Elastotic Changes in the Gastrointestinal Tract: A Review of Literature

April 25, 2012

[N A J Med Sci. 2012;5(2):78-81.] PDF File

Ines Krammer, MD;* Hallie Kretsinger; Bruno Märkl, MD

Elastotic changes are benign lesions in the gastrointestinal  tract that often appear as polyps and show histologically a remarkable increase in elastic  fibers. Because of their hyaline and amorphous appearance in hematoxylin and eosin (H&E) stained  slides, elastotic changes often resemble amyloidosis. However, they are negative in Congo  red staining. Reviewing the literature we found 35 cases in 12 publications in the English  and French literature since 1985. The results indicate that the patients’ age ranged from  24 to 88 years (mean 58.2, median 58) and presented a balanced gender ratio (M/F = 17/18).  Usually the lesions presented as polyps or irregular mucosal areas. Mostly, they were found  during endoscopic examination in the colon or rectum (16 cases), while six cases were located  in the stomach and only two in the small bowel. Some authors consider the alterations to be  a reactive process, e.g. within (gastric) ulcers, whereas others speculate about a connection  with systemic diseases of the connective tissue (Ehlers-Danlos Syndrome, Pseudoxanthoma elasticum).  Based on our observations we distinguished angioelastosis, showing a relation to submucosal  vessels, as a separate entity from gastrointestinal elastofibroma, that presents the same  histologic morphology as Elastofibroma dorsi. Conclusion: Elastofibromatous changes in the  gastrointestinal tract are quite common benign findings and should be considered as a possible  differential diagnosis in examining gastrointestinal specimens. 

Key Words: gastrointestinal  tract, polyps, elastosis, angioelastosis, elastofibroma gastrointestinal tract, polyps,  elastosis, angioelastosis, elastofibroma 

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INTRODUCTION 

Elastofibroma dorsi was first described in 1961  by Järvi and Saxén1 as a fibrous lesion with abnormal elastic fibers located mostly in subscapular soft tissue.  In other anatomic regions -especially in the gastrointestinal tract- elastotic changes were  found in different locations, including the stomach, small bowel, colon and rectum (see Table 1). In general however, gastrointestinal lesions found only minor reflection in the literature  with single cases reports and small series. Depending on the authors, these changes were termed  elastofibroma, pre-elastofibroma-like lesion, colonic elastoma, elastosis or gastrointestinal  angioelastosis. 

The cases mostly appeared as polyps or polypoid  changes in endoscopic or macroscopic examination, sometimes with surface ulceration.  One case is described with no gross alterations, but was just a coincident finding after  rectal biopsy.2 Because elastotic changes in the gastrointestinal tract (GIT) are not described in common  textbooks they seem to be widely unknown and are not generally accepted as own entity.  This, however, may cause problems in evaluating such specimens which are identifyed by the  clinicians and do not fit into conventional categories of polyp causing lesions. 

Histologically, all of these changes had submucosal  and or mucosal deposits of increased elastic fibers that appeared amorphous and eosinophilic  in H&E stained slides and, thus, amyloid-like. However, when Congo red staining was performed,  the results were always negative, in contrast to elastic staining, which highlighted the  elastic fibers. In some of the cases, relations to submucosal blood vessels are described. 

METHODS 

We performed a literature search within the Medline  and Google Scholar data bases using combinations of the terms elastosis, elastotic,  gastrointestinal, colon, rectum, colorectal and stomach, esophagus. The identified publications  were screened for relevance and the reference lists were checked concerning publications that  were not included yet. The search period was not restricted. 

It is the goal of this review to describe the  spectrum of elastotic changes in the GIT which is an alteration that did not find recognition  in textbooks and can therefore cause diagnostic problems. 

MORPHOLOGY 

In 1985, Enjoji et al. were the first to publish  a case report about an elastofibromatous lesion in the stomach (see Table 1). The female patient  underwent partial gastrectomy because of a gastric ulcer and grossly showed a wide thickening  of the antral wall with rubbery elastic consistency.3 There are 5 further cases that report similar changes in the stomach, mostly in the antrum.4-7 One of these cases also had a gastric ulcer,6 others showed erosion or inflammatory changes,4,7 two patients were suspected to have gastric cancer5,7 and another two had undergone a Billroth-operation.4 Only one of the gastric cases refers to evidence of bilateral subscapular Elastofibroma  dorsi.3


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Table 1. Data of 35 published cases of elastofibromatous changes in the gastrointestinal tract.
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After the first report, others followed concerning  elastofibromas or elastotoic changes not only in the stomach, but also in the colorectal region  and in the small bowel. In the ileum, there are only three cases present, the first of which  grossly appeared as a polyp.8 The second patient suffered from small bowel obstruction with ulceration and hemorrhagic  mucosa.4 The third case was an incident finding in an autopsy case, which disclosed similar changes  in the bronchial system.9

The remaining cases are colorectal biopsy or  colectomy specimens with polypoid, sometimes cobblestone-like alterations.2,4,8,10-13

All 35 full published cases histologically had  elastofibromatous characteristics in common: granular and/or fibrillar, eosinophilic,  amorphous masses within connective submucosal tissue with scattered fibroblasts,  small blood vessels and serrated outlines. Because these changes are highly suspicious for  amyloidosis, Congo red staining was quite often performed, but always with negative results,  as well as von Kossa staining. Elastic staining (Verhoeff’s or EvG, respectively) showed  consistent positivity of the amorphous material in all cases and identified it as elastic  fibers. 

Our group became the first to divide gastrointestinal  elastosis into two main different groups or entities which are explained in the following. 

Angioelastosis 

We suggested the term angioelastosis for a lesions  that shows an association between the densely accumulated elastic fibers and the wall of submucosal  blood vessels.8 Importantly, these elastic fibers have an untruncated structure and can reach the mucosal  layer. Therefore, the vessel component can be missing in normal biopsies lacking submucosal  structures (Figure 1A). In the literature we identified 12 cases according to the descriptions  of the authors we probably would clasify as angioelastosis. We strongly believe that the vast  majority of cases with elastotic changes belong to this group. All of them are located either  in the colorectal region or rarely in the small bowel. 

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Figure 1. Specimens of colonic polyps.
A) H&E staining – Specimen of a colonic polyp with enlargement of the Lamina propria by an accumulation of fibroelastic fibers.
B) Elastica-van-Giesson-staining and higher magnification of A) – the elastic fibers show the typical truncated and globular morphology of an elastofibroma.
C) Elastica-van-Giesson-staining – Specimen of a rectum polyp with amorphous hyalinization of the Lamina propria.
D) Higher magnification of C) reveals a heavy accumulation of elastic fibers.
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Elastofibroma 

Lesions presenting with typical bended and truncated  fibers with serrated outlines are probably true elastofibromas which are better known in the  subscapular region. These alterations are typically located in the submucosal layer of the  stomach causing a remarkable thickening (Figure 1C and 1D). Only 3 cases (2 in the stomach,  one in the sigma) have been published up to now.3,7,12 We found 2 additional cases in our files (unpublished data). In 2009, Kai et al.  published a case of an elastofibroma in the stomach with a dense accumulation of elastic  fibres with a remarkable association to vessels at the border of the lesion. These latter  lesion areas were termed PVFLs (perivascular fibrotic lesions) by the authors.5

It has to be stated that the well-known elastotic  vessel alterations caused by neuroendocrine tunors (NET) do not fit into these categories  and have to be separated from the lesions described in this review. 

The Clinical Meaning of Elastotic Changes  in the GIT 

Based on unpublished data, we estimate the frequency  of elastotic changes or angioelastosis to be about 1-2% of all incoming GIT biopsy specimens  regardless of the indication for the endoscopy.Thus, it is a rare condition but not as exceedingly  rare as originally thought. As mentioned before, these lesions often present as polyps or  irregular mucosal areas. Neoplasia or even malignancy must be ruled out in such cases.  Because the histological appearance is suggestive for amyloidosis Congo stain is performed  often but turns out to be negative. As long as an elastic staining is not performed the cases  remain unclear. On the other hand the endoscopic aspect can be very serious, prompting surveillance  in short intervals because of unsatisfying histological findings. In contrast, elastotic changes  are almost always associated with benign conditions. Previous endoscopic biopsies,  previous radiation, atrophic gastritis and healed or healing ulcerations are the most frequent  underlying findings. Therefore, angioelastosis seems to be a reactive process. In the opposite  there is growing evidence that true elastofibromas are of neoplastic origin with benign behavior. Despite this usually benign nature, some elastotic lesions can cause severe stenosis,  which lead to surgical resection to avoid complete stenosis and again to rule out a malignant  process. 

CONCLUSION 

The number of published cases shows that elastofibromatous  changes in the gastrointestinal tract are not as rare as originally thought. They appear in  the stomach as wall thickening -e.g. related to ulceration or post-interventional status-  as well as in small bowel, colon and rectum, often as polypoid lesions. As the elastic fibers  build amorphous masses, the H&E staining gives the impression of amyloidosis. In order to  diagnose correctly, a Congo staining should be performed to show a negative result,  and an EvG staining should accentuate the increase of elastic fibers. 

Even though the concrete cause of elastofibromatous  changes remains unclear, authors agree that there is probably an association with alterations  such as ulcers, inflammatory processes, radiotherapy or surgical treatment and, therefore,  they can be seen as reactive lesions. Nevertheless, in some cases excessive elastotic changes  are caused by a neoplastic process. It is well known that particular neuroendocrine tumors  (NET) are often accompanied by remarkable elastotic changes. We found elastotic changes also  in association with colonic adenoma (2 cases) and invasive colon cancer. The thesis by Kharsa  et al., stating that systemic diseases of the connective tissue, such as Ehlers-Danlos Syndrome  or Pseudoxanthoma elasticum, are the underlying cause could never (not even in their own study)  be proved.11 There might possibly be a relation to elastofibrosis in other locations in the same patient,  but only two cases are known: one with changes in the small bowel and bronchus,  and a single single case report with changes in the stomach and ‘classic’ Elastofibroma dorsi  located in the scapular region.3,9

We recently assigned two different categories  of elastotic gastrointestinal changes, terming one of them gastrointestinal angioelastosis  to confirm the involvement of submucosal blood vessels. The other one is thought to have exactly  the same histological features as Elastofibroma dorsi and should consistently be termed gastrointestinal  elastofibroma, analogous to the subscapular parent. 

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Ines Krammer, MD;* Hallie Kretsinger; Bruno Märkl,  MD

Institute of Pathology, Klinikum Augsburg,  Augsburg, Germany 

*Corresponding Author: Institute  of Pathology, Klinikum Augsburg, Stenglinstrasse 2, 86156 Augsburg, Tel: +49 821 400 2150,  FAX: +49 821 400 2162. (Email: Ines.Krammer@klinikum-augsburg.de) 

CONFLICT OF INTEREST 

None. 

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REFERENCES  

1. Järvi O, Saxen E. Elastofibroma dorse. 1961;51 (Suppl 144):83-84.
2. Goldblum JR, Beals T, Weiss SW. Elastofibromatous change of the rectum. A lesion mimicking amyloidosis. Am J Surg Pathol. 1992;16(8):793-795.
3. Enjoji M, Sumiyoshi K, Sueyoshi K. Elastofibromatous lesion of the stomach in a patient with elastofibroma dorsi. Am J Surg Pathol. 1985;9(3):233-237.
4. Hobbs CM, Burch DM, Sobin LH. Elastosis and elastofibromatous change in the gastrointestinal tract: a clinicopathologic study of 13 cases and a review of the literature. Am J Clin Pathol. 2004;122(2):232-237.
5. Kai K, Kusano K, Sakai M, et al. Active neovascularization and possible vascular-centric development of gastric and periscapular elastofibromas. Virchows Arch. 2009;454(2):181-188.
6. Lau KN, Sindram D, Ahrens WA, Agee N, Martinie JB, Iannitti DA. Gastric elastofibroma. Am Surg. 2010;76(12):1446-1448.
7. Saint-Paul MC, Musso S, Cardot-Leccia N, et al. Elastofibroma of the stomach. Pathol Res Pract. 2003;199(9):637-639.
8. Märkl B, Kerwel TG, Langer E, et al. Elastosis of the colon and the ileum as polyp causing lesions: A study of six cases and review of the literature. Pathol Res Pract. 2008;204(6):395-399.
9. Schiffman R. Elastofibromatous lesion. Am J Surg Pathol. 1993;17(9):951.
10. Hayashi K, Ohtsuki Y, Sonobe H, et al. Pre-elastofibroma-like colonic polyp: another cause of colonic polyp. Acta Med Okayama. 1991;45(1):49-53.
11. Kharsa G, Molas G, Potet F, Baglin AC, Vaury P, Grossin M. Colonic elastoma. A pathologic study of 7 cases. Ann Pathol. 1992;12(6):362-366.
12. Sakatani T, Shomori K, Adachi H, Hosoda A, Ito H. Elastofibroma of the sigmoid colon. Pathol Res Pract. 2000;196(3):205-207.
13. Vesoulis Z, Ravichandran P, Agamanolis D, Roe D. Elastofibromatous polyp of the sigmoid colon–a case report and review of gastrointestinal elastofibromas. Can J Gastroenterol. 2003;17(4):275-277.
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