunal diverticulum 5.0 cm x 5.0 cm, about 50 cm distal to the duodeno-jejunal junction was found and resected with a segment of small intestine. Microscopic examination showed a tumour of the cut open diverticular wall, with epithelioid to spindled cells, demonstrating a mitotic rate of 1-2 per 5 mm2, confined to, while infiltrating the wall of the diverticulum. The immunohistochemical profile of positive staining for CD117, DOG-1, smooth muscle actin and CD34, and negative expression of desmin and S100 protein, clinched the diagnosis of GIST. Based on the AFIP Criteria for risk stratification,1 the patient was categorised as having moderate risk for disease progression, and was not offered further targeted imatinib as an immediate measure. The patient has remained well at the time of writing i.e. 8 months following excision, and continues on active surveillance by the surgical and oncological teams, with the option of imatinib, should the necessity arise. This case is presented not merely for the sake of documenting its rarity, but as a reminder to stay alert for uncommon conditions in histopathology practice.

Jejunal gastrointestinal stromal tumor that developed in a patient with neurofibromatosis type 1: a case report.

BACKGROUND: Neurofibromatosis type 1 (NF1) is known to be associated with the frequent occurrence of unique gastrointestinal stromal tumors (GISTs), preferably occurring in the small intestine, with no mutations in the c-kit proto-oncogene or platelet-derived growth factor receptor-alpha (PDGFRA), with a high tendency for multifocal development, indolent nature, with low proliferation activity and favorable prognosis. CASE PRESENTATION: A woman in her forties visited her local doctor complaining of menstrual pain; a large mass was detected in her lower abdomen, and she was referred to our hospital. The patient had hundreds of skin warts and café au lait spots. The patient's mother had been diagnosed with type 1 neurofibromatosis. The patient met the diagnostic criteria for NF1 and was diagnosed with NF1. Ultrasonography showed a large heterogeneous cystic mass with various echo patterns, solid compartments and multiple septations. Magnetic resonance imaging showed a multilocular cystic mass with liquid content exhibiting various intensities, including that of blood. A small round solid mass was also observed close to the cystic tumor. Contrast-enhanced computed tomography showed that the round solid mass showed strong enhancement in the early phase, unlike the cystic tumor component. Open laparotomy revealed a multicystic exophytic tumor measuring 11.5 cm originating from the jejunal wall, 20 cm distal to the duodenojejunal flexure. A solid tumor measuring 2.1 cm was also found on the anal side of the large tumor. We resected the short segment of the jejunum, including the two lesions. Microscopic findings revealed that the cystic and solid tumors consisted of spindle-shaped tumor cells showing little atypia with a fascicular or bundle arrangement. Nuclear mitosis was scarce. Immunostaining of the tumor cells showed positive staining for KIT and DOG1 and negative staining for S100 and desmin. The NF1 patient was diagnosed with multiple GISTs accompanied by intratumoral hemorrhagic denaturation arising from the jejunum. The TNM staging was pT4N0M0, stage IIIA. CONCLUSION: We report a case of GISTs associated with NF1 that showed a jejunal origin, multifocal development and few mitotic figures. The recurrence risk, survival prognosis and need for adjuvant chemotherapy, particularly in cases where the initial GIST exhibits a very indolent pathology in NF1-related GISTs, remain to be elucidated.

Influence of Escherichia coli infection on intestinal mucosal barrier integrity of germ-free piglets.

AIMS: We focused on investigating the influence of Escherichia coli (E. coli) on the intestinal barrier. MATERIAL AND METHODS: We studied changes in the distribution and secretory activities of goblet cells and enteroendocrine cells (EECs), as well as changes in the population of mast cells (MCs) in the jejunal and colonic mucosa of germ-free (GF) piglets as a healthy control group and GF piglets whose intestines were colonised with E. coli bacteria on day 5. KEY FINDINGS: The results suggest that the colon of GF piglets is more resistant and less prone to coliform bacterial infection compared to the jejunum. This can be confirmed by a lower degree of histopathological injury index as well as an improvement of the morphometric parameters of the colonic mucosa, together with a significantly increased (p < 0.05) expression of MUC1/EMA, and ZO-3. We also observed a significant decrease in the population of activated MCs (p < 0.001) and EECs (p < 0.001). These findings may indicate a rapid response and better preparation of the intestinal barrier for possible pathological attacks and the subsequent development of mucosal lesions during the development and progression of the intestinal diseases. SIGNIFICANCE: To date, gut-targeted therapeutic approaches that can modulate bacterial translocation and chronic inflammation are still in their infancy but represent one of the most promising areas of research for the development of new effective treatments or clinical strategies in the future. Therefore, a better understanding of these processes can significantly contribute to the development of these targeted strategies for disease prevention and treatment.

An adding up of an exceptionally rare case report: Primary Squamous cell carcinoma of Jejunum from North India.

Squamous cell carcinoma (SCC) of lower gastrointestinal tract is a rare tumor and duodenum is the most common site of involvement. In addition, involvement of jejunum by SCC is further exceptionally rare, and only nominal cases are present world literature. Being a very infrequent finding, a clinician and a pathologist must be aware of this rare entity. Diagnosis requires histopathology along with clinico-radiological correlation since merely histopathology is unable to differentiate between primary and metastatic tumor. Treatment modality is totally different in primary and secondary tumor of lower gastrointestinal tract. Being an extremely rare case of primary SCC of jejunum in an elderly female makes it eligible to be acknowledged at the global platform of medical literature.

Prenatal and postnatal peculiarities and consequences of intestinal loop patency disorder with expansive giant cystic abdominal mass in a preterm newborn.

AIM: To analyse the findings in a newborn (36 gestational weeks, birth weight: 4,030 grams, birth length: 48 cm, Apgar score 7/8/8 points) with prenatal suspicion of intestinal obstruction at the duodenum/jejunum level. The patient required urgent surgery on the first day of life. RESULTS: Examination of the abdominal cavity confirmed the presence of a cystic mass with a volume of approximately 800 ml, which was located at the site of jejunal atresia. As part of the surgical solution, the cystic formation and the atretic segment of the intestine were resected with subsequent end-to-end jejuno-jejunal anastomosis and Bishop‒Koop ileostomy. The histological examination of 3 samples collected confirmed the presence of mucous membrane and smooth muscle. CONCLUSION: The cyst anatomically communicated with the aboral section of the jejunum, but the lumen of the jejunum was functionally obturated by solid whitish masses. The histological examination confirmed the diagnostic features of a cyst of intestinal origin. The ileum and colon were patent throughout, but of smaller diameter, so a Bishop-Koop relieving anastomosis was indicated. The condition of the child at the age of 9 months was stabilised and surgical closure of the stoma was carried out (Tab. 1, Fig. 8, Ref. 21). Text in PDF www.elis.sk Keywords: newborn, jejunal atresia, intestinal cyst.

Acute Stress Regulates Sex-Related Molecular Responses in the Human Jejunal Mucosa: Implications for Irritable Bowel Syndrome.

Irritable bowel syndrome (IBS) is a prevalent gastrointestinal disorder linked to intestinal barrier dysfunction and life stress. We have previously reported that female sex per se determines an increased susceptibility to intestinal barrier dysfunction after cold pain stress (CPS). We aimed to identify sex-related molecular differences in response to CPS in healthy subjects to understand the origin of sex bias predominance in IBS. In 13 healthy males and 21 females, two consecutive jejunal biopsies were obtained using Watson's capsule, at baseline, and ninety minutes after CPS. Total mucosal RNA and protein were isolated from jejunal biopsies. Expression of genes related to epithelial barrier (CLDN1, CLDN2, OCLN, ZO-1, and ZO-3), mast cell (MC) activation (TPSAB1, SERPINA1), and the glucocorticoid receptor (NR3C1) were analyzed using RT-qPCR. NR3C1, ZO-1 and OCLN protein expression were evaluated through immunohistochemistry and western blot, and mucosal inflammation through MC, lymphocyte, and eosinophil numbering. Autonomic, hormonal, and psychological responses to CPS were monitored. We found an increase in jejunal MCs, a reduced CLDN1 and OCLN expression, and an increased CLDN2 and SERPINA1 expression 90 min after CPS. We also found a significant decrease in ZO-1, OCLN, and NR3C1 gene expression, and a decrease in OCLN protein expression only in females, when compared to males. CPS induced a significant increase in blood pressure, plasma cortisol and ACTH, and subjective stress perception in all participants. Specific and independent sex-related molecular responses in epithelial barrier regulation are unraveled by acute stress in the jejunum of healthy subjects and may partially explain female predominance in IBS.

Synchronous cutaneous malignant peripheral nerve sheath tumor and jejunal gastrointestinal stromal tumor and submucosal angiomyolipoma in type 1 neurofibromatosis: A case report and literature review.

RATIONALE: Type 1 neurofibromatosis (NF1) is one of the most prevalent genetic conditions. NF1 is characterized by cutaneous plexiform neurofibromas and café au lait skin pigmentation, and is inherited in an autosomal dominant trait with mutation in the neurofibromin 1 gene on chromosome 17. Neurofibromin is involved in Ras proto-oncogene regulation. Accordingly, NF1 may lead to malignancies, with a lifetime cancer risk of 60%. Malignant peripheral nerve sheath tumor (MPNST) is the leading cause of mortality due to NF1. The relevance of gastrointestinal stromal tumor (GIST) in NF1 is increasingly being reported in the literature and NF1-associated GIST has been identified to have an alternative molecular pathogenesis. PATIENT CONCERNS: A 62-years-old female had a 7 × 5 cm growing back mass in the background of various sized cutaneous neurofibromas with café au lait spots. Computed tomography performed in the workup revealed a 4.1 cm enhancing mass near the ileal mesentery. DIAGNOSES: NF1 affected by cutaneous MPNST of the back, and synchronous GIST and submucosal angiomyolipoma (AML) of the jejunum. INTERVENTIONS: The patient underwent laparoscopic jejunal mass excision, and excision and flap coverage for the back mass owing to the suspicion of multiple MPNSTs. However, the abdominal masses were diagnosed as GIST and AML following confirmation of the immunohistochemical profiles. Accordingly, the patient was administered adjuvant radiotherapy to the MPNST after surgery. OUTCOMES: Symptomatic improvements were achieved, and no subsequent relapses were observed. LESSONS: Although MPNST and GIST are not rare neoplasm in NF1, only 2 case reports have been published on the synchronous occurrence of these tumors. Moreover, no case report has been published on AML in NF1, except 1 renal AML in segmental neurofibromatosis. Identifying the clinical and pathologic significances of the NF1 is important to achieve improved diagnostic accuracy.

Diagnostic and Therapeutic Challenges in the Management of Acute Massive Overt Bleeding of Jejunal Gastrointestinal Stromal Tumours: Case Series.

INTRODUCTION: Jejunal gastrointestinal stromal tumours (GIST) are rare mesenchymal tumours. Acute massive overt bleeding from jejunal GIST is very rare and poses both diagnostic and therapeutic challenges in emergent conditions. METHODS: A case series with retrospective analysis of prospectively maintained database of patients presenting with acute massive overt bleeding secondary to histologically proven jejunal GIST was done. Clinical characteristics, endoscopic and imaging diagnostic features, histological findings, surgical procedures and outcomes in these patients were studied. RESULTS: Three patients were included in this case series. Mean age of presentation was 49.0 years with two male and one female patient. All three patients presented with melena and hemodynamic instability, resuscitated with adequate blood transfusions. Routine endoscopic assessment were inconclusive. Multiphasic Computed Tomographic Angiography (CTA) revealed hypodense hypervascular mass in jejunum in all three patients. One patient was unresponsive to blood transfusion and underwent emergency exploratory laparotomy. One patient underwent laparoscopic resection and reconstruction. Mean length of hospital stay was 5.3 days. Histopathological examination confirmed jejunal GIST in all three patients with microscopically negative resection margins. Two patients were disease free till 18-month follow-up and the one patient lost to follow-up after 1 year. CONCLUSION: Multiphasic CTA is a single-step diagnostic tool for localisation of bleed and assessment of tumour characteristics in emergent conditions. Surgical resection is the mainstay of treatment for both control of bleed and to provide oncologically clear resection margins.

Molecular detection and patho-morphological study of enteric Escherichia coli pathotypes in diarrheic neonatal calves.

To understand the pathology of natural cases of E. coli pathotypes infection in bovine calves, 45 cases of bovine calves, below one month of age, died due to enteritis were studied. Total seventeen cases (37.77%) turned positive for different pathotypes of E. coli by RT-PCR. Out of seventeen positive samples for E. coli, six cases (35.29%) were positive for eae gene, three cases (17.64%) for bfp gene and eight cases (47.05) for fimA gene of E. coli. Gross lesions in these cases showed pin-point to ecchymotic hemorrhages in the mucosa of jejunum, ileum and colon. The draining mesenteric lymph nodes were swollen, enlarged and showed cord -like structure. Histopathology of small intestine showed, villi lining cells were sloughed off, tips of villi capillary plexus were congested and hemorrhagic, and skipping lesions of microabscesses in the crypts of mucosa were observed. In the duodenum, necrosis of crypts and infiltration of mononuclear cells in the lamina propria and around Brunner's gland. In mesenteric lymph nodes the subscapular space were infiltrated with mononuclear cells with depletion of lymphoid follicles in cortical area. Peri-trabecular and medullary sinuses of mesenteric lymph nodes were necrosed.

Obscure gastrointestinal bleeding from a large jejunal lipoma treated using an endoscopic unroofing technique with double balloon enteroscopy: a case study.

Small intestinal lipomas are rare, but may cause obscure gastrointestinal bleeding. The endoscopic unroofing technique excises only the upper third of the lipoma and allows both histological confirmation and complete treatment with minimal risk of perforation. We present a rare case of obscure gastrointestinal bleeding caused by a jejunal lipoma. A 75-year-old man on antiplatelet therapy presented to our department with melena and anemia. Computed tomography revealed he had a 45-mm jejunal submucosal tumor with fat attenuation. Endoscopic resection using an endoscopic unroofing technique with double balloon enteroscopy was successfully performed. The tumor was confirmed to be a lipoma.

Enterococcus faecium supplementation prevents enteritis caused by Escherichia coli in goats.

The probiotic Enterococcus faecium is a gut microbe with immunomodulatory effects, which has been widely used to prevent diarrhoea in pigs and birds. Escherichia coli is a common pathogen that causes inflammatory bowel disease in animals. The aim of this study was to investigate the protective effects of E. faecium on enteritis in goats. Forty goats were randomly divided into 4 treatment groups: control, E. faecium, E. coli, and E. faecium + E. coli. The changes of physiological indicators and diarrhoea scoring were evaluated on days -4, -2, 0, 2, 4, 6, and 8. The pathological examination, inflammatory cytokines mRNA expression and bacterial counts in jejunum and caecum were detected on day 4 and 8. The results showed that body temperature, respiratory rate, heart rate and leukocyte counts all increased from the 2nd to the 6th day after feeding with E. coli, and the diarrhoea score was significantly increased. However, E. faecium-pretreated goats had lower body temperatures and fewer leukocytes than E. coli-treated goats on day 2, as well as decreased diarrhoea scores. E. coli treatment caused histopathological damage and morphological changes in the jejunum and caecum, while pretreatment with E. faecium significantly alleviated these injuries. E. faecium pretreatment can reduce the load of E. coli and increase the prevalence of Lactobacillus, thereby balancing the microbiota in the intestine. Furthermore, E. coli-infected goats pretreated with E. faecium showed obvious inhibition of Toll-like receptor 4, interleukin (IL)-1ß, IL-6, IL-8 and tumour necrosis factor-α mRNA expression in the jejunum and caecum compared to that in the E. coli treatment group. In conclusion, the addition of E. faecium to goat feed is beneficial for improving clinical symptoms, maintaining intestinal mucosa integrity, balancing the microbiota and decreasing inflammatory responses in E. coli-induced intestinal injury.

Duodenal Ampulla Neuroendocrine Tumor with GISTs of the Proximal Jejunum: A Case Report.

Neuroendocrine tumors (NEN) are a type of heterogenous, slow-growing tumors, that only in about half of the cases can be found in the gastrointestinal tract. Half of these is in the small intestine. The ampullary NENs are rare, accounting for less than 1% of gastroenteropancreatic NENs. Gastrointestinal stromal tumors (GIST) are a more common type of tumors of the gastrointestinal tract that consist of pacemaker cells. The occurrence of both tumors simultaneously is rare, but in patients with neurofibromatosis type 1, the co-existence of NEN and GIST is more often. Here we report a case of simultaneous occurrence of a well-differentiated NEN and a GIST in a patient without neurofibromatosis. Also, we provide a short review of the current knowledge and treatment strategies regarding these tumors.