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Choi W, Lauwers GY, Slavik T. Inflammatory disorders of the stomach. MORSON AND DAWSON'S GASTROINTESTINAL PATHOLOGY 2024:135-194. [DOI: 10.1002/9781119423195.ch11] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/02/2025]
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2
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Surapaneni D, Azam B, Dasi SC. Eosinophilic Ascites: A Rare Diagnosis With an Even Rarer Etiology. Cureus 2024; 16:e68511. [PMID: 39364509 PMCID: PMC11447649 DOI: 10.7759/cureus.68511] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 09/02/2024] [Indexed: 10/05/2024] Open
Abstract
Eosinophilic ascites (EA) is a rare and often challenging clinical manifestation of eosinophilic gastroenteritis (EGE), a condition characterized by eosinophilic infiltration in various layers of the gastrointestinal tract. EA specifically involves the abnormal accumulation of eosinophils in the peritoneal cavity, which can lead to significant abdominal distension and discomfort. EGE is an inflammatory disorder that can affect the mucosal, muscular, or serosal layers of the gastrointestinal tract, primarily resulting from a combination of genetic predisposition, environmental triggers, and immune responses. This case report discusses a 39-year-old male who presented with persistent abdominal distension, significant weight loss, vomiting, and chronic diarrhea. Clinical evaluation revealed marked eosinophilia and EA, prompting a series of diagnostic tests to differentiate it from other potential causes such as parasitic infections and malignancies. Imaging studies indicated moderate ascites and intestinal wall thickening. The patient was diagnosed with eosinophilic enteritis, and treatment with corticosteroids led to substantial clinical improvement. This case highlights the diagnostic challenges and management strategies associated with both EA and EGE, emphasizing the importance of recognizing these rare manifestations of eosinophilic gastrointestinal disorders.
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Affiliation(s)
- Devipriya Surapaneni
- Internal Medicine, Saveetha Medical College and Hospital, Saveetha Institute of Medical and Technical Sciences, Chennai, IND
| | - Bilal Azam
- Medical Gastroenterology, Saveetha Medical College and Hospital, Saveetha Institute of Medical and Technical Sciences, Chennai, IND
| | - Sharath Chandra Dasi
- Internal Medicine, Saveetha Medical College and Hospital, Saveetha Institute of Medical and Technical Sciences, Chennai, IND
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3
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Kourti A, Dimopoulou A, Zavras N, Sakellariou S, Palamaris K, Kanavaki I, Fessatou S. Inflammatory fibroid polyp of the anus in a 12-month-old girl: Case report and review of the literature. J Paediatr Child Health 2022; 58:1313-1316. [PMID: 35730111 DOI: 10.1111/jpc.16080] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/30/2022] [Revised: 05/13/2022] [Accepted: 05/31/2022] [Indexed: 11/28/2022]
Abstract
Inflammatory fibroid polyp (IFP) is a rare, usually solitary and intraluminal polypoid benign tumour that can affect any part of the gastrointestinal (GI) tract. Its aetiology is unknown and clinical presentation depends on the site of involvement. We present the case of a 12-month-old girl with IFP and review all reported cases of IFP in children and adolescents <18 years. A 12-month-old girl presented with rectal bleeding. The patient underwent colonoscopy which revealed an anus polyp. Surgical resection was performed and histopathological examination of the specimen showed features of IFP. A literature review of 20 cases (including ours) between 1966 and January 2022 is also presented. To our knowledge, this is the youngest reported patient with IFP and the first in the anal area.
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Affiliation(s)
- Afroditi Kourti
- Department of Paediatric Gastroenterology, Hepatology and Nutrition, Third Department of Paediatrics, Attikon University General Hospital, National and Kapodistrian University of Athens, Athens, Greece
| | - Anastasia Dimopoulou
- Department of Pediatric Surgery, Attikon University General Hospital, National and Kapodistrian University of Athens, Athens, Greece
| | - Nikolaos Zavras
- Department of Pediatric Surgery, Attikon University General Hospital, National and Kapodistrian University of Athens, Athens, Greece
| | - Stratigoula Sakellariou
- First Department of Pathology, Medical School, National and Kapodistrian University of Athens, Athens, Greece
| | - Kostas Palamaris
- First Department of Pathology, Medical School, Laiko General Hospital, National and Kapodistrian University of Athens, Athens, Greece
| | - Ino Kanavaki
- Department of Paediatric Gastroenterology, Hepatology and Nutrition, Third Department of Paediatrics, Attikon University General Hospital, National and Kapodistrian University of Athens, Athens, Greece
| | - Smaragdi Fessatou
- Department of Paediatric Gastroenterology, Hepatology and Nutrition, Third Department of Paediatrics, Attikon University General Hospital, National and Kapodistrian University of Athens, Athens, Greece
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4
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Koutri E, Papadopoulou A. Eosinophilic Gastrointestinal Disorders Beyond Eosinophilic Esophagitis. TEXTBOOK OF PEDIATRIC GASTROENTEROLOGY, HEPATOLOGY AND NUTRITION 2022:361-378. [DOI: 10.1007/978-3-030-80068-0_27] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
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Buda C, Garipoli C, Penna G, D'Aquino A, Galletti C, Facciolà A, Fedele F. Endoscopic mucosal resection of a large inflammatory fibroid polyp (Vanek's tumor): a case report. ACTA BIO-MEDICA : ATENEI PARMENSIS 2021; 92:e2021062. [PMID: 34212909 PMCID: PMC8343751 DOI: 10.23750/abm.v92i3.11317] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 02/06/2021] [Accepted: 03/15/2021] [Indexed: 11/24/2022]
Abstract
Vanek’s Tumor (inflammatory fibroid polyp) is a rare benign mesenchymal lesion occurring throughout the digestive tract. Classical Vanek’s tumor (“gastric”) contains concentric formations of proliferating spindle cells, which are CD34 positive. Atypical-inflammatory pseudotumor-like Vanek’s tumor (“intestinal”) lacks concentric formations and is CD34 negative. A 70-years-old man patient presented during hematochemical routine tests, sideropenic anemia and leukopiastrinosis. The patient performed osteomyelitis biopsy and esophagogastroduodenoscopy (EGD) showing a gastric wall with nodular appearance and, in antrum pre-pyloric, a polypoid pedunculated lesion, measuring approximately 3 cm in diameter, surrounded by hyperemic mucosa. The lesion then was removed by en bloc endoscopic mucosal resection (EMR) and histo-morphological, immune-cytochemical and biomolecular evaluations were performed. The data were compatible with a benign polyp fibroid inflammatory (Vanek’s Tumor). The results of this study suggest that endoscopic mucosal resection is a safe and efficacy solution for the resection of these gastrointestinal polyps and the two morphological patterns of Vanek’s tumor more probably represent only variants of one type of tumor than two different lesions. BRAF mutations were not shown growth PDGFRA wild-type Vanek’s tumor. (www.actabiomedica.it)
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Affiliation(s)
- Carmelo Buda
- Department of Human Pathology of the Adult and the Developmental Age "G. Barresi", University of Messina, Messina, Italy, .
| | - Claudia Garipoli
- Department of Human Pathology of the Adult and the Developmental Age "G. Barresi", University of Messina, Messina, Italy, .
| | - Giuseppa Penna
- Department of Human Pathology of the Adult and the Developmental Age "G. Barresi", University of Messina, Messina, Italy, .
| | - Antonio D'Aquino
- Department of Human Pathology of the Adult and the Developmental Age "G. Barresi", University of Messina, Messina, Italy, .
| | - Claudio Galletti
- Department of Human Pathology of the Adult and the Developmental Age "G. Barresi", University of Messina, Messina, Italy, .
| | - Alessio Facciolà
- Department of Biomedical and Dental Sciences and Morphofunctional Imaging, University of Messina, Messina, Italy.
| | - Francesco Fedele
- a:1:{s:5:"en_US";s:112:"Department of Biomedical and Dental Sciences and Morphofunctional Imaging, University of Messina, Messina, Italy";}.
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Gaballa S, Hlaing KM, Mahler N, Hargrove R, Roberts M. Eosinophilic Gastroenteritis Presenting As Unexplained Chronic Abdominal Pain. Cureus 2020; 12:e8640. [PMID: 32685309 PMCID: PMC7364395 DOI: 10.7759/cureus.8640] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/31/2020] [Accepted: 06/15/2020] [Indexed: 02/04/2023] Open
Abstract
A 27-year-old Caucasian female was hospitalized three times over a four-month period for recurrent, intermittent abdominal pain associated with nausea and diarrhea. No signs or symptoms of gastrointestinal (GI) bleeding were present. A stool occult blood test and stool enteric pathogen tests were negative. A complete blood count (CBC) revealed a peripheral blood eosinophil count of 1080 cells /µL without any inflammatory reaction. An upper endoscopy showed grossly normal-appearing esophageal and duodenal mucosa; however, a gastric mucosal biopsy showed an eosinophil infiltration of ≥20 eosinophils/high power field (HPF). Based on these findings, she was diagnosed with eosinophilic gastroenteritis (EGE). A definitive diagnosis of EGE should be confirmed with both an analysis of gastrointestinal mucosal biopsy and an elevated peripheral blood eosinophil count. Specifically, histological evaluation of the mucosal tissue must show an eosinophilic infiltration rate of 20 eosinophils/HPF. The diagnosis should be followed by an extensive review of the patient's allergic disease history.
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Affiliation(s)
- Salem Gaballa
- Internal Medicine, LewisGale Medical Center, Salem, USA
| | - Kyaw M Hlaing
- Internal Medicine, LewisGale Medical Center, Salem, USA
| | - Nathan Mahler
- Internal Medicine, LewisGale Medical Center, Salem, USA
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Sunkara T, Rawla P, Yarlagadda KS, Gaduputi V. Eosinophilic gastroenteritis: diagnosis and clinical perspectives. Clin Exp Gastroenterol 2019; 12:239-253. [PMID: 31239747 PMCID: PMC6556468 DOI: 10.2147/ceg.s173130] [Citation(s) in RCA: 76] [Impact Index Per Article: 12.7] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/24/2018] [Accepted: 04/26/2019] [Indexed: 12/14/2022] Open
Abstract
Eosinophilic gastroenteritis (EGE) is a digestive disorder in children and adults that is characterized by eosinophilic infiltration in the stomach and intestine. The underlying molecular mechanisms predisposing to this disease are unknown, but it seems that hypersensitivity response plays a major role in its pathogenesis, as many patients have a history of seasonal allergies, food sensitivities, asthma, and eczema. Symptoms and clinical presentations vary, depending on the site and layer of the gastrointestinal wall infiltrated by eosinophils. Laboratory results, radiological findings, and endoscopy can provide important diagnostic evidence for EGE; however, the cornerstone of the diagnosis remains the histological examination of gastric and duodenal specimens for evidence of eosinophilic infiltration (>20 eosinophils per high-power field), and finally clinicians make the diagnosis in correlation with and by exclusion of other disorders associated with eosinophilic infiltration. Although spontaneous remission is reported in around 30%–40% of EGE cases, most patients require ongoing treatment. The management options for this disorder include both dietary and pharmacological approaches, with corticosteroids being the mainstay of therapy and highly effective. The subsequent course is quite variable. Some patients have no recurrences, while a few experience recurrent symptoms during or immediately after corticosteroid interruption. An alternative therapeutic armamentarium includes mast-cell stabilizers, leukotriene antagonists, antihistamines, immunomodulators, and biological agents. In this review, we provide a summary of the different diagnostic tools utilized in practice, as well as the different therapeutic approaches available for EGE management.
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Affiliation(s)
- Tagore Sunkara
- Division of Gastroenterology and Hepatology, Mercy Medical Center, Des Moines, IA 50314, USA.,Division of Gastroenterology, St. Barnabas Hospital Health System, New York, NY, 10457, USA
| | - Prashanth Rawla
- Department of Internal Medicine, Sovah Health, Martinsville, VA, 24112, USA
| | - Krishna Sowjanya Yarlagadda
- Division of Gastroenterology and Hepatology, Mercy Medical Center, Des Moines, IA 50314, USA.,Division of Gastroenterology, St. Barnabas Hospital Health System, New York, NY, 10457, USA
| | - Vinaya Gaduputi
- Division of Gastroenterology, St. Barnabas Hospital Health System, New York, NY, 10457, USA
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Klingbeil KD, Azab B, Moller MG. Low-grade appendiceal mucinous neoplasm and endometriosis of the appendix. World J Surg Oncol 2017; 15:226. [PMID: 29258523 PMCID: PMC5738062 DOI: 10.1186/s12957-017-1294-1] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/02/2017] [Accepted: 12/08/2017] [Indexed: 01/07/2023] Open
Abstract
Background A distended, mucous-filled appendix is known as an appendiceal mucocele. They are a rare form of an appendiceal mass and develop from both benign and malignant processes. Mucoceles can develop secondarily to an obstruction, such as from a fecalith, scarring or, rarely, endometriosis. Only 12 cases of non-neoplastic appendiceal mucoceles caused by endometriosis have been previously described. The association between neoplastic appendiceal mucoceles in the presence of endometriosis is described for the first time in this report. Case presentation A 57-year-old woman presented with a chief complaint of worsening abdominal pain over the past 3 months. Imaging studies revealed an appendiceal mass. Laparoscopic evaluation confirmed an appendiceal mucocele, and the patient underwent complete appendectomy. No evidence of mucinous or endometrial deposits were present within the abdominal cavity. Pathological diagnosis revealed low-grade appendiceal mucinous neoplasm (LAMN) with evidence of endometriosis within the muscularis propria of the appendix. The patient recovered without complications and her abdominal pain completely resolved. Conclusions Endometriosis of the appendix is a rare manifestation and is most often identified as an incidental finding. Endometriosis leading to an obstructive mucocele of the appendix is an exceedingly rare finding, having only been described 12 times in the medical literature. LAMN in the presence of endometriosis of the appendix is described for the first time in this report. The association between appendiceal neoplasms in the presence of endometriosis requires further research in order to optimize operative treatment.
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Affiliation(s)
- Kyle D Klingbeil
- University of Miami Miller School of Medicine, 1475 NW 12th Ave, 3rd Flr, Miami, FL, 33136, USA.
| | - Basem Azab
- University of Miami Miller School of Medicine, 1475 NW 12th Ave, 3rd Flr, Miami, FL, 33136, USA.,Department of Surgical Oncology, Sylvester Comprehensive Cancer Center, Miami, FL, 33136, USA
| | - Mecker G Moller
- University of Miami Miller School of Medicine, 1475 NW 12th Ave, 3rd Flr, Miami, FL, 33136, USA.,Department of Surgical Oncology, Sylvester Comprehensive Cancer Center, Miami, FL, 33136, USA
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Klingbeil KD, Balaban A, Fertig RM, Gamret AC, Gong Y, Torres C, Satahoo SS. Inflammatory fibroid polyp of the gastric antrum presenting as hypovolemic shock: Case report and literature review. Intractable Rare Dis Res 2017; 6:304-309. [PMID: 29259861 PMCID: PMC5735286 DOI: 10.5582/irdr.2017.01060] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/05/2022] Open
Abstract
Inflammatory fibroid polyps (IFP) are an extremely rare entity that arise within the submucosa of the gastrointestinal tract, and represent less than 0.1% of all gastric polyps. They are most commonly localized to the gastric antrum, small intestines and recto-sigmoid colon. IFPs are most commonly found incidentally upon endoscopic evaluation in the absence of symptoms. Presenting symptoms depend on the location of the tumor, although polyps located in the stomach most commonly present with epigastric pain and early satiety. Classic histologic features include perivascular onion skinning of spindle cells with an abundance of eosinophilic infiltration. The prompt diagnosis and management of IFP is essential due to its underlying risk for intussusception, outlet obstruction and acute hemorrhage. In addition, recent evidence has shown that IFP is driven by an activating mutation in the platelet derived growth factor receptor alpha (PDGFRA) gene, suggesting a neoplastic etiology. Herein, we discuss a case of a 65-year-old woman with an inflammatory fibroid polyp of the gastric antrum who initially presented with early hypovolemic shock and melena. Diagnosis was made by endoscopic visualization, biopsy and immunohistochemical analysis.
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Affiliation(s)
- Kyle D. Klingbeil
- University of Miami, Miller School of Medicine, Miami, FL, USA
- Address correspondence to: Dr. Kyle D. Klingbeil, University of Miami Miller School of Medicine, 1600 NW 10th Ave #1140, Miami, Florida 33136, USA. E-mail:
| | | | | | | | - Yuna Gong
- Oak Hill Hospital, Graduate Medical Education, Department of Internal Medicine, Miami, FL, USA
| | - Carolyn Torres
- University of Pennsylvania, College of Liberal and Professional Studies, Philadelphia, PA, USA
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Munjal A, Al-Sabban A, Bull-Henry K. Eosinophilic Enteritis: A Delayed Diagnosis. J Investig Med High Impact Case Rep 2017; 5:2324709617734246. [PMID: 29051893 PMCID: PMC5638174 DOI: 10.1177/2324709617734246] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/01/2017] [Accepted: 08/19/2017] [Indexed: 12/28/2022] Open
Abstract
Eosinophilic gastrointestinal disorders are a rare and complex group of disorders that are characterized by eosinophilic infiltration of the gastrointestinal tract. Patients often present with a wide range of signs and symptoms as any length or layer of the GI tract can be involved such as mucosal, muscular, or serosal. As a part of the workup, patients frequently undergo computed tomography scans and multiple endoscopies before the diagnosis is finally made as was true in our case of a 59-year-old male patient presenting with 2 months of nausea, abdominal pain, and weight loss. He underwent esophagogastroduodenoscopies, colonoscopies, video capsule study, and balloon enteroscopy before the diagnosis was confirmed histologically. Endoscopic and radiographic findings can be variable and are usually unpredictable. The diagnosis is confirmed on histopathological examination of biopsies that must show >15-50 eosinophils/high-power field based on the location in the GI tract. In our patient, erythema, scalloping, whitish exudate, and patches of villous blunting were noted in the duodenum to proximal ileum endoscopically with >50 eosinophils/high-power field confirming the diagnosis of eosinophilic enteritis. This class of diseases is often found in patients with a history of allergic disorders suggestive of hypersensitivity in the etiology of the disease although our patient had no such known history. Elimination diets and steroids are the mainstay of therapy and often lead to complete resolution of symptoms as well as endoscopic and radiographic findings in up to 90% of patients as was seen in our patient, although some patients have a chronic remitting course.
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11
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Calderon MG, Caivano VC, Bagnaresi S, de Oliveira Lira JO, Raimundo RD, de Abreu LC, Correa JA. A unique case of inflammatory fibroid polyp in the duodenum of a female adolescent: Case report and literature review. Medicine (Baltimore) 2017; 96:e6131. [PMID: 28225494 PMCID: PMC5569430 DOI: 10.1097/md.0000000000006131] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
Abstract
BACKGROUND Inflammatory fibroid polyp (IFP) is a very rare benign condition in children that can occur throughout the gastrointestinal tract. It is characterized as a polypoid lesion originating in the submucosa, composed of connective tissue and eosinophilic infiltrate. It is most common in the stomach and in adults between the fifth and seventh decades of life. Its occurrence is unusual in the duodenum. CASE SUMMARY One case of duodenal IFP was described and the literature is reviewed with emphasis on the clinical and pathological features of IFP in children. A case of an IFP in the duodenum of a 13-year-old girl, who presented with abdominal pain, weight loss, vomiting, and constipation. The patient underwent exploratory laparotomy; a stenosing tumor of the third duodenal portion was found. The affected segment was resected and an end-to-end anastomosis between the duodenum and jejunum segment was performed. Immunohistochemically, actin and CD34 were positive, Ki67 was positive in <1% of cells, and the proteins CD117 and S100 were negative. CONCLUSION To our best of our knowledge, this is the fourth report of IFP in adolescents, the first in a female's duodenum.
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Affiliation(s)
| | | | | | | | - Rodrigo Daminello Raimundo
- Laboratório de Delineamento de Estudos e Escrita Científica, Faculdade de Medicina do ABC (FMABC), Santo André, Brazil
| | - Luiz Carlos de Abreu
- Laboratório de Delineamento de Estudos e Escrita Científica, Faculdade de Medicina do ABC (FMABC), Santo André, Brazil
| | - João Antonio Correa
- Full professor, Angiology and Vascular Surgery, Faculdade de Medicina do ABC (FMABC), Santo André, Brazil
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Uppal V, Kreiger P, Kutsch E. Eosinophilic Gastroenteritis and Colitis: a Comprehensive Review. Clin Rev Allergy Immunol 2016; 50:175-88. [PMID: 26054822 DOI: 10.1007/s12016-015-8489-4] [Citation(s) in RCA: 89] [Impact Index Per Article: 9.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
Eosinophilic gastrointestinal disorders, including eosinophilic esophagitis, gastroenteritis, and colitis, refer to a spectrum of clinical diseases that present with variable degrees of infiltration of the gastrointestinal tract by eosinophils in the absence of other known causes of tissue eosinophilia. Clinical symptoms and laboratory findings are usually non-specific and may or may not be accompanied by peripheral blood eosinophilia. The extent of eosinophilic infiltration of the gastrointestinal wall varies from mucosal to transmural and serosal involvement. Diagnosis requires presence of gastrointestinal symptoms, demonstration of gastrointestinal eosinophilia by biopsy, and exclusion of other known causes of tissue eosinophilia. Many studies have pointed toward the eosinophil as the major offender; however, the exact functional role of the eosinophil in the pathogenesis of eosinophilic gastrointestinal disorders remains unclear. The roles of T-helper-2 cytokines and other mediators, such as eotaxin-1 and interleukin-5, have gained significant importance in the pathobiology of eosinophilic gastrointestinal disorders. Current understanding of treatment is based on case reports and a few case series, as there is a lack of large prospective studies. Steroids are currently the mainstay of therapy, but the roles of other drugs such as leukotriene inhibitors, mast cell stabilizers, interleukin-5 inhibitors, and anti-immunoglobulin E, along with other targets in the immune pathway, are currently being explored.
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Affiliation(s)
- Vikas Uppal
- Division of Pediatric Gastroenterology and Nutrition, Nemours/Alfred I. duPont Hospital for Children, 1600 Rockland Rd, Wilmington, DE, 19803, USA
| | - Portia Kreiger
- Department of Pathology, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA
| | - Erika Kutsch
- Division of Pediatric Gastroenterology and Nutrition, Nemours/Alfred I. duPont Hospital for Children, 1600 Rockland Rd, Wilmington, DE, 19803, USA.
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Hua S, Cook D, Walker MM, Talley NJ. Pharmacological treatment of eosinophilic gastrointestinal disorders. Expert Rev Clin Pharmacol 2016; 9:1195-209. [PMID: 27191032 DOI: 10.1080/17512433.2016.1190268] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
INTRODUCTION Eosinophilic gastrointestinal disorders (EGIDs) are increasingly prevalent chronic inflammatory diseases characterized by eosinophilic infiltration of the gastrointestinal (GI) tract, in the absence of other known causes of eosinophilia. AREAS COVERED Clinical management of EGIDs is challenging, as there are currently limited therapeutic options available. The most common EGID is eosinophilic esophagitis (EoE), and rarer forms are eosinophilic gastritis, eosinophilic gastroenteritis, and eosinophilic colitis. Clinical presentation depends on the affected GI site. Recently duodenal eosinophilia has been recognized to commonly be present in patients with functional dyspepsia. This review will provide an overview of the pathogenesis and therapeutic management of EGIDs, with particular focus on the pharmacological strategies for these conditions. Expert commentary: Despite the considerable progress made in understanding the pathogenesis of EGIDs, there is still an urgent need for the development of specific and effective therapeutic approaches. Therapeutic management protocols are required that are based on rigorous clinical investigation in large prospective controlled trials to better understand the risks, benefits and limitations of each therapy. More well-defined and consistent end-points are also required to assess treatment outcomes, as there has been variability between patient reported outcomes, clinical outcomes, and histological outcomes in the studies to date.
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Affiliation(s)
- Susan Hua
- a School of Biomedical Sciences and Pharmacy , University of Newcastle , Callaghan , NSW , Australia
- b Hunter Medical Research Institute , New Lambton Heights , NSW , Australia
| | - Dane Cook
- c John Hunter Hospital , New Lambton Heights , NSW , Australia
| | - Marjorie M Walker
- b Hunter Medical Research Institute , New Lambton Heights , NSW , Australia
- d School of Medicine & Public Health , University of Newcastle , Callaghan , NSW , Australia
| | - Nicholas J Talley
- b Hunter Medical Research Institute , New Lambton Heights , NSW , Australia
- d School of Medicine & Public Health , University of Newcastle , Callaghan , NSW , Australia
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Khadanga S, Dugar D, Khurana U, Satapathy SP. Inflammatory fibroid polyp (Vanek's tumour): my worst nightmare in the emergency department. BMJ Case Rep 2016; 2016:bcr-2015-213688. [PMID: 26873916 DOI: 10.1136/bcr-2015-213688] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/12/2023] Open
Abstract
A middle-aged woman presented in shock with bleeding per rectum for 2 days without any significant past medical or surgical illness. Abdominal sonography, oesophagogastroduodenoscopy and sigmoidocolonoscopy were unremarkable. Despite 6 units of blood transfusion, 3000 mL of plasma expanders and ionotropes, the arterial pressure gradually decreased. We had no choice but to undergo life-threatening yet possibly life saving laparotomy. We found a 1×1 cm ileal bleeding mucosal polyp about 20 cm away from the ileocaecal junction. We resected and anastomosed end to end. Perioperatively, the patient was transfused with another 4 units of blood product. Immunohistopathology revealed a large number of CD34 positive spindle cells and eosinophils with extensive thick-walled capillaries compatible with inflammatory fibroid polyp. This case of Vanek's tumour is reported for its atypical site, which presented as massive obscure gastrointestinal haemorrhage without any prior warning signs.
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Affiliation(s)
| | - Dharmendra Dugar
- Department of Surgery, Hitech Medical College and Hospital, Bhubaneswar, India
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Han LL, Zhang JP, Li DY, Wang HL, Lin S, Li JS. Effect of glucocorticoid on expression of Eotaxin-3 in eosinophilic gastroenteritis. Shijie Huaren Xiaohua Zazhi 2015; 23:4428-4432. [DOI: 10.11569/wcjd.v23.i27.4428] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
AIM: To detect the expression of eosinophil chemotactic factor-3 (Eotaxin-3) in eosinophilic gastroenteritis (EG) and investigate the effect of glucocorticoid on Eotaxin-3 expression.
METHODS: Forty-eight patients with EG and 24 healthy controls were included. Patients with EG were equally divided into two groups, a placebo group and a prednisone group. Eotaxin-3 expression was measured by enzyme-linked immunosorbent assay in the serum and tissue samples of all subjects.
RESULTS: The expression levels of Eotaxin-3 in serum and tissue were significantly elevated in the EG group compared to normal controls (P < 0.05). Eotaxin-3 in serum and tissue significantly decreased after treatment (P < 0.05), although its change had no significance in the placebo group. A positive correlation was observed between eosinophil (Eos) count and Eotaxin-3 in serum and tissue in the EG group.
CONCLUSION: Eotaxin-3 may be involved in the pathophysiological process of EG. Hormone therapy may inhibit the expression of Eotaxin-3. Serum Eotaxin-3 may be used as a non-invasive index for evaluating the treatment effect in EG.
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Villanueva MS, Alimi Y. Microscopic colitis (lymphocytic and collagenous), eosinophilic colitis, and celiac disease. Clin Colon Rectal Surg 2015; 28:118-26. [PMID: 26034409 PMCID: PMC4442721 DOI: 10.1055/s-0035-1549365] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Abstract
Multiple tests are needed to diagnose a patient with noninfectious diarrhea. Some patients will be mistakenly labeled as diarrhea-predominant irritable bowel syndrome (IBS-D) because of nonspecific computed tomographic scans and grossly normal endoscopic findings. It is crucial to understand other less common pathologies to avoid these instances of misdiagnosis. This article focuses on microscopic colitis (MC), eosinophilic colitis (EC), and celiac disease. MC is an inflammatory condition of the colon that presents with two subtypes, only to be differentiated by histology. EC is a rare chronic inflammatory process. Depending on the extent of the disease, it can present with mild diarrhea, malabsorption, or at its worst, cause obstruction and perforation. Celiac disease affects the small bowel, but interestingly can present similarly to colitis. Both MC and EC respond to oral budesonide. Patients with celiac disease improve on gluten-free diets. These treatments are distinctly different from typical IBS-D care plans.
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Affiliation(s)
| | - Yewande Alimi
- Georgetown University Hospital, Department of Surgery, Washington, District of Columbia
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Kwiatkowski AP, Paśnik K. Large inflammatory fibroid polyp of cardia managed laparoscopically - a case report and review of the literature. Wideochir Inne Tech Maloinwazyjne 2014; 9:623-6. [PMID: 25562003 PMCID: PMC4280427 DOI: 10.5114/wiitm.2014.46448] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/23/2013] [Revised: 08/24/2013] [Accepted: 09/14/2014] [Indexed: 11/24/2022] Open
Abstract
Inflammatory fibroid polyp or Vanek's tumor of the gastrointestinal tract is a rare, benign neoplasm. The lesion may occur at any age but is commonest in the sixth and seventh decades and is more often found in women. It is mostly situated in the stomach, especially in the antrum. Symptoms depend on the location and the size of the lesion. We present a case of a 67-year-old woman with a large inflammatory polyp of the cardia with a diameter of 10 cm × 5.5 cm × 7.5 cm imitating gastrointestinal stromal tumor (GIST). The patient complained of abdominal pain, nausea and mild anemia. We performed laparoscopic topical excision of the tumor. The postoperative course was uneventful. The 6-month follow-up did not reveal recurrence. The clinical image of a large Vanek's tumor can imitate symptoms of gastric cancer or GIST. Topical laparoscopic excision with a 1-2 cm healthy wall margin is a sufficient and adequate way of treatment.
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Affiliation(s)
- Andrzej P Kwiatkowski
- Department of General, Oncological, Metabolic and Thoracic Surgery, Military Institute of Medicine, Warsaw, Poland
| | - Krzysztof Paśnik
- Department of General, Oncological, Metabolic and Thoracic Surgery, Military Institute of Medicine, Warsaw, Poland
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18
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Eosinophilic gastritis with gastric outlet obstruction mimicking infantile hypertrophic pyloric stenosis. J Pediatr Gastroenterol Nutr 2014; 59:e9-e11. [PMID: 25222812 DOI: 10.1097/mpg.0b013e31828ee524] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
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Abstract
AbstractEosinophils play a crucial role in the inflammatory response in conjunction with both innate and adaptive immunity. Eosinophils have long been recognized as inflammatory leukocytes that are particularly important in patients with parasitic infestations. However, recent studies in veterinary medicine demonstrate a number of canine eosinophilic gastrointestinal (GI) disorders unrelated to a parasitic infestation. Although the underlying pathophysiology behind eosinophilic infiltration of the canine GI tract remains uncertain, medical intervention aiming to decrease the activation of eosinophils seems effective in reducing symptoms and preventing organ damage. This review focuses on the biology of eosinophils and their products. It describes, the composition of eosinophil granules, mechanisms of eosinophil activation, and eosinophil-related disease processes leading to organ damage. Even though the main clinical signs of canine eosinophilic gastroenteritis, vomiting and diarrhea, are similar to those of other types of gastroenteritis, the clinical response and prognosis are worse for this condition. The clinical signs and diagnostic approach for eosinophilic GI disorders are described and compared between canine and human patients for each region of GI tract, from the esophagus to the colon. Moreover, the current treatments for this syndrome in canine and human patients are summarized and paralleled. The comparative study of canine and human patients with eosinophilic gastroenteritis will advance the understanding of this syndrome in both species and may lead to the development of novel treatment strategies.
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Siminas S, Qasem E, Shukla R, Turnock R. Inflammatory fibroid polyp: a rare benign tumor of the alimentary tract in children presenting as intussusception-case report and review of literature. European J Pediatr Surg Rep 2013; 2:16-9. [PMID: 25755961 PMCID: PMC4336055 DOI: 10.1055/s-0033-1354746] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/10/2013] [Accepted: 07/25/2013] [Indexed: 12/18/2022] Open
Abstract
Inflammatory fibroid polyp (IFP) represents a rare cause of gastrointestinal polypoid disease in childhood. Τhe lesion has been described by various names beyond the currently accepted term, including “Vanek's tumour,” eosinophilic or submucosal granuloma, gastric fibroma with eosinophilic infiltration, inflammatory pseudotumor, and hemangiopericytoma. The etiopathogenesis and origin of the mesenchymal spindle-shaped cells that comprise the polyp remains enigmatic. Recent studies have shown familial occurrence, expression of platelet-derived growth factor receptor (PDGFRA) and oncogenic PDGFRA mutations in the majority of lesions, suggestive of a neoplastic nature. We present a rare case of a 10-year-old boy with an IFP of the terminal ileum, who presented acutely with intussusception and was treated with a right hemicolectomy. Postoperative course was uneventful and the patient has been asymptomatic during follow-up. Histopathology and immunohistochemical analysis excluded inflammatory myofibroblastic tumor (negative for Alk1, desmin, smooth muscle actin [SMA]), gastrointerstinal stromal tumors (GIST) (negative for CD117) and schwannoma (negative for S100). The lesion was positive for CD34 and faintly for vimentin. Despite the classification of IFPs as a mesenchymal benign neoplasm, in the vast majority of cases, surgical excision alone was curative, and no reports exist of a malignant transformation. A cautious approach with periodic surveillance of the affected children seems reasonable though.
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Affiliation(s)
- Sotirios Siminas
- Department of Paediatric Surgery and Urology, Manchester Children's Hospital, Manchester, United Kingdom
| | - Eyas Qasem
- Department of Paediatric Surgery, Alder Hey Children's Hospital, Liverpool, United Kingdom
| | - Rajeev Shukla
- Department of Paediatric Histopathology, Alder Hey Children's Hospital, Liverpool, United Kingdom
| | - Richard Turnock
- Department of Paediatric Surgery, Alder Hey Children's Hospital, Liverpool, United Kingdom
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21
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Ingle SB, Hinge (Ingle) CR. Eosinophilic gastroenteritis: An unusual type of gastroenteritis. World J Gastroenterol 2013; 19:5061-5066. [PMID: 23964139 PMCID: PMC3746377 DOI: 10.3748/wjg.v19.i31.5061] [Citation(s) in RCA: 82] [Impact Index Per Article: 6.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/24/2013] [Revised: 06/23/2013] [Accepted: 07/01/2013] [Indexed: 02/06/2023] Open
Abstract
Eosinophilic gastroenteritis (EGE) is a rare disorder characterized by eosinophilic infiltration of the bowel wall with various gastrointestinal manifestations. Till date only 280 cases have been described in the literature. A high index of suspicion, by excluding other causes of peripheral eosinophilia, is a pre requisite for accurate diagnosis. EGE is an uncommon gastrointestinal disease affecting both children and adults. It was first described by Kaijser in 1937. Presentation may vary depending on location as well as depth and extent of bowel wall involvement and usually runs a chronic relapsing course. This condition can respond to low dose steroid therapy, thereby preventing grave complications like ascites and intestinal obstruction that might need surgical intervention. The natural history of EGE has not been well documented. Eosinophilic gastroenteritis is a chronic, waxing and waning condition. Mild and sporadic symptoms can be managed with reassurance and observation, whereas disabling gastrointestinal (GI) symptom flare-ups can often be controlled with oral corticosteroids. When the disease manifests in infancy and specific food sensitization can be identified, the likelihood of disease remission by late childhood is high. GI obstruction is the most common complication. Fatal outcomes are rare.
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22
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Mori A, Enweluzo C, Grier D, Badireddy M. Eosinophilic gastroenteritis: review of a rare and treatable disease of the gastrointestinal tract. Case Rep Gastroenterol 2013; 7:293-8. [PMID: 23904840 PMCID: PMC3728613 DOI: 10.1159/000354147] [Citation(s) in RCA: 27] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/19/2023] Open
Abstract
Eosinophilic gastroenteritis is a rare disease of the gastrointestinal tract characterized by crampy abdominal pain, nausea, vomiting, diarrhea, gastrointestinal bleeding, and weight loss associated with peripheral eosinophilia leading to eosinophilic infiltrates in stomach and intestine, usually in a patient with a prior history of atopy. In this article, we describe our encounter with a 59-year-old female presenting with severe abdominal pain, nausea, vomiting, and weight loss with an extensive evaluation including an upper endoscopy with biopsies resulting in a diagnosis of eosinophilic gastroenteritis. The patient was eventually treated with oral prednisone for three weeks with complete resolution of her symptoms.
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Affiliation(s)
- Amit Mori
- Section on Hospital Medicine, Department of Internal Medicine, Winston Salem, N.C., USA
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23
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Inflammatory fibroid polyps of the gastrointestinal tract: spectrum of clinical, morphologic, and immunohistochemistry features. Am J Surg Pathol 2013; 37:586-92. [PMID: 23426127 DOI: 10.1097/pas.0b013e31827ae11e] [Citation(s) in RCA: 88] [Impact Index Per Article: 7.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Inflammatory fibroid polyps (IFPs) are rare, benign tumors that can arise throughout the gastrointestinal tract. Although the molecular pathogenesis of these lesions has been well characterized, their morphologic features often vary. We report the clinicopathologic findings of the largest series of IFPs to date. A total of 83 IFPs seen at our institution were collected between 1999 and 2012. The specimens included 64 biopsies and 19 resections. A review of the clinical features identified a modest female predominance (47 women and 36 men) with patients ranging in age from 26 to 87 years (mean, 60 y). Involved sites included the esophagus (n=2), stomach (n=31; mainly antrum), small intestines (n=17), appendix (n=1), large intestines (n=31; majority within the rectosigmoid), and anal canal (n=1). Although most patients had a nonspecific presentation, those with small intestinal lesions frequently presented with intussusception. Grossly, the tumors ranged in size from 0.2 to 4.2 cm (mean, 1.7 cm). Histologically, IFPs were centered within the submucosa in all resection specimens, but mucosal extension was found in 74 of 83 (89%) cases. The tumors varied in both cellularity and degree of vascularity. However, the characteristic feature of perivascular onion skinning was present in only 54% (45/83) of the cases. In addition, a short fascicular growth pattern was also noted in 36% (30 of 83) of cases, whereas both features were present in 14 cases (17%). Eosinophils were present in 94% (78 of 83) of cases but varied widely in number from abundant (20/hpf) to sparse (1/hpf). Interestingly, in those cases with sparse eosinophils, prominent hyalinization was also present (11 of 78, 13%). In addition, although the majority of IFPs expressed CD34, 6 of 44 (14%) were negative. No associated dysplasia or malignancy was seen. IFPs represent a diverse set of submucosal-based lesions that commonly extend into the mucosa, making them amenable to endoscopic biopsy. Although their classic histologic features of perivascular onion skinning and predominance of eosinophils are well described, they may alternatively present with a short fascicular growth pattern, a sparse number of eosinophils, and prominent hyalinization.
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Jin JS, Wu CS, Yeh CH, Huang BP, Tsao TY. Inflammatory fibroid polyp of rectum mimicking rectal cancer. Kaohsiung J Med Sci 2013; 29:460-3. [PMID: 23906237 DOI: 10.1016/j.kjms.2012.12.007] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/10/2012] [Accepted: 06/21/2012] [Indexed: 12/21/2022] Open
Abstract
Inflammatory fibroid polyps (IFPs) are rare benign tumors of the rectum. Mutation and activating platelet-derived growth factor receptor alpha (PDGFRA) contribute to tumor development. We present a case of IFPs in the middle rectum that mimic rectal cancer. A 65-year-old woman presented with the symptom of fresh blood in the stool and body weight loss of 6 kg in the preceding 3 weeks. A rectal polypoid tumor was noted upon digital examination. Sigmoidoscopy showed a middle rectal tumor measuring 3 × 2.7 cm with obstruction. Computed tomography (CT) scans of the abdomen showed a rectal tumor that had invaded the sacral bone and was associated with four enlarged lymph nodes greater than 1 cm. The radiological report suggested a diagnosis of rectal cancer with lymph node metastases. To remove the obstruction, the patient was initially treated with excision of the tumor and loop sigmoidal colostomy to the abdomen wall. Total mesorectal resection of rectal and sacral tumor followed 10 days later. Histopathological examination of the rectal and sacral tumor showed proliferation of vessels, fibroblast-like spindle cells, and mixed inflammatory cells, including the plasma cells and eosinophils. The spindle cells were diffusely positive to PDGFRA and were focal positive to CD34 and smooth muscle actin. Based on histopathological and immunohistochemical findings, the diagnosis of IFP is indicated. This was the first reported case of IFPs of the rectum presenting with lymph node enlargement and attachment to the sacrum mimicking rectal cancer.
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Affiliation(s)
- Jong-Shiaw Jin
- Department of Pathology, Tungs' Taichung MetroHarbor Hospital, Wuqi Township, Taichung County, Taiwan
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25
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Ihimoyan A, Chelimilla H, Balar B. An inflammatory fibroid polyp in the hepatic flexure of the colon treated with argon plasma coagulation, endoscopic clipping and polypectomy. Case Rep Gastroenterol 2012; 6:63-8. [PMID: 22423241 PMCID: PMC3304079 DOI: 10.1159/000336401] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/19/2022] Open
Abstract
Inflammatory fibroid polyp (IFP) is a rare benign polypoid lesion of the gastrointestinal tract. Most IFPs occur in the stomach and colonic occurrence is very rare. Histologically IFP is characterized by a mixture of numerous small vessels, fibroblasts and edematous connective tissue associated with marked inflammatory infiltration by eosinophils. We present a rare case of a pedunculated IFP in the hepatic flexure of the colon treated successfully with a combination of argon plasma coagulation, endoclipping and polypectomy. A 74-year-old asymptomatic female underwent a screening colonoscopy in our hospital. A 12-mm pedunculated polyp was found at the hepatic flexure of the colon. After saline injection, we attempted to remove the polyp with a hot snare. However the polyp stalk was extremely difficult to resect despite several attempts with the hot snare. We placed an endoclip at the base of the stalk and then applied argon plasma coagulation at 1.0 l/min and 40 W. After these measures we were able to resect the stalk and the polyp was retrieved. Histologically the polyp was located in the submucosa of the gastrointestinal tract. Proliferation of spindle cells and infiltration of inflammatory cells such as plasma cells and eosinophils were observed. The spindle cells were positive for CD34 and S100 but negative for c-kit and muscle markers. These findings are consistent with a histopathological diagnosis of IFP.
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Affiliation(s)
- Ariyo Ihimoyan
- Division of Gastroenterology, Bronx-Lebanon Hospital, Bronx, N.Y., USA
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26
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Alfadda AA, Storr MA, Shaffer EA. Eosinophilic colitis: epidemiology, clinical features, and current management. Therap Adv Gastroenterol 2011; 4:301-9. [PMID: 21922029 PMCID: PMC3165205 DOI: 10.1177/1756283x10392443] [Citation(s) in RCA: 84] [Impact Index Per Article: 6.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023] Open
Abstract
Primary eosinophilic gastrointestinal disorders (EGIDs) represent a spectrum of inflammatory gastrointestinal disorders in which eosinophils infiltrate the gut in the absence of known causes for such tissue eosinophilia. EGIDs can be subgrouped as eosinophilic esophagitis (EE), eosinophilic gastroenteritis (EG), and eosinophilic colitis (EC). The least frequent manifestation of EGIDs is EC. EC is a heterogeneous entity with a bimodal age distribution, presenting with either an acute self-limited bloody diarrhea in otherwise healthy infants or as a more chronic relapsing colitis in young adults. The pathophysiology of primary EC appears related to altered hypersensitivity, principally as a food allergy in infants and T lymphocyte-mediated (i.e. non-IgE associated) in young adults. In adults, symptoms include diarrhea, abdominal pain, and weight loss. Endoscopic changes are generally modest, featuring edema and patchy granularity. Although standardized criteria are not yet established, the diagnosis of EC depends on histopathology that identifies an excess of eosinophils. Therapeutic approaches are based on case reports and small case series, as prospective randomized controlled trials are lacking. Eosinophilic colitis in infants is a rather benign, frequently food-related entity and dietary elimination of the aggressor often resolves the disorder within days. Adolescent or older patients require more aggressive medical management including: glucocorticoids, anti-histamines, leukotriene receptors antagonists as well as novel approaches employing biologics that target interleukin-5 (IL-5) and IgE. This review article summarizes the current knowledge of EC, its epidemiology, clinical manifestations, diagnosis, and treatment.
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Affiliation(s)
- Abdulrahman A. Alfadda
- Division of Gastroenterology, Faculty of Medicine, University of Calgary, Alberta, Canada
| | - Martin A. Storr
- Division of Gastroenterology, Faculty of Medicine, University of Calgary, Alberta, Canada
| | - Eldon A. Shaffer
- Professor of Medicine, Division of Gastroenterology, Faculty of Medicine, University of Calgary, Teaching Research and Wellness Building, Room 6D48, 3280 Hospital Drive NW, Calgary, Alberta, Canada T2N 4N1
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27
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Hurrell JM, Genta RM, Melton SD. Histopathologic diagnosis of eosinophilic conditions in the gastrointestinal tract. Adv Anat Pathol 2011; 18:335-48. [PMID: 21841404 DOI: 10.1097/pap.0b013e318229bfe2] [Citation(s) in RCA: 72] [Impact Index Per Article: 5.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Eosinophils, a constitutive component of the columnar-lined gastrointestinal tract, play an essential role in allergic responses and parasitic infections. The tissue density of these cells also increases in a variety of conditions of uncertain etiology. With the exception of the esophageal squamous epithelium, in which no eosinophils are normally present, the population of normal eosinophils in the remainder of the luminal gut is poorly defined. Therefore, histopathologists must rely on their subjective judgment to determine when a diagnosis of eosinophilic gastritis, enteritis, or colitis should be rendered. Eosinophilic esophagitis is currently the best defined and most studied eosinophilic condition of the digestive tract; therefore, the confidence in accurate diagnosis is increasing. In contrast, the characteristic clinicopathologic features of eosinophilic conditions affecting other parts of the digestive tract remain somewhat elusive. This review was designed to present pathologists with simple and practical information for the biopsy-based histopathologic diagnosis of eosinophilic esophagitis, gastritis, enteritis, and colitis. It was prepared by critically reviewing more than 200 articles on the topic, along with incorporating evidence accumulated through our own collective experience. We anticipate that by increasing pathologists' confidence in reporting these abnormal but often nameless eosinophilic infiltrates, we can help better define and characterize their significance.
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Affiliation(s)
- Jennifer M Hurrell
- Department of Pathology, VA North Texas Health Care System and University of Texas Southwestern Medical Center, Dallas, USA
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28
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Shahzad G, Moise D, Lipka S, Rizvon K, Mustacchia PJ. Eosinophilic enterocolitis diagnosed by means of upper endoscopy and colonoscopy with random biopsies treated with budenoside: a case report and review of the literature. ISRN GASTROENTEROLOGY 2011; 2011:608901. [PMID: 21991521 PMCID: PMC3168459 DOI: 10.5402/2011/608901] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 11/18/2010] [Accepted: 12/16/2010] [Indexed: 12/15/2022]
Abstract
Intense infiltration of gastrointestinal and colonic mucosa with eosinophils or acidophilic gastroenteritis (EG) is a relatively uncommon picture for a pathologist endoscopist especially outside the pediatric age group and is highly suggestive of an ongoing chronic inflammatory process. Existing literature projected a hypothetical association with allergy but the exact pathophysiology is still unknown. Association with malabsorption, protein losing enteropathy, and refractory ulcers with gastrointestinal bleeding makes the clinical presentation more complicated. We present a unique case of diarrhea and abdominal pain in the clinical presentation with associated peripheral eosinophilia, asthma, and gastroesophageal reflux disease (GERD). The patient's symptoms abated after initiation of budesonide.
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29
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Alfadda AA, Storr MA, Shaffer EA. Eosinophilic colitis: an update on pathophysiology and treatment. Br Med Bull 2011; 100:59-72. [PMID: 22012125 DOI: 10.1093/bmb/ldr045] [Citation(s) in RCA: 51] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/02/2023]
Abstract
BACKGROUND Primary eosinophilic gastrointestinal disorders, a spectrum of inflammatory conditions, occurs when eosinophils selectively infiltrate the gut in the absence of known causes for such tissue eosinophilia. These may be classified into eosinophilic esophagitis, eosinophilic gastroenteritis and eosinophilic colitis (EC). This review focuses on EC: its pathogenesis, epidemiology, clinical presentation, diagnosis and current approach to treatment. SOURCES OF DATA A literature review published in English was performed using Pubmed, Ovid, Google scholar search engines with the following keywords: eosinophilic gastrointestinal disorder, EC, eosinophils, colitis and gastrointestinal. AREAS OF AGREEMENT The basis for primary EC appears related to increased sensitivity to allergens, principally as a food allergy in infants and a T lymphocyte-mediated event in adults. Endoscopic changes are generally modest, featuring edema and patchy granularity. AREAS OF CONTROVERSY Clear clinical and pathological diagnostic criteria of EC and its management strategy. GROWING POINTS Intestinal involvement of EC is primarily mucosal, presenting as a mild self-limited proctitis in infants and self-limited colitis in young adults. Therapeutic approaches based on case reports tend to use either elimination diets to avoid a presumed allergen; agents traditionally used in inflammatory disease or targeted drugs like anti-histamines or leukotriene receptor antagonists. AREAS TIMELY FOR DEVELOPING RESEARCH Prospective randomized controlled trials addressing the disease natural history, possible preventive methods and effective medical approach and long-term prognosis are required.
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Affiliation(s)
- Abdulrahman A Alfadda
- Division of Gastroenterology, Faculty of Medicine, University of Calgary, Teaching Research & Wellness Building, Room 6D48, 3280 Hospital Dr NW, Calgary, AB, Canada T2N4N1
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30
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Silva AC, Levy L, Trindade JC, Mendonça P, Silva C, Lopes AI. Faecal and serum levels of eosinophil cationic protein in a healthy paediatric population. Scandinavian Journal of Clinical and Laboratory Investigation 2009; 67:757-66. [PMID: 17852809 DOI: 10.1080/00365510701308337] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/30/2022]
Abstract
BACKGROUND Eosinophil cationic protein (ECP) has been regarded as an excellent marker of eosinophil activation in various diseases where eosinophil-mediated inflammation plays a role. Recently, it has been suggested as a faecal marker of intestinal inflammation in several immune-mediated diseases with gastrointestinal expression. Owing to the scarcity of information at paediatric age, the establishment of reference values is necessary before further clinical studies. OBJECTIVE To determine faecal and serum ECP levels in healthy children and their association with other biological parameters, thereby providing background additional validation data for this age group. METHODS Faecal and serum ECP levels were available from healthy Caucasian children recruited at a regular outpatient clinic. Exclusion criteria were: chronic illnesses, acute illness, mucosal bleeding and recent pharmacological medication. Faecal and serum ECP levels and faecal a1AT were determined by commercial radioimmunoassay and serum IgE by fluoroenzyme immunoassay Uni-CAP. RESULTS Mean and median faecal ECP levels were 1.93 microg/g and 1.20 microg/g, respectively (range 0.41-22.20),while the corresponding serum ECP levels were 13.50 microg/L and 9.54 microg/L, respectively (range 0.20-74.8). The cut-offs found were 2.80 microg/g and 16.89 microg/L for faecal and serum ECP, respectively. A significant (p=0.001) increase in serum, but not in faecal, ECP levels was found among patients with high peripheral eosinophil blood count. Neither faecal nor serum ECP levels were influenced by serum IgE levels. CONCLUSIONS Faecal and serum ECP levels, as determined in the present study, add background information concerning reference levels at paediatric age for further studies indifferent clinical settings.
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Affiliation(s)
- A C Silva
- Paediatric Department, University Hospital de Santa Maria, Lisbon, Portugal.
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31
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Abstract
Eosinophilic gastroenteritis is a clinicopathological disease affecting both children and adults that is characterized by patchy or diffuse eosinophilic infiltration of the gastrointestinal tract with variable resultant clinical gastrointestinal manifestations. The eosinophil, eotaxin, and Th-2 cytokines are important in pathogenesis of this disease entity. It may be confused with parasitic and bacterial infections (including Helicobacter pylori), inflammatory bowel disease, hypereosinophilic syndrome, myeloproliferative disorders, periarteritis, allergic vasculitis, scleroderma, drug injury, and drug hypersensivity. Obtaining the correct diagnosis is important, and a pathologist usually makes this distinction. Effective treatments include systemic/topical corticosteroids, specific food elimination or an elemental diet, certain drugs, and even surgery. A variety of new therapeutic approaches are now under trial.
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O'Kane AM, O'Donnell ME, McCavert M, Taylor K, Lee J, Wilkinson AJ. Inflammatory fibroid polyp of the ileum causing recurrent intussusception and chronic ischaemia: a case report. CASES JOURNAL 2008; 1:244. [PMID: 18925962 PMCID: PMC2577644 DOI: 10.1186/1757-1626-1-244] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 05/22/2008] [Accepted: 10/16/2008] [Indexed: 12/23/2022]
Abstract
INTRODUCTION Inflammatory fibroid polyp is a rare condition of the gastrointestinal tract which can lead to intestinal obstruction. CASE PRESENTATION We present a case of a 65-year-old woman presenting with a 1-month history of intermittent generalised abdominal pain. Computerised tomography imaging demonstrated intussusception of the small bowel. Histology of the resected small bowel revealed an inflammatory fibroid polyp with evidence of chronic ischaemia related to repeated intussusception. This rare complication of inflammatory fibroid polyps is reviewed. CONCLUSION Although computed tomography is useful in confirming an anatomical abnormality, final diagnosis requires histopathological analysis. Operative resection of the affected segment is recommended.
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Affiliation(s)
- Anna M O'Kane
- Department of Surgery, Belfast City Hospital, Lisburn Road, Belfast, BT9 7AB, Northern Ireland, UK
| | - Mark E O'Donnell
- Department of Surgery, Belfast City Hospital, Lisburn Road, Belfast, BT9 7AB, Northern Ireland, UK
| | - Mark McCavert
- Department of Surgery, Belfast City Hospital, Lisburn Road, Belfast, BT9 7AB, Northern Ireland, UK
| | - Kirsty Taylor
- Department of Radiology, Belfast City Hospital, Lisburn Road, Belfast, BT9 7AB, Northern Ireland, UK
| | - Jack Lee
- Department of Surgery, Belfast City Hospital, Lisburn Road, Belfast, BT9 7AB, Northern Ireland, UK
| | - Alan J Wilkinson
- Department of Surgery, Belfast City Hospital, Lisburn Road, Belfast, BT9 7AB, Northern Ireland, UK
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34
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Pratt CA, Demain JG, Rathkopf MM. Food allergy and eosinophilic gastrointestinal disorders: guiding our diagnosis and treatment. Curr Probl Pediatr Adolesc Health Care 2008; 38:170-88. [PMID: 18522855 DOI: 10.1016/j.cppeds.2008.03.002] [Citation(s) in RCA: 27] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/21/2023]
Affiliation(s)
- C Allan Pratt
- Pediatric Gastroenterology of Alaska, Anchorage, Alaska, USA
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35
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Abstract
Eosinophilic gastroenteritis is an infrequently diagnosed condition that is characterized by prominent eosinophilic infiltration of the stomach or small intestine, generally localized to one level of the intestinal wall; the variable organ locus and wall depth produce heterogeneous clinical presentations. A strong association with atopy is present in most cases, supported by circumstantial evidence and the demonstration of Th-2 proinflammatory cytokine profiles in animal studies. A high degree of suspicion is required to establish the diagnosis, which must be based on intense gastrointestinal eosinophilia. Management is directed toward removal of offending allergens and use of anti-inflammatory agents. Novel and emerging treatments on the horizon are biologic therapies and selective anti-eosinophil agents.
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Talley NJ. Gut eosinophilia in food allergy and systemic and autoimmune diseases. Gastroenterol Clin North Am 2008; 37:307-32, v. [PMID: 18499022 DOI: 10.1016/j.gtc.2008.02.008] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/21/2023]
Abstract
Eosinophilic gastroenteritis is a rare disease characterized by striking tissue eosinophilia in any layer of the gut wall; however, many diseases can cause increased gut eosinophilia. Allergic reactions to food are an important cause of gut eosinophilia. Not all adverse reactions to food are IgE mediated, and most cases of IgE-mediated food allergy do not have eosinophilic gastroenteritis. Parasitic, bacterial, and viral pathogens as well as certain systemic diseases such as vasculitis can cause gut eosinophilia. These heterogeneous conditions are reviewed in this article.
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Affiliation(s)
- Nicholas J Talley
- Division of Gastroenterology and Hepatology, Mayo Clinic Campus, Mayo Clinic, Jacksonville, FL 32224, USA.
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37
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A rare cause of anaemia (2008: 3b). Eur Radiol 2008; 18:1300-2. [PMID: 18463878 DOI: 10.1007/s00330-007-0775-y] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/24/2007] [Revised: 07/27/2007] [Accepted: 08/31/2007] [Indexed: 10/22/2022]
Abstract
Inflammatory fibroid polyp is a rare benign lesion of the gastrointestinal tract, frequently seen in the antrum of the stomach. We report a case of a 50-year-old man with a large inflammatory fibroid polyp and located in the cardia of the stomach. Corresponding computed tomography, endoscopy and histology findings are discussed.
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Sall I, Bouchentouf SM, El Kaoui H, Aitali A, Achour A, Zentar A, Sair K, Janati IM. [Intestinal intussusception on an inflammatory fibroid polyp (Vanek's tumors)]. GASTROENTEROLOGIE CLINIQUE ET BIOLOGIQUE 2007; 31:978-979. [PMID: 18166889 DOI: 10.1016/s0399-8320(07)78309-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/25/2023]
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39
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Khan S, Kandula L, Orenstein SR. Educational clinical case series in pediatric allergy and immunology. Pediatr Allergy Immunol 2007; 18:629-39. [PMID: 18001437 DOI: 10.1111/j.1399-3038.2007.00659.x] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/21/2023]
Abstract
Eosinophilic inflammation may occur in any part of the intestinal tract from the esophagus to the rectum. Despite 70 yr having passed since the first reference to a case of eosinophilic gastroenteritis, the epidemiology and natural history of eosinophilic gastrointestinal disorders are still poorly known. Insights into their etiology and pathogenesis have revealed an important role for allergens; interleukins 4, 5, and 13; the eotaxin family of chemokines; and eosinophil-derived proteins. Diagnosis is confirmed by typical histologic features in a patient with a suggestive clinical phenotype. Treatment involves eliminating triggering allergens, making dietary restrictions the first choice of therapy in a compliant patient; corticosteroids [topical in eosinophilic esophagitis (EE)], despite the potential for serious side effects, are used with success in refractory and non-compliant patients. In this study we discuss EE and gastroduodenitis against the backdrop of clinical case presentations.
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Affiliation(s)
- Seema Khan
- Thomas Jefferson University Medical College, Philadelphia, PA, USA.
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40
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Hirasaki S, Matsubara M, Ikeda F, Taniguchi H, Suzuki S. Inflammatory fibroid polyp occurring in the transverse colon diagnosed by endoscopic biopsy. World J Gastroenterol 2007; 13:3765-6. [PMID: 17659744 PMCID: PMC4250656 DOI: 10.3748/wjg.v13.i27.3765] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
A case of an inflammatory fibroid polyp occurring in the transverse colon and diagnosed by endoscopic biopsy is reported. The patient was an 82-year-old man who visited our hospital for further evaluation of occult blood in stool. The Colonoscopy revealed a small, red, and peduncular polyp, about 6 mm in diameter, in the transverse colon. Histological examination of the biopsy specimen obtained from the polyp revealed proliferation of fibroblasts and infiltration of inflammatory cells such as plasma cells and eosinophils. This polyp was diagnosed as an inflammatory fibroid polyp, which can appear in many different locations throughout gastrointestinal tract, though still rare in the transverse colon.
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Affiliation(s)
- Shoji Hirasaki
- Second Department of Internal Medicine, Sumitomo Besshi Hospital, 3-1 Ohji-cho, Niihama 792-8543, Japan.
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41
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Sheth A, Palmer R, Jordan PA, Manas K, Bhalodia A. Eosinophilic gastroenteritis of the pancreas: an unusual cause of obstructive jaundice. J Clin Gastroenterol 2006; 40:623-5. [PMID: 16917405 DOI: 10.1097/00004836-200608000-00012] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Abstract
Eosinophilic gastroenteritis (EG) is a rare gastrointestinal disorder of undetermined etiology and is manifest by eosinophilic infiltration of any area of gastrointestinal tract, most frequently stomach and small intestine. Peripheral eosinophilia is present in about 80% of patients. Definitive diagnosis requires histologic evidence of eosinophilic infiltration; which is usually patchy in distribution. Steroids are the mainstay of treatment. We present a case of 47-year-old man with abdominal pain, jaundice, and marked eosinophilia. Endoscopic retrograde cholangio-pancreatogram revealed a dilated common bile duct. There was biopsy proven eosinophilic infiltration in stomach, duodenum, gall bladder, and pancreas. Obstructive jaundice is an extremely rare manifestation of EG. This unusual case illustrates the wide variety of gastrointestinal manifestations caused by EG and emphasizes the importance of clinical suspicion and endoscopic mucosal biopsies in diagnosis of EG. This entity should be considered in the patients with chronic and relapsing gastrointestinal symptoms.
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Affiliation(s)
- Ankur Sheth
- Department of Internal Medicine, Louisiana State University Health Sciences Center, Shreveport, USA.
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42
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Abstract
Understanding the mechanisms by which eosinophils migrate into and across the intestinal epithelium can provide alternative therapeutic targets for conditions characterized by eosinophilic cryptitis and crypt abscesses. Eosinophil migration is dependent on adhesion molecules such as selectins. Human eosinophils express L-selectin and P-selectin counterligand P-selectin glycoprotein ligand-1 (PSGL-1). The tetrasaccharide sialyl Lewis(x) (sLe(x)) binds to all three selectins, so compounds that mimic sLe(x), such as TBC1269, are potential antagonists. We hypothesized that eosinophils migrate from the basolateral to the apical surface of intestinal epithelium through the orchestrated effects of selectins. TBC1269 was added to fluorescently labeled HL-60 clone 15 eosinophils as well as human blood eosinophils, in incremental amounts. Subsequently, blocking antibodies toward L-selectin and PSGL-1 were used in a similar manner. HL-60 eosinophils were allowed to migrate into T-84 monolayers. The number of migrated HL-60 cells was calculated by comparing fluorescence with known cell densities. HL-60 and human eosinophils that were undergoing migration were significantly lower in the presence of TBC1269. This effect was concentration dependent, and near complete inhibition of migration was seen at a TBC1269 concentration of 10 mg/mL. In addition, HL-60 eosinophil migration was significantly lower in the presence of the blocking antibodies to PSGL-1 and L-selectin (39.2 and 51.6% inhibition, respectively). Simultaneous blocking of PSGL-1 and L-selectin resulted in inhibition of 76.0% of the migration. The results of this study suggest a major role for selectins in the intestinal epithelial migration of differentiated eosinophils. sLe(x), L-selectin, and the P-selectin counterligand PSGL-1 can be potential therapeutic targets.
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Affiliation(s)
- Sonia Michail
- Department of Pediatrics, Division of Gastroenterology and Nutrition and Wright State University School of Medicine, Dayton, OH 45404, USA.
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43
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Quack I, Sellin L, Buchner NJ, Theegarten D, Rump LC, Henning BF. Eosinophilic gastroenteritis in a young girl--long term remission under Montelukast. BMC Gastroenterol 2005; 5:24. [PMID: 16026609 PMCID: PMC1187886 DOI: 10.1186/1471-230x-5-24] [Citation(s) in RCA: 44] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/05/2005] [Accepted: 07/18/2005] [Indexed: 12/16/2022] Open
Abstract
Background Eosinophilic gastrointestinal disorders are an emerging disease entity characterized by eosinophilic infiltration of the intestinal wall. Oral steroids can be still considered as first line treatment. Unfortunately relapses are quite common. Usually long term low-dose prednisone or immunosuppressive therapy is required, which is especially problematic in young patients. Thus a reliable steroid sparing agent with low side effects suitable for long term use is needed. There are strong hints to a similar pathophysiology of eosinophilic gastrointestinal disorders to that of asthma. Indeed leukotriene D4 plays an important role in the recruitment of eosinophils into the intestinal tissue causing damage. This patho-mechanism provides the rationale for the treatment with a leukotriene D4 receptor antagonist. Recently there have been first reports about successful short term use of Montelukast in eosinophilic gastrointestinal disorders. Case presentation We report the case of a 17 year old girl with a long history of severe abdominal complaints leading to several hospitalizations in the past. Mimicking the picture of an intestinal tuberculosis she received an anti mycobacterial treatment without any success. Marked eosinophilia in blood, ascites and tissue samples of the intestinal tract finally lead to the diagnosis eosinophilic gastroenteritis. Tapering off prednisone caused another severe episode of abdominal pain. At that point leukotriene antagonist Montelukast was started at a dose of 10 mg once daily. Steroids could be tapered off completely within six weeks. The patient has been free of symptoms for over two years by now. Routine examinations, blood tests and endoscopy have rendered regular results. So far no side effects were noted. Conclusion Here report about successful long term remission of eosinophilic gastroenteritis under Montelukast. Further randomized control trials are required to asses the full benefits of Montelukast therapy in the whole spectrum of eosinophilic gastrointestinal disorders.
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Affiliation(s)
- Ivo Quack
- Department of Internal Medicine, Gastroenterology Unit, Marienhospital, Ruhr University, Herne, Germany
| | - Lorenz Sellin
- Department of Internal Medicine, Gastroenterology Unit, Marienhospital, Ruhr University, Herne, Germany
| | - Nikolaus J Buchner
- Department of Internal Medicine, Gastroenterology Unit, Marienhospital, Ruhr University, Herne, Germany
| | | | - Lars C Rump
- Department of Internal Medicine, Gastroenterology Unit, Marienhospital, Ruhr University, Herne, Germany
| | - Bernhard F Henning
- Department of Internal Medicine, Gastroenterology Unit, Marienhospital, Ruhr University, Herne, Germany
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44
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Abstract
Eosinophilic gastroenteritis despite its uncommon occurrence is one of the most important primary eosinophilic gastrointestinal disorders, and most commonly presents with abdominal pain. The terminology is, however, misleading because all levels of the gastrointestinal tract from the esophagus to the rectum may be affected. A history of atopy and allergies is present in 25-75% cases. The heterogeneity in the clinical presentations of EG is determined by the site and depth of eosinophilic infiltration. Eosinophilic intestinal inflammation also occurs secondarily in the gastrointestinal tract in inflammatory bowel disease, autoimmune diseases, as reactions to medications, infections, hypereosinophilia syndrome, and after solid organ transplantation. Recent investigations providing an insight into the pathogenesis of eosinophilic gastroenteritis support a critical role for allergens, eosinophils, Th-2 type cytokines, and eotaxin in mediating eosinophilic inflammation. The diagnosis is confirmed by demonstrating prominent tissue eosinophilia on histopathology. Treatment recommendations based on data extrapolated from retrospective, uncontrolled studies, and expert opinion support the use of restricted diets, corticosteroids, leukotriene receptor antagonists, and mast cell stabilizers. Many unanswered questions remain with regard to the natural history, optimal duration of therapy, safer steroid-sparing long-term treatment agents, and the means of reliable and non-invasive follow-up.
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Affiliation(s)
- Seema Khan
- Division of Gastroenterology, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh, 3705 Fifth Avenue, Pittsburgh, PA 15213, USA.
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45
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Abstract
To evaluate the clinical significance of colonic eosinophilia, we conducted a retrospective study of all children older than 1 year evaluated at Children's Hospital of Pittsburgh from January 1999 to June 2001 with a description of colonic eosinophilia in the pathology report. Medical records were reviewed. Diagnoses were confirmed by contacting the patients. Histological slides (H&E) were reviewed by an investigator blind to the patients' data. Biopsies were grouped according to the site they were taken from and then screened at low power for areas of maximal eosinophilia for further quantitative analysis. Results of manual counts were validated by image analysis using Metaphor Image Analysis Software. Sixty-nine children with colonic eosinophilia were identified (36 male; mean age, 135.2 +/- 55.4 months). Their final diagnoses were inflammatory bowel disease in 32% (group A), irritable bowel syndrome in 33% (group B), food allergies in 10% (group C), and other diagnoses in 25% (group D). The maximal eosinophil count per crypt area was significantly (P < 0.05) higher in group A vs groups B, C, and D (34.8 +/- 17.1 vs 21.3 +/- 8.8, 25.4 +/- 16.7, and 24.2 +/- 9.7, respectively). The total cellularity of the lamina propria was considered high only in group A (P < 0.05 vs groups B and C). A mostly equal vertical distribution of eosinophils throughout the lamina propria was found significantly more frequently in group A vs groups C (P = 0.04) and D (P = 0.007). We conclude that children with inflammatory bowel disease have an equal distribution of eosinophils throughout the lamina propria, with intraepithelial and intracryptal eosinophils and with a higher overall total cellularity. In irritable bowel syndrome and patients with a variety of other diagnoses, including allergies, the distribution is mostly superficial, with a lower total cellularity.
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Affiliation(s)
- Licia Pensabene
- Department of Pediatrics, Division of Pediatric Gastroenterology, Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA
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46
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Mann NS, Leung JW. Pathogenesis of esophageal rings in eosinophilic esophagitis. Med Hypotheses 2005; 64:520-3. [PMID: 15617859 DOI: 10.1016/j.mehy.2004.08.021] [Citation(s) in RCA: 25] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/22/2004] [Accepted: 08/13/2004] [Indexed: 10/26/2022]
Abstract
Eosinophilic esophagitis and eosinophilic gastroenteritis is being recognized more frequently among the adult patients. The disease is characterized by massive infiltration of the wall of gastrointestinal tract by sheets of eosinophils. The clinical features depend upon the site of involvement. They include dyspepsia, dysphagia, nausea, vomiting, chest pain, diarrhea and protein-losing enteropathy. Eosinophilic esophagitis may present as chest pain, dysphagia or dyspepsia. The characteristic endoscopic feature of eosinophilic esophagitis is the formation of fine concentric mucosal rings (corrugated esophagus). Regarding the pathogenesis of these mucosal rings our hypothesis is that mast cells in the esophageal wall in response to allergens release histamine, eosinophilic chemotactic factor and platelet activating factor, etc. which activate eosinophils to release toxic cationic proteins. Activation of acetyl choline by histamine may cause contraction of the muscle fibers in the muscularis mucosae resulting in the formation of esophageal rings. This hypothesis can be tested by demonstrating the contraction of muscle layers of muscularis mucosae with the use of high frequency endoscopic ultrasonic probe introduced via the biopsy channel of an endoscope.
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Affiliation(s)
- N S Mann
- V.A. Medical Center, 150 Muir Road, Martinez, CA 94553, USA.
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47
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Abstract
Eosinophilic gastroenteritis is an uncommon disease with an obscure etiology, although associations with allergy, the idiopathic hypereosinophilic syndrome, and connective tissue disease have been reported. We present the case of a 37-year-old woman with a history of idiopathic thrombocytopenic purpura who presented with refractory nausea, vomiting, and abdominal pain. Imaging studies were significant for bowel wall thickening and ascites, while laboratory studies revealed a positive antinuclear antibody (ANA), a positive anti-double stranded (DS) DNA antibody, low complement, and proteinuria. Exploratory laparotomy with gastric and small bowel biopsies established the diagnosis of eosinophilic gastroenteritis. In addition, the patient met clinical criteria for the diagnosis of systemic lupus erythematosus. Previous studies have described eosinophilic gastroenteritis in patients with scleroderma, polymyositis, or dermatomyositis. This is the first report to our knowledge of an individual with eosinophilic gastroenteritis and systemic lupus erythematosus.
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Affiliation(s)
- David A Barbie
- Department of Internal Medicine, Massachusetts General Hospital, Boston, MA, USA.
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48
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Santos GDC, Alves VAF, Wakamatsu A, Zucoloto S. Inflammatory fibroid polyp: an immunohistochemical study. ARQUIVOS DE GASTROENTEROLOGIA 2004; 41:104-7. [PMID: 15543383 DOI: 10.1590/s0004-28032004000200007] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/21/2022]
Abstract
BACKGROUND Inflammatory fibroid polyp is a localized lesion, which arises in the submucosa of the gastrointestinal tract, most often in the stomach. Although it is generally believed to represent a reactive, nonneoplastic condition, its histogenesis remains controversial. AIM To study inflammatory fibroid polyp by immunohistochemistry in an attempt to further clarify their histogenesis. MATERIAL AND METHODS Nine cases were studied by immunohistochemistry using a panel of antibodies against smooth-muscle actin, vimentin, S-100 protein, factor VIII- R.Ag and macrophage (HAM-56). RESULTS There was a strong diffuse positive staining pattern in the spindle cells with vimentin antibody. A patchy staining for smooth-muscle actin was observed in these cells. Immunophenotyping revealed a heterogeneous reaction with HAM-56. In edematous areas, HAM-56-positive cells show voluminous cytoplasm and reniform nuclei. In cell-rich areas, the HAM-56-positive cells had fusiform cytoplasm. Stains for S-100 and factor VIII RAg were negative in the proliferating elements. CONCLUSIONS The present immunohistochemical study refutes the suggested neural or vascular nature of the lesion. The strong positivity for vimentin in all cases suggests a major component of spindle cells best recognizable as fibroblasts. These results would favor the existence of a span of morphological and immunohistochemical patterns possibly indicating evolutive phases of an inflammatory reaction.
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Affiliation(s)
- Gilda da Cunha Santos
- Department of Pathology, School of Medicine, Federal University of São Paulo, Ribeirão Preto, SP, Brazil.
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49
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Michail S, Abernathy F. A new model for studying eosinophil migration across cultured intestinal epithelial monolayers. J Pediatr Gastroenterol Nutr 2004; 39:56-63. [PMID: 15187782 DOI: 10.1097/00005176-200407000-00012] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/21/2023]
Abstract
OBJECTIVE Eosinophils play an important role in some gastrointestinal inflammatory conditions. Stimulated eosinophils migrate across the vascular endothelial wall and into the intestinal epithelium where by-products such as proteases may contribute to intestinal epithelial damage. Little is known about the epithelial migration of the eosinophils in the gut. The lack of data is attributable in part to the scarcity of human eosinophils for studies. HL-60-differentiated eosinophils present a means to perform studies on eosinophil function and chemotaxis. HL-60 clone 15 can be induced to differentiate into cells closely resembling human eosinophils. The authors describe a novel model for studying eosinophil migration across the intestinal epithelium. METHODS Fluorescent-labeled HL-60 eosinophils were incubated for 150 minutes on the basolateral surface of confluent and inverted T-84 monolayers separated by fluoroblock insert membranes. Chemotactic gradients of n-formyl methionyl leucyl phenylalanine (fMLP), eotaxin, and platelet aggregating factor (PAF) were used in variable concentrations. Changes in transepithelial electrical resistance (TEER) were compared with baseline values. RESULTS Differentiated HL-60 eosinophils undergo migration in response to fMLP, PAF, and eotaxin. Migration is associated with a drop in TEER. CONCLUSION In this model, HL-60-differentiated eosinophils migrate in response to stimulants chemotactic for human eosinophils. The transepithelial migration of eosinophils is associated with epithelial barrier dysfunction, which may contribute to the development of disease.
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Affiliation(s)
- Sonia Michail
- Department of Pediatrics, Division of Gastroenterology and Nutrition, Wright State University School of Medicine, and The Children's Medical Center in Dayton, Ohio, USA.
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50
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Bouhmidi A, Lorente Poyatos R, Romero Cara P, Ibáñez Martín JJ, Casado Caballero F, Quintero Fuientes D, Martín Ruiz JL. [Eosinophilic enteritis as a rare cause of ascites]. GASTROENTEROLOGIA Y HEPATOLOGIA 2004; 26:480-1. [PMID: 14534020 DOI: 10.1016/s0210-5705(03)70398-7] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
Eosinophilic gastroenteritis is an infrequent entity characterized by tissular eosinophilia that can affect different layers of the intestinal wall. This entity can affect any area of the digestive apparatus from the esophagus to the rectum. Clinical manifestations depend on the affected layers and range from barely perceptible symptoms to intestinal obstruction or ascites. We present the case of an 18-year-old woman who showed abdominal ascites as a rare form of presentation with difficult differential diagnosis with peritoneal carcinomatosis.
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Affiliation(s)
- A Bouhmidi
- Hospital Clínico Universitario San Cecilio. Granada. España
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