Ulcerative colitis, infliximab, and hepatic epithelioid hemangioendothelioma: who is to blame? Case Report

Abstract

Epithelioid hemangioendothelioma (EH) is a rare vascular neoplasm that was first described by Weiss and Enzinger [1982]. The natural history and clinical course of EH is largely unknown given its rarity, but its malignant potential is thought to range between that of a benign hemangioma to a malignant angiosarcoma [Woodall et al. 2008;Mehrabi et al. 2006;Uchimura et al. 2001]. Primary disease can involve soft tissue and visceral organs to include heart, spleen, lung, bone, and lymph nodes [Woodall et al. 2008;Mehrabi et al. 2006]. Primary malignant hepatic epithelioid hemangioendothelioma (HEH) is an exceptionally rare tumor with a reported incidence of less than 0.1 per 100,000, with less than 440 published reports in the literature [Mehrabi et al. 2006]. The exact etiopathogenesis of HEH is unknown but possible etiologic factors have been described to include oral contraceptives, vinyl chloride, asbestos, hepatic trauma, viral hepatitis, primary biliary cirrhosis, and alcohol consumption [Mehrabi et al. 2006].

Epithelioid Hemangioendothelioma of the Bowel in Crohn’s Disease: The First Reported Case

“…[11][12][13] Furthermore, some studies suggest that the use of immunosuppressive drugs in patients with IBD may pose an increased risk for developing neoplasms, most commonly lymphomas. 14,15 A significant proportion of these secondary tumors are not detected prior to bowel resection, and this phenomenon emphasizes the need to carefully dissect and sample these specimens. Only 2 cases of EHE have been described in the setting of IBD: pulmonary EHE in association with CD and hepatic EHE in association with ulcerative colitis, and both patients were treated with anti-tumor necrosis factor agents.…”

Pulmonary Epithelioid Hemangioendothelioma in a Patient with Crohn’s Disease

“…No cases of PEH in the setting of IBD were identified. At least one previous case study has described hepatic epithelioid hemangioendothelioma in association with IBD [ 5 ]. Increased levels of vascular endothelial growth factor have been shown to be upregulated in patients with IBD, suggesting increased angiogenesis in the setting of IBD [ 5 ].…”

“…At least one previous case study has described hepatic epithelioid hemangioendothelioma in association with IBD [ 5 ]. Increased levels of vascular endothelial growth factor have been shown to be upregulated in patients with IBD, suggesting increased angiogenesis in the setting of IBD [ 5 ]. It may be that chronic increase in inflammatory cytokines prompting increased angiogenesis in the endothelium primes this site as a potential nidus for primary disease [ 5 ].…”

“…Increased levels of vascular endothelial growth factor have been shown to be upregulated in patients with IBD, suggesting increased angiogenesis in the setting of IBD [ 5 ]. It may be that chronic increase in inflammatory cytokines prompting increased angiogenesis in the endothelium primes this site as a potential nidus for primary disease [ 5 ]. While there is currently insufficient data to fully explore this, further study may shed light on a possible association between chronic inflammatory conditions and this rare endothelial neoplasm.…”

“…Response to chemotherapy is minimal at best with prognosis varying from complete spontaneous regression to rapid onset of end stage disease. To our knowledge, there is only one case in the literature of a hepatic epithelioid hemangioendothelioma and there are no cases of PEH described in the setting of inflammatory bowel disease [ 5 ]. We report a 14-year-old male with Crohn's disease (CD) and pulmonary epithelioid hemangioendothelioma.…”