Epithelioid hemangioendothelioma of the liver and pregnancy (Literature review and clinical case)

Abstract

The article is devoted to the analysis of scientific publications about the etiology, pathogenetic mechanisms, and management of the patients with epithelioid hemangioendotheliomas.Epithelioid hemangioendothelioma is the vascular tumor that affects liver, spleen, lungs, and various other organs and tissues. The tumor is malignant with high metastatic potential and variable course. The frequency of it is less than 0.1 per 100,000 population. The etiology is unknown. The role of the molecular and genetic mechanisms is studied, and today an association of the WWTR1-CAMTA1 protein in the development of epithelioid hemangioendothelioma was determined.Visual examination, computed tomography and magnetic resonance imaging, which are characterized by typical signs: «white target sign», «lollipop sign» are used for the diagnosis. There are no treatment standards for epithelioid hemangioendothelioma. Chemotherapy, radiofrequency ablation, surgery, and liver transplantation are usedOnly 8 cases of epithelioid hemangioendothelioma during pregnancy have been described in the medical literature, three cases of which are connected with one woman. Among them: progressive liver tumor with negative outcomes – the perinatal and maternal mortality; epithelioid hemangioendothelioma of the liver with the birth of a live, full-term baby; multifocal malignant hemangioendothelioma of the liver with premature birth by cesarean section and the development of sepsis in the mother; palate tumor involving the oropharynx and the birth of a child in physiological terms; presacral tumor, the diagnosis of which was confirmed after pregnancy; recurrent course of intracranial epithelioid hemangioendothelioma with liver and heart damage and three obstetric cases.The article describes a clinical case of epithelioid hemangioendothelioma of the liver in a pregnant woman with a history of resection of the left lobe of the liver. The management of this case with timely diagnosis, delivery at 35 weeks by cesarean section of the child in a satisfactory state is presented. The woman delivered a male infant weighing 2500 g, height 48 cm in satisfactory condition. Thromboprophylaxis was performed for 10 days, lactation was excluded.