Vascular malformations and hemangiolymphangiomas of the gastrointestinal tract: morphological features and clinical impact

Abstract

Purpose: The purpose of our study was to describe the morphological features of gastrointestinal vascular malformations (VM) and of hemangiolymphangiomas (HLA) and to establish correlations with clinical characteristics. Significant findings: Fifteen VMs and 12 HLAs that were encountered over a period of 22 years, were retrospectively analyzed. The VMs often involved the colon, small intestine, but also the stomach, whereas none of the HLAs arose in the stomach. VMs were more frequently associated with gastrointestinal bleeding, ulcer and were larger than HLAs (p<0.01 for all comparisons). Intralesional hemorrhage and thrombosis were associated with VM (p=0.02 and p=0.05). Surgical resection was performed for 1 HLA and 14 VMs. Vessel abnormalities such as shunt vessels, wall tufts (excrescences) and arterialized veins were more frequent in VMs (p=0.01, p=0.04 and <0.01, respectively) whereas aneurysm-like cavities were observed in both lesion types. Mucosal abnormal vessels were observed only in VMs, whereas HLAs were associated with mucosal lymphatic clusters (p<0.01). Most HLAs contained a D2-40 hetero-geneously positive lymphatic component, were Glut-1 negative and CD31 reactive. There was no statistical difference in occurrence of associated autoimmune, tumoral and cardiovascular conditions between the two patient groups.

Conclusions: The results of our study suggest that morphological features such as increased size, ulcer, thrombosis, hemorrhage and presence of aberrant mucosal vessels favor the diagnosis of VM. Co-existence of other clinical conditions such as cardiovascular disease, encountered in association with both lesion types, might exacerbate a tendency towards hemorrhage.

Keywords: Vascular malformation; gastrointestinal tract; hemangiolymphangioma; histology; immunohistochemistry.

Figure 1

Line A. Intestinal hemangiolymphangiomas. Hemangiolymphangiomas consisted of mucosal and submuco-sal proliferations of capillary-type blood vessels (a, hematoxylin and eosin stain, original magnification ×10), and of lymphatic-type vessels (b, hematoxylin and eosin stain, original magnification ×10). D2-40, as well as CD31 were expressed by endothelial cells (arrows) in the lymphatic proliferations (d and e, hematoxylin and eosin stain, original magnification ×40). Line B. Gross view and microscopic features of an intestinal vascular malformation. Macroscopi-cally, there was a hemorrhagic polypoid intestinal lesion (e). On microscopy, the lesion was ulcerated (arrow), throm-botic and hemorrhagic (arrowhead) (f, hematoxylin and eosin stain, original magnification ×2). There was a vascular submucosal cluster (arrow) (g, hematoxylin and eosin stain, original magnification ×2) with a large vessel (arrow) (h, hematoxylin and eosin stain, original magnification ×4). Line C. Abnormal vascular lesions in vascular malformations. Thick walled vessels were observed in the mucosa on biopsy specimen (i, hematoxylin and eosin stain, original magnification ×40). The vascular clusters in the submucosa contained vessels of indeterminate nature or shunt vessels (arrows) (j and k, hematoxylin and eosin stain, original magnification ×10) or aneurysm-like dilatations (arrow) (l, hematoxylin and eosin stain, original magnification, ×4).