Recurrence of Epithelioid Hemangioendothelioma during Pregnancy: Case Report and Systematic Review

Abstract

Introduction: Epithelioid hemangioendothelioma (EHE) is a family of blood vessel tumors originating in blood vessels, bone, brain, kidney, liver, and lung. EHE is more common in women, and chemotherapy, radiation, and surgery have brought few successes.

Case presentation: We present a case of a 28-year-old woman whose EHE recurred during pregnancy, suggesting hormonal involvement. We conducted a systematic review to provide analysis and interpretation of the potential significance of her disease recurring, with fatal outcome, during pregnancy.

Discussion: Very little research has explored the use of individual hormonal markers. Strongly positive expression of placenta growth factor (PlGF) and 17-beta estradiol receptors have been reported. Expression of PlGF is noteworthy in our case, in that our patient's disease quickly and dramatically flared in the 25th week of pregnancy, near the peak in maternal PlGF production. PlGF binds to vascular endothelial growth factor-1 (VEGF-1), and PlGF may accelerate VEGF-induced angiogenesis. Taken together, these factors may explain our patient's EHE recurrence and rapid flare-up during pregnancy. Treatment of EHE with VEGF inhibition, potentially in combination with other antiangiogenic and tumor-inhibiting therapies such as lenalidomide, thalidomide, sorafenib, and sunitinib, may also hold promise.

Figure 1.

Hematoxylin/eosin stain showing epithelioid cells with cytoplasmic vacuoles (magnification ×400).

Figure 2.

Hematoxylin/eosin stain showing epithelioid cells with cytoplasmic vacuoles (magnification ×600).

Figure 3.

Immunohistochemical stain positive for vascular marker CD31 (magnification ×600).

Figure 4.

Immunohistochemical stain positive for vascular marker CD34 (magnification ×600).

Figure 5.

Computed tomography angiography of the chest, showing stable disease in 2008.

Figure 6.

Computed tomography angiography of the chest, showing stable disease in 2011.

Figure 7.

Computed tomography angiography of the chest, following disease recurrence in 2012.

Figure 8.

Timeline of the patient’s case. CT = computed tomography scan.