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Hepatic Epithelioid Hemangioendothelioma (HEHE)
This mouthful of a disease is usually considered a very rare condition, but as pathology interrogation of percutaneous biopsy becomes more exotic, it may be that HEHE is not as rare as one might expect.
We had just completed a course of irreversible electroporation (IRE) in a young female patient who presented with upper abdominal pain. Her subsequent CT scan showed what was thought to be multiple liver metastases. However no primary was evident and histology confirmed the pathology. This course is not unusual and a similar case report with an exhaustive review of the condition was recently published (Case Rep Oncol Med. 2013; 2013: 243939). There are also increasing reports of HEHE in Chinese populations with exposure to hepatitis C and there is a possbility of confusion between HEHE and HCC.
Clinically HEHE patients are younger and appear quite well with vague symptoms, most commonly upper quadrant pain. The liver may be quite enlarged and the tumors are usually multiple with a tendency to coalesce. HEHE may occur in several other sites including brain, meninges, bone and lymph nodes. As the WHO considers HEHE to be a malignant potential condition, liver transplantation is recommended.
Our patient was understandably anxious about the 25% to 30% incidence of recurrence in the transplant or elsewhere. Our patient had the typical gene translocation of HEHE and the only other aetiology we have found is a possible occult Bartonella infection. She developed several new lesions in her first year of our treatment which suggests that she was not one of the group of HEHE who have an indolent nonprogressive course. Since 2010 we had been treating her with Rivampicin for the Bartonella and IRE for the liver masses.
We chose IRE because of the number of liver lesions and the proximity of most of them to vital structures including stomach, right ventricle, diaphragm, and major bile ducts and portal vein branches. Her Bartonella antibodies have disappeared and we have been able to control the liver lesions with multiple sessions of IRE. These were initially at 1 month intervals and lately at longer intervals. He disease has been quiescent for the past 6 months and she is now beginning a 12 month cycle of MR followup. The most useful sequence for us is a delayed liver acquisition with volume acquisition with Primovist (Bayer).
CT shows an arterial blush and then profound washout in the portal venous phase in treated and untreated lesions unless the lesion has fibrosed completely which takes some time to develop. Although there is a Japanese case report of a profound blush on angiography this was not our experience and our MR specialists have been unable to reliably identify which lesions are untreated and which are treated. This has been a significant challenge for follow-up assessment and targeting during IRE.
We have used CT/ultrasound fusion, arterial coil markers, and a Robotic guidance device (MAXIO EX) to help guide the electrode placement. After 3 years we have ablated every lesion at least once, except one small lesion high in segment VIII which appeared to involute spontaneously. The very satisfying thing from our point of view is the complete absence of any arterial, venous of biliary damage even after a large number of IRE ablation sessions. This confirms in our mind the remarkable safety of this technique and suggests that it should be much more widely used even for multiple tumors. Of course in an aggressive tumor the treatment schedule would need to be more compressed.
This patient is naturally happy with the result.
So, the next time you see multiple round, low-attenuation lesions on CT with minimal symptoms in a young female, think HEHE.
