Fibroid polyps of intestinal tract are inflammatory‐reactive proliferations of CD34‐positive perivascular cells

Wille, P.; Borchard, F

doi:10.1046/j.1365-2559.1998.00433.x

Cited by 48 publications

(25 citation statements)

References 18 publications

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“…In this study, these concentric formations were recognizable in 17 cases (cases 2, 3,5,6,7,8,9,10,11,12,13,14,16,21,22,23,24). Similarly, positive immunohistochemical staining of spindle and stellate cells with antibody against CD34 became one of the diagnostic criteria of Vanek's tumor [8,9].…”

Section: Discussionsupporting

confidence: 53%

Comparison of morphological, immunohistochemical, and molecular genetic features of inflammatory fibroid polyps (Vanek´s tumors)

et al. 2010

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Vanek's tumor (inflammatory fibroid polyp) is a rare benign lesion occurring throughout the digestive tract. Histologically, two patterns can be recognized. Classical Vanek's tumor contains concentric formations of proliferating spindle cells which are CD34 positive. Atypical, inflammatory pseudotumor-like Vanek's tumor lacks concentric formations and is CD34 negative. Recently, mutations in platelet-derived growth factor receptor alpha (PDGFRA) were reported in gastric and small intestinal Vanek's tumors. In this study, KIT exons 9, 11, 13, and 17, PDGFRA exons 12, 14, and 18, and a part of exon 15 BRAF for point mutation V600E were screened in 23 cases of Vanek's tumor, both classical (n = 16) and inflammatory pseudotumor-like (n = 7). No mutations in all analyzed exons of KIT and BRAF and in exon 14 of PDGFRA were detected. Six Vanek's tumors harbored activating mutations in PDGFRA exons 12 (n = 5) and 18 (n = 1) respectively: S566_E571delinsK (n = 1), S566_E571delinsR (n = 4), and D842 del (n = 1). The mutations were detected in the classical (n = 5), as well as inflammatory pseudotumor-like (n = 1) Vanek's tumors. The results of this study suggest that the two morphological patterns of Vanek's tumor more probably represent only variants of one type of tumor than two different lesions. Furthermore, BRAF mutations were not shown to drive growth of PDGFRA wild-type Vanek's tumors.

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Section: Discussionsupporting

confidence: 53%

Comparison of morphological, immunohistochemical, and molecular genetic features of inflammatory fibroid polyps (Vanek´s tumors)

et al. 2010

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“…6 Despite many ultrastructural and immunohistochemical studies suggesting dendritic, fibroblastic, fibrohistiocytic, histiocytic, myofibroblastic, neural, and vascular differentiation, an origin of spindle cells comprising these polyps has remained enigmatic. [7][8][9][10][11][12] A recent study of 23 gastric inflammatory fibroid polyps showed an expression of plateletderived growth factor receptor (PDGFRA) and oncogenic PDGFRA mutations in a majority of analyzed tumors, and suggested that the inflammatory fibroid polyp is a neoplasm driven by activated PDGFRA. 13 The PDGFRA gene maps to chromosome 4q12 and encodes a transmembrane glycoprotein of type III receptor tyrosine kinase.…”

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confidence: 99%

Gain-of-function PDGFRA mutations, earlier reported in gastrointestinal stromal tumors, are common in small intestinal inflammatory fibroid polyps. A study of 60 cases

et al. 2009

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The inflammatory fibroid polyp is a rare benign lesion occurring throughout the digestive tract. It usually forms a solitary mass, characterized by a proliferation of fibrovascular tissue infiltrated by a variable number of inflammatory cells. The etiology of this lesion is unknown and conflicting histogenetic theories have been proposed. Recently, mutations in platelet-derived growth factor receptor (PDGFRA) and PDGFRA expression were reported in gastric inflammatory fibroid polyps. In this study, PDGFRA exons 12, 14, and 18 were screened for activating mutations in 60 small intestinal inflammatory fibroid polyps. In addition, the PDGFRA expression was evaluated immunohistochemically. Mutations in PDGFRA were identified in 33 of 60 (55%) cases, whereas 95% expressed PDGFRA. There were 26 deletions, three deletion-insertions, duplication, and single nucleotide substitution in exon 12, and a single nucleotide substitution and deletion in exon 18. The majority (n ¼ 23) of exon 12 deletions were 1837_1851del leading to S566_E571delinsR. However, 1835_1852delinsCGC leading to the same S566_E571delinsR, were found in two tumors. Three inflammatory fibroid polyps had 1836_1850del leading to S566_E571delinsK. A complex deletion-insertion affecting a similar region (1837_1856delinsGATT-GATGATC) and leading to S566_I573delinsRIDDL was identified once. In addition, duplication and single nucleotide substitution were found 5 0 to the common inflammatory fibroid polyp mutational 'hot spot'. These mutations consist of 1808_1828dup leading to I557_E563dup, and 1821T4A resulting in 561V4D substitution. A 2664A4T and 2663_2674del leading to 842D4V and D842_H845del, respectively, were identified in exon 18. Similar gain-of-function PDGFRA mutations reported in gastrointestinal stromal tumors have been considered to be a driving pathogenetic force. This study showed consistent expression and common mutational activation of PDGFRA in small intestinal inflammatory fibroid polyps as in their gastric counterparts, and these lesions should be considered PDGFRA-driven benign neoplasms. We also suggest that these polyps may develop from earlier described PDGFRA-positive mesenchymal cells distributed along the villus membrane after oncogenic PDGFRA activation.

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“…Microscopically, they are characterized by increased proliferation of mononucleate and monomorphic, bland, spindle, and stellate cells all wound within a fibromyxoid stroma with the dominant inflammatory infiltrate being eosinophils 9 . Regularly also, network of variably sized blood vessels are present 10 .…”

Section: Discussionmentioning

confidence: 99%

Rectal inflammatory fibroid polyp in a Nigerian: case report & brief review of literature

Oluyemi¹,

Nicholas

2016

Afr H. Sci.

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Background: Inflammatory fibroid polyps are very unusual gastrointestinal tumours. Furthermore, their occurrence in the rectum is a rarity indeed. Objective: To document this very rare pathologic entity in a patient from our region of Africa and highlight some pertinent clinical and pathologic aspects of the disease. Case report: We present a 59 year old Nigerian who had neither significant previous medical history nor current complaint and had a routine colonoscopy done. The finding in the rectum was of a small polypoidal mass which was excised whole and whose histopathologic findings were consistent with a diagnosis of inflammatory fibroid polyp. Conclusion: A unique case of rectal inflammatory fibroid polyp in a Nigerian is hereby presented. This, to the best of our knowledge, is the first such documented case from our region of the world. Hence, the entity should be considered in the differential diagnosis of colonic polyps from the continent.

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Fibroid polyps of intestinal tract are inflammatory‐reactive proliferations of CD34‐positive perivascular cells

Abstract: Because of the consistent positivity of the spindle cells of FP for CD34 we suggest an origin of these lesions from primitive perivascular or vascular cells. This origin and a probable relationship to gastrointestinal stromal tumours (GIST) is discussed.

Cited by 48 publications

References 18 publications

Comparison of morphological, immunohistochemical, and molecular genetic features of inflammatory fibroid polyps (Vanek´s tumors)

Comparison of morphological, immunohistochemical, and molecular genetic features of inflammatory fibroid polyps (Vanek´s tumors)

Gain-of-function PDGFRA mutations, earlier reported in gastrointestinal stromal tumors, are common in small intestinal inflammatory fibroid polyps. A study of 60 cases

Rectal inflammatory fibroid polyp in a Nigerian: case report & brief review of literature

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