Inflammatory Fibroid Polyp Research Articles

Short-term outcome of endoscopic submucosal dissection using a clutch cutter for subepithelial lesions within the esophagogastric submucosa: a Japanese prospective observational study.

The efficacy and safety of endoscopic submucosal dissection using a clutch cutter (ESD-CC) for subepithelial lesions within the esophagogastric submucosa (SELEGSM) has not been investigated. This study aimed to assess the efficacy and safety of ESD-CC for the treatment of SELEGSM. This prospective study included 15 consecutive patients with 18 SELEGSMs diagnosed by endoscopic ultrasonography. The primary outcomes were short-term outcomes including en bloc resection rate, R0 resection rate, procedure time, and complication rate. The secondary outcome was final histological diagnosis. Among the participants, 18 lesions were identified: 12 in the stomach (nine patients) and six in the esophagus (six patients). The en bloc resection rate was 94.4% (17/18). The R0 resection rate was 88.9% (16/18). The median operating time was 39 min, and no instances of perforation or bleeding were observed. The final diagnoses of SELEGSM included six neuroendocrine tumors (33.3%), six granular cell tumors (33.3%), two ectopic pancreases (11.1%), one inflammatory fibroid polyp (5.6%), one leiomyoma (5.6%), one lipoma (5.6%), and one leiomyosarcoma (5.6%). ESD-CC appears to be a technically efficient and safe approach for SELEGSM resection, suggesting its potential as a valuable treatment option.

Benign Tumors and Tumor-Like Conditions of Ampulla and Small Intestine: The PathologyOutlines.com Review.

Small intestine and ampulla can be involved in a heterogeneous group of benign tumors and tumor-like diseases, potentially mimicking malignant neoplasms. In-depth knowledge of them is critical for practicing pathologists since they represent potential pitfalls in routine diagnostic activity. Such conditions include the following: (1) Brunner gland lesions, including Brunner gland hyperplasia and hamartoma; (2) polyps, such as adenoma, hyperplastic, hamartomatous, and inflammatory; (3) mesenchymal proliferations encompassing inflammatory fibroid polyp, inflammatory myofibroblastic tumor, leiomyomas, lipomas, and lipomatosis of the ileocecal valve; (4) fibrosis-associated diseases, such as idiopathic retroperitoneal fibrosis, reactive nodular fibrous pseudotumor, and sclerosing peritonitis; (5) disorders of lymphatic vessels, including lymphangiectasia and lymphangiomas; and (6) other rare conditions/miscellanea, such as enteritis cystica profunda, intussusception, ischemia, and pneumatosis cystoides intestinalis. This review, inspired by the content of the PathologyOutlines website (https://www.pathologyoutlines.com), aims to provide a reference point in this complex scenario, summarizing the essential histopathological features of all these entities for better addressing routine practice and differential diagnoses.

Inflammatory fibroid polyp of the esophagus: a systematic review.

Esophageal inflammatory fibroid polyp (IFP) is a rare benign tumor of the gastrointestinal tract with limited available data on clinicopathologic features and treatment strategies. A systematic review of the literature in PubMed/Medline and Scopus databases was performed for articles concerning esophageal IFP in adult population. A total of 16 studies were identified, involving 16 patients with a Male-Female Ratio of 3:1 and mean age of 50.38 years. Clinical presentation of esophageal IFP included progressive dysphagia in 56.3% of cases, with additional symptoms such as epigastric and retrosternal pain, weight loss, vomiting, and melena. Diagnostic modalities involved endoscopy in all cases, with endoscopic ultrasound (EUS) employed in 50% of cases and tissue biopsy performed during endoscopy in 87.5% of the patients. Therapeutic approach of esophageal IFP consisted of surgical resection in 75% of the patients and endoscopic resection in the remaining 25%, with various surgical procedures employed based on tumor location. Follow-up data, available for 11 patients over a median duration of 15.5 months, indicated two instances of recurrence following endoscopic resection, while the other nine patients remained asymptomatic with no evidence of recurrence. Esophageal IFP is a rare benign tumor of the gastrointestinal tract presenting with dysphagia, regurgitation, and heartburn. Resection, either endoscopic or surgical, is the primary treatment approach. Prognosis for esophageal IFP is favorable, with low recurrence rates. Further research is required to investigate potential risk factors and etiology for this lesion, and to explore novel therapeutic approaches that may improve patient outcomes.

Inflammatory fibroid polyps of small and large intestine: Report of two cases

Inflammatory fibroid polyps (IFPs) are uncommon benign solitary mesenchymal neoplasms which may develop anywhere in the gastrointestinal tract. These submucosal lesions can occur in individuals of any age but are more frequently observed in the 6th or 7th decade of life. Although rarely associated with adenoma or adenocarcinoma, IFPs are typically managed through local excision. The clinical presentation varies depending on the anatomical location of the polyp. Gross examination typically reveals localized submucosal sessile polypoidal masses. Microscopically, IFPs are characterized by inflammatory cell infiltrate, spindle shaped cells and prominent capillaries. These benign lesions have unknown pathogenesis. Below mentioned are two case report of inflammatory fibroid polyps which lead to intussusception.

Inflammatory fibroid polyp– a rare aetiology for adult ileocolic intussusception

We report a case of an adult ileocolic intussusception secondary to an inflammatory fibroid polyp (IFP), a rare and unexpected association between a surgical condition, and an underlying histopathological process. Intussusception is an uncommon surgical diagnosis in adults, and an IFP is rare mesenchymal tumour. Surgical intervention of the intussusception in an adult patient, given the uncertain aetiology of the lead point, is recommended as gold standard.

AB151. SOH24AB_229. Inflammatory fibroid polyp of the small intestine presenting as small bowel obstruction with intussusception: a case report and systematic review of the literature

AB151. SOH24AB_229. Inflammatory fibroid polyp of the small intestine presenting as small bowel obstruction with intussusception: a case report and systematic review of the literature

A large inflammatory fibroid polyp of jejunum with unusual presentation: very difficult to diagnosis in paediatric patient

Inflammatory fibroid polyps (IFP), also known as Vanek tumours, are one of the rarest groups of gastrointestinal tract polyps. They represent 0.1-3.0% of all polyps in this organ system. Most common location is the stomach, mainly the antrum (70%), ileum (19%), and colon (6%). Large polyps of the stomach can cause intermittent obstruction, described as “ball valve syndrome”. This case report, reported in SMS medical college Jaipur in one year six months old child. We report a case of a 1 year 6 months female child with complaint of abdominal lump noted since 7 days, due to this reduced oral intake. On examination single, around 5×5 cm size non-tender lump is situated in left hypogastric region. On CECT abdomen shows mesenteric origin, may be desmoid tumour, Castleman disease, mesenteric haemangioma or inflammatory myo-fibroblastic tumour. Further MRI abdomen suggestive of inflammatory myo-fibroblastic tumour or NHL. Tumour marker study shows serum ferritin and LDH level raised. On exploration revealed a single, 6×5×4 cm lobulated jejunal mass encircle the jejunum, for this resection and anastomosis done. On histopathological report, suggestive of inflammatory fibroid polyp. Patient discharged successfully on 6th pod without any complication. Exploration done and a mass, which encircle the jejunum found. Resection and anastomosis done and patient discharged on 6th pod without any complications. Such an occurrence was incidental in the reported case, which can confuse our diagnosis, so knowledge about this type of disease is very important, especially in paediatrics population.

A rare case of jejunal Vanek’s tumor causing intussusception in an adult: a case report and comprehensive literature overview

Abstract Inflammatory fibroid polyp, or Vanek's tumor, is an uncommon benign small bowel tumor and a rare cause of intussusception in adults. This case involves a 62-year-old man with persistent abdominal pain, diagnosed with jejunoileal intussusception. A 4 cm inflammatory fibroid polyp was discovered during surgery, leading to distal jejunal resection. Despite the rarity of adult intussusceptions, they should be considered in abdominal pain diagnoses. The optimal management approach, whether en bloc resection or initial reduction with limited resection, remains debated.

Gel immersion EMR of small-bowel inflammatory fibroid polyp using double-balloon endoscopy

Video 1A case of an inflammatory fibroid polyp of the ileum that was safely resected using gel immersion EMR with double-balloon endoscopy.

Histopathologic features of inflammatory fibroid polyps and risk of cancer development: A case series

Introduction 
 Inflammatory fibroid polyp (IFP) is seen in the gastrointestinal tract. In most cases, somatic mutations in PDGFRA gene were observed and the true neoplastic origin of this entity was understood. It is a benign mesenchymal tumor and rarely recurs. The aim of the study was to reveal the histopathologic features of our IFP cases and to investigate the risk of cancer in one case.
 
 Materials and Methods
 We performed a retrospective clinicopathological analysis of patients (n=12) who were reported as IFP between 2012 and 2022. Formalin-fixed, paraffin-embedded and hematoxylin-eosin (H&E)- stained slides of the cases were re-examined by two pathologists microscopically. 
 
 Results
 All patients were adults and ranged in age from 30 to 85. Male to female ratio was 1:2. One polyp was localized in small intestine, one in colon, and the others in stomach. Histologically, there were hyperplastic epithelial changes (7/12), low-grade (3/12), and high-grade dysplasia (1/12) accompanying IFPs. One case was associated with early gastric adenocarcinoma.
 
 Discussion
 Although IFP appears as a benign mesenchymal tumor, it should be kept in mind that dysplasia and rarely carcinoma may accompany this lesion. Larger case series are needed to elucidate the mechanisms of dysplasia and carcinoma development associated with IFP.

Multiple Inflammatory Fibroid Polyps in a Female; A case Report

The inflammatory fibroid polyp (IFP) usually presents as a solitary benign polyp that arises from the submucosa of the gastrointestinal tract. It occurs more commonly in males and stomach is the favored site followed by ileum, colon and duodenum. Multiple inflammatory fibroid polyps (IFPs) is a rare occurrence, so we thought of presenting a case of a 40 years old female with multiple inflammatory fibroid polyps (IFPs). She presented with abdominal pain and abdominal fullness. Her C.T. abdomen showed multiple lobulated soft tissue masses causing intussusception. Surgical resection was done, and histopathological examination of these polyps showed features of IFPs. A literature review of 9 cases (including ours) between 2000-2022 is also presented. To our knowledge this is the third female patient with multiple IFPs.

Inflammatory fibroid polyp: A series of 29 cases and a systematic review of the literature

An inflammatory fibroid polyp (IFP) of the gastrointestinal tract is a localized, benign mesenchymal lesion consisting of spindle-shaped stromal cells, eosinophilic granulocytes, and some lymphocytes and plasma cells. The discovery of a frequent mutation of the platelet-derived growth factor receptor A (PDGFRA) gene was the first hint of a gene-regulating process in IFPs. The aim of this study was to investigate the interaction of inflammatory processes and the role of mutation and expression of the PDGFRA gene in the development of IFPs for the first time. We used immunohistochemistry to analyze the composition of inflammatory cells and next generation sequencing (NGS) to provide a broad overview of gene mutations.We report on 29 cases of IFP. The mean age, gender differences, and localization were compatible with the literature. Spindle cell histomorphology was present in 79% of cases showing a typical onion skin-like perivascular arrangement and significantly high CD34 positivity (p = 0.002, Fisher’s exact test). Eosinophilic granulocytes were present in an average density of 60 ± 49/high power field (HPF) (range: 15–200), and there was a significantly higher rate of IFPs larger than 2 cm in size (p = 0.018, Wilcoxon test). All but one cases could be analyzed by NGS. Mutations were observed in 17 cases (60.7%), including 13 (46.4%) mutations in the PDGFRA gene. Among the gastric lesions, mutations were found in exon 18 of the PDGFRA gene with amino acid exchange (Asp842Val) for eight out of 10 cases and in exon 12 in two cases. All three cases in the small intestine revealed mutation of the PDGFRA gene in exon 12. We found no PDGFRA mutation in our colonic cases. PDGFRA expression was significantly correlated with mutations of the same gene (p = 0.005, Fisher’s exact test) and especially with mutations in exon 12 of the same gene (p < 0.001, Fisher’s exact test). Interestingly, three of our cases (10.3%) without mutation or expression of the PDGFRA gene revealed an unusually high concentration of IgG-positive plasma cells (average: 140 ± 26/HPF, range: 110–160) and IgG4-positive plasma cells (average: 87 ± 21/HPF, range: 60–100). For comparison, an IgG4/IgG ratio of more than 0.4 is commonly observed in IgG4-related diseases. Our molecular results were in accordance with 113 genetically analyzed cases published to date. There was a correlation between the IFP site and mutation variants of the PDGFRA gene. IFPs were localized in the stomach in 49.1% of cases, in the small intestine in 47.3%, and in the colon in 3.6%. Exon 12 of the PDGFRA gene was mutated in 41.1% of cases and primarily occurred in the small intestine (82.6%). Exon 18 was mutated in 22.3% of cases and primarily occurred in the stomach (80.0%). The mutated codon interval 566–571 in exon 12 and codon 842 in exon 18 were compatible, as observed in a gastrointestinal stromal tumor. Conclusively, the correlation between mutation and expression of the PDGFRA gene points to different pathways in IFPs. Additionally, our data hint at a morphological but not genetic overlap between IFPs and IgG4-related pseudotumors.

Inflammatory fibroid polyp presenting as intussusception

Inflammatory fibroid polyp is a benign mesenchymal tumor most commonly arising in the stomach and ileum. It needs to be distinguished from other mesenchymal tumors of the GIT which is done with the help of morphological features and with the help of IHC.

Minimally invasive colonoscopy treatment of inflammatory fibroid polyps in the terminal ileum

To identify the characteristics of inflammatory fibroid polyps (IFP) in the terminal ileum and to investigate the methods, safety, and efficacy of colonoscopic minimally invasive dissection and resection therapies for its treatment. Colonoscopy and colonoscopic ultrasonography were used to diagnose patients with protruding mucosal lesions in the terminal ileum, and the results suggested a high suspicion of IFPs. Colonoscopic minimally invasive dissection and resection were performed for these patients, and IFP was confirmed by postoperative pathological examination and immunohistochemical staining. Twelve cases of IFP from April 2016 to December 2020 in our hospital were examined pathologically and immunohistochemically. The IFPs in the terminal ileum were all successfully excised by colonoscopy. There were no postoperative perforation, bleeding, or recurrence cases during the follow-up. The features of the lesions, as well as the efficacy of colonoscopic minimally invasive dissection and resection, were reviewed. Terminal ileum IFPs have corresponding colonoscopic and endoscopic ultrasonographic features. For IFPs less than 2 cm in size and within 10 cm of the ileocecal valve, removal by colonoscopy was proven less invasive, safe, and effective.

A Rare PDGFRA Exon 15 Germline Mutation Identified in a Patient With Phenotypic Manifestations Concerning for GIST-Plus Syndrome: A Case Report and Review of Literature.

Molecular alterations in PDGFRA are well-described as drivers of sporadic gastrointestinal stromal tumors (GISTs) and inflammatory fibroid polyps (IFPs). However, a small number of families with germline PDGFRA mutations in exons 12, 14, and 18 have been reported, forming the basis of an autosomal dominant inherited disorder with incomplete penetrance and variable expressivity, now referred to as PDGFRA-mutant syndrome or GIST-plus syndrome. Phenotypic manifestations of this rare syndrome include multiple gastrointestinal GISTS, IFPs, fibrous tumors, and other variable features. Herein, we report the case of a 58-year-old female who presented with a gastric GIST and numerous small intestinal IFPs, found to harbor a previously undescribed germline PDGFRA exon 15 p.G680R mutation. Somatic tumor testing was performed on the GIST, a duodenal IFP, and an ileal IFP utilizing a targeted next-generation sequencing panel, revealing additional and distinct secondary PDGFRA exon 12 somatic mutations in each of the 3 tumors. Our findings raise important considerations regarding mechanisms of tumor development in patients with underlying germline PDGFRA alterations and highlight the potential utility of expanding currently available germline and somatic testing panels to include exons outside the typical hotspot regions.

Endoscopic resection of a huge Vanek's tumor (inflammatory fibroid polyp).

A 43-year-old man vomited a “sausage-like” mass into the mouth after drinking a bottle of beer; the mass was then re-swallowed. He subsequently experienced intermittent retrosternal discomfort. Esophagogastroduodenoscopy (EGD) at the local hospital showed a huge esophageal tumor. The patient was transferred to our hospital for further endoscopic resection.

Identification of an Activating PDGFRA Deletion in a Novel Sinonasal Soft Tissue Neoplasm.

Spindle cell tumours in the sinonasal area are diagnostically challenging. We identified a neoplasm that defied histopathological classification using current criteria. The case was subjected to histopathological, immunohistochemical and molecular analysis using a large small variant DNA panel. The tumour comprised cytologically bland epithelioid spindle cells with a rich vasculature, which lack expression of actin and other smooth muscle markers, CD34 and beta-catenin. An activating insertion/deletion in exon 12 of the PDGFRA gene was detected. This alteration has previously been described in gastrointestinal stromal tumours and inflammatory fibroid polyps of the GI tract, but the site, histological, and immunophenotypic features in this tumour are distinct. We describe a novel sinonasal spindle cell tumour characterised by an activating insertion/deletion in exon 12 of PDGFRA. The diagnosis of PDGFRA-activated sinonasal spindle cell tumour should be considered in difficult to classify mesenchymal lesions at this site.

GLI1 Immunohistochemistry Distinguishes Mesenchymal Neoplasms With GLI1 Alterations From Morphologic Mimics.

Glioma-associated oncogene 1 ( GLI1 ) alterations have been described in pericytoma with t(7;12), gastroblastoma, plexiform fibromyxoma, and an emerging class of GLI1 -rearranged or amplified mesenchymal neoplasms including "nested glomoid neoplasm". The immunophenotype of these tumor types is nonspecific, making some cases difficult to diagnose without sequencing. The utility of GLI1 immunohistochemistry (IHC) in distinguishing nested glomoid neoplasms and pericytomas with t(7;12) from morphologic mimics is unknown. To investigate the diagnostic value of GLI1 IHC, we determined its sensitivity and specificity in a "test cohort" of 23 mesenchymal neoplasms characterized by GLI1 alterations, including 12 nested glomoid neoplasms (7 GLI1 -rearranged, 4 GLI1 amplified, and 1 unknown GLI1 status), 9 pericytomas with t(7;12), 1 gastroblastoma, and 1 malignant epithelioid neoplasm with PTCH1 :: GLI1 fusion. GLI1 IHC was 91.3% sensitive in this cohort; all tumors except 2 pericytomas with t(7;12) expressed GLI1. GLI1 was also expressed in 1 of 8 (12%) plexiform fibromyxomas. Nineteen of 22 GLI1-positive tumors showed nuclear and cytoplasmic staining, while 3 showed nuclear staining only. GLI1 IHC was 98.0% specific; among morphologic mimics [40 well-differentiated neuroendocrine tumors, 10 atypical lung carcinoids, 20 paragangliomas, 20 glomus tumors, 20 solitary fibrous tumors, 10 Ewing sarcomas, 10 alveolar rhabdomyosarcomas (ARMS), 10 BCOR -altered sarcomas, 10 myoepitheliomas, 9 myopericytomas, 9 epithelioid schwannomas, 9 ossifying fibromyxoid tumors, 10 biphasic synovial sarcomas, 10 PEComas, 31 gastrointestinal stromal tumors, 10 inflammatory fibroid polyps, 11 pseudoendocrine sarcomas], 5 of 249 tumors expressed GLI1 (2 well-differentiated neuroendocrine tumors, 1 ARMS, 1 Ewing sarcoma, 1 BCOR -altered sarcoma). GLI1 IHC was also performed on a separate cohort of 13 molecularly characterized mesenchymal neoplasms in which GLI1 copy number gain was identified as a putatively secondary event by DNA sequencing (5 dedifferentiated liposarcoma [DDLPS], 2 adenosarcomas, 2 unclassified uterine sarcomas, 1 leiomyosarcoma, 1 ARMS, 1 intimal sarcoma, 1 osteosarcoma); 2 DDLPS, 1 ARMS, and 1 unclassified uterine sarcoma expressed GLI1. Lastly, because pleomorphic sarcomas sometimes show GLI1 amplification or copy number gain, GLI1 IHC was performed on a separate "pleomorphic sarcoma" cohort: GLI1 was expressed in 1 of 27 DDLPS, 1 of 9 leiomyosarcomas, and 2 of 10 pleomorphic liposarcomas, and it was negative in 23 well-differentiated liposarcomas and 9 unclassified pleomorphic sarcomas. Overall, GLI1 IHC was 91.3% sensitive and 98.0% specific for mesenchymal tumor types with driver GLI1 alterations among morphologic mimics. GLI1 expression was less frequent in other tumor types with GLI1 copy number gain. Given its specificity, in the appropriate morphologic context, GLI1 IHC may be a useful diagnostic adjunct for mesenchymal neoplasms with GLI1 alterations.

Inflammatory Fibroid Polyps and Platelet-Derived Growth Factor Receptor Mutation Analysis

Aim: Inflammatory fibroid polyp is one of the most rarely seen tumors in the gastrointestinal tract. It is clinically confusing as it can cause many different findings. On the other hand, it may histopathologically mimic a malignant lesion. Our knowledge about the presence of platelet-derived growth factor receptor α (PDGFRA) mutation in inflammatory fibroid polyps are increasing. Cases: Herein, histopathological and immunohistochemical findings of three different tumors, two localized in the small intestine and one in the stomach, were interpreted as compatible with inflammatory fibroid polyp. PDGFR mutation analysis was performed in both cases and the presence of mutation was detected. Conclusion: The pathogenesis and histogenesis of inflammatory fibroid polyps are uncertain. We obtained results supporting the presence of PFGR mutations in IFPs mimicking both benign and malignant tumors.

Jejunal Intussusception Secondary to Large Inflammatory Fibroid Polyp

Abstract Introduction/Objective Inflammatory fibroid polyp (IFP) is a benign mesenchymal lesion that can arise throughout the gastrointestinal tract but are most common in the stomach, followed by the ileum and more rarely in the duodenum and jejunum. Clinical manifestations include intestinal obstruction, abdominal pain, intussusception, and bleeding. Histologically these are characterized by submucosal location, bland spindle stromal cells, abundant eosinophils and lack of lineage specific markers. Methods/Case Report We report the case of a 68-year-old woman with history of recurrent small bowel obstruction due to Crohn’s disease who presented with jejunal intussusception that required ileocolic resection. Gross examination revealed a 4.2 cm well-circumscribed, lobulated mass with a gray-white and fleshy cut surface. Histologic sections showed a submucosal, nodular proliferation of bland spindle cells embedded within a loose, edematous stroma associated with abundant eosinophils. No hypercellularity, necrosis, hemorrhage, or mitotic figures were identified. Immunohistochemical studies revealed that the lesional cells were positive for vimentin and negative for desmin, SMA, S100, CD1a, CD117, DOG1, ALK, Melan-A, HMB-45, CD34, and STAT6. Ki-67 proliferative index was &amp;lt;1%. The mass was classified as an IFP. Results (if a Case Study enter NA) NA Conclusion IFPs usually express CD34 and negative cases have been described as atypical IFPs. Activating mutations of PDGFRA appear to define this lesion molecularly and are responsible for the recruitment of eosinophils. PDGFRA mutations have been detected in both CD34 positive and negative cases suggesting they represent variants of the same entity. The putative origin of this lesion is hypothesized to be a distinct type of stromal cell: the telocyte. Surgical excision is curative for symptomatic IFP.