Hepatic epithelioid hemangioendothelioma: how fast does it grow and which findings could have prevented diagnostic delay?—a case report

Abstract

Background: Hepatic epithelioid hemangioendothelioma (HEHE) is a rare neoplastic disease of varied presentation and unspecific radiological signs in the early stages. The diagnostic delay can lead to metastatic disease, thus increasing the tumor burden and reducing the treatment options. HEHE is usually deemed a slow-growing tumor, but its speed of growth is poorly reported and still unknown.Case Description: In this case report, we documented a HEHE diagnosed in a young woman who had complaints of abdominal pain, weight loss and bloating for a long time. The typical findings observed in histological studies were not promptly recognized in the histological analyzes, even after two laparoscopicguided liver biopsies, delaying the diagnosis until extrahepatic tumor spreading. Findings observed in computed tomography, magnetic resonance imaging and histological studies are presented. The coalescence of nodules and the rising of giant masses, occupying large parts of the liver in a specific time span, were registered and quantified. As opposed to prior reports, the results show that hepatic HEHE can grow rapidly, reinforcing the need of early diagnosis, thus avoiding the complications presented herein. Conclusions:The findings observed via radiological and histological imaging that could have avoided the diagnosis delay are depicted and discussed, showing that HEHE can rise faster than previously documented.

Hepatic epithelioid hemangioendothelioma: pitfalls in the treatment of a rare liver malignancy

“…These uncommon tumors can pose several challenges to the clinician, beginning with establishing the correct diagnosis. HEHE is more common in middle-aged women, but as the accompanying article to this editorial highlights, it can also afflict women in much younger age ( 3 ). Clinically, HEHE is most often asymptomatic as it develops in the normal liver in the absence of cirrhosis.…”

“…The accompanying case-report by Ribeiro et al ( 3 ) highlights several “real-world” challenges that clinicians must be mindful of when dealing with uncommon liver neoplasms, such as HEHE, which do not always fit their textbook descriptions. First of all, although HEHE typically afflicts middle-aged women, in this case the patient was a female in her teenage years, therefore clinicians should not rely on demographics alone to rule-out certain liver neoplasms.…”

Hepatic epithelioid hemangioendothelioma: is it actually “indolent”?

“…In their recently published report, Dr. Ribeiro presented a case of rapidly progressing HEHE with delayed diagnosis ( 10 ). The authors used volumetric data from MRI showing a tumor percentage growth from 9% to 70% of liver, and a tumor growth of 964% over two years.…”