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Int J Clin Exp Pathol 2011;4(5):430-443

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

Adriana Handra-Luca, Elizabeth Montgomery

Department of Pathology, Johns Hopkins Medical Institutions (JHMI), Baltimore, USA; Sydney Kimmel Comprehensive Cancer Center, Johns
Hopkins Medical Institutions, Baltimore, USA; APHP Université Paris Nord/13 Medecine, Bobigny, France.

Received April 26, 2011; accepted May 28, 2011; Epub June 2, 2011; published June 20, 2011

Abstract: 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. 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 heterogeneously 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. 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. (IJCEP1104010)

Keywords: Vascular malformation, hemangiolymphangioma, gastrointestinal tract, histology, immunohistochemistry

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Address all correspondence to:
Adriana Handra-Luca, MD, PhD
Johns Hopkins Medical Institutions
Department of Pathology
1550 Orleans St. CRB2, 3M41
Baltimore, MD 21231
Tel: 001 410 955 3511
Fax: 001 410 614 0671

Elizabeth Montgomery, MD
Johns Hopkins Medical Institutions
Department of Pathology
401 N. Broadway 2242 Weinberg
Baltimore, MD 21231
Tel: 001 410 6142308
Fax: 001 4432873818