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A 15-year-old girl was admitted to our hospital with a 9-month history of upper abdominal pain and loss of appetite. Her history showed no indication of hepatitis. Her abdomen appeared to be even and soft, and the liver and spleen could not be felt below the costal margin. Percussion pain in hepatic region was negative. Gastroscopy showed no abnormalities. An abdominal ultrasound examination revealed the presence of hepatic hypoechoic areas and an abdominal CT scan showed multiple roundlike low-density masses in both hepatic lobes. The edges of the focal lesions were smooth and continuous, with a heterogeneous center, the roundlike edges were enhanced, but the enhancement in the focal lesions was not obvious. The findings suggested that it could be a metastatic tumor (Fig.1). In order to obtain a definitive diagnosis, a liver biopsy was conducted, resulting in a pathological determination of epithelioid hemangioendothelioma. The patient left our hospital because of financial difficulty, without furthure examinations and treatment.
CT scan shows multiple roundlike low density masses in both hepatic lobes.
Discussion
Epithelioid hemangioendothelioma(EHE) is a rare vascular tumor which has been gradually recognized in recent years. It occurs most often in soft tissue, such as lungs, bones, brain and small intestine etc., but rarely in the liver. Hepatic EHE has its origin in mesenchymal tissue and its relative malignancy lies between haemangioma and hemangioendotheliosarcoma.
Based on many cases, EHE accurs most often in adults, especially in females, resulting in a female to male ratio of 1.6 to 1. The average age of the patients is 47 years. The etiology of this tumor is still unknown, but it may relate to use of oral contraceptives, pregnancy hormonal imbalance, vinyl chloride toxicity, hepatic trauma or viral hepatitis. Our patient had no etiological relationship with this tumor except for menstrual disorders. The clinical manifestations of the disease are nonspecific and variable, such as epigastric discomfort or stomachache. Occasionally, symptoms of jaundice, fever, and finally weight loss can appear.
Hepatic EHE is usually multiple, distributing throughout the whole liver making it difficult to use imaging to distinguish hepatic EHE from other hepatic tumors. The diagnosis mainly depends on liver biopsy, showing relatively distinctive organizational and immunohistochemical markers. Histologically, tumors are constituted of dendritic and epithelioid cells, often including vacuoles that fill the cellular atrium. Ground substance which has hyalinization consists of fibra. Immunohistochemistry shows positive reaction for at least one kind of endothelial tumor marker (factor VIII related antigen, CD34 and/or CD31), making diagnosis easy in most cases.
Hepatic EHE is slow-growing and has a low metastatic rate, of about 27%~45%, most commonly metastasizing to the lungs (81%). The prognosis is relatively good, according to some reports indicating that the 5-year survival of EHE patients is 43%[1]. Surgical resection is the main therapeutic method for EHE, EHE is insensitive to radio and chemotherapy. Because of the tumor’s multicenteral origin and multimodal character, it is difficult to treat by operation. Liver transplantation is a possible surgical treatment for an unresectable EHE.
- Received February 15, 2007.
- Accepted March 4, 2007.
- Copyright © 2007 by Tianjin Medical University Cancer Institute & Hospital and Springer
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