JRSM >
Volume 98, Number 8 >
Pp. 364-365
1 Medical School, University of Birmingham B15 2TH, UK
2 Liver Unit, Queen Elizabeth Hospital, Birmingham B15 2TH, UK
3 Department of Pathology, Queen Elizabeth Hospital, Birmingham B15 2TH,
UK
Correspondence to: Mr Simon Bramhall FRCS
Primary liver tumours have a varied prognosis. Orthotopic liver transplantation has the potential to cure some of these cancers, and these cases need to be identified.
CASE HISTORY
A woman of 47 reported a dull ache in her right shoulder which, over the next few months, worsened and moved to her abdomen; it was associated with epigastric fullness, nausea, anorexia and tiredness. Fifteen months from symptom onset she had lost 5 kg and was experiencing a grabbing right upper quadrant and epigastric pain on deep breathing and coughing. She was unable to lie flat because of the pain. Initially, dietary treatment for gallstones slightly lessened the symptoms, but at twenty-three months the pain was incapacitating—severe, though fluctuating, and now extending to the left upper quadrant. She was admitted for investigation.
On examination there were no stigmata of chronic liver disease, masses or organomegaly. Ultrasound showed no abnormalities, and ultrasound-guided liver biopsy at this stage was inconclusive. Her alcohol history was 1–2 units per week. The differential diagnosis included acute-on-chronic cholecystitis, peptic ulceration and recurrent acute pancreatitis. A second ultrasound-guided liver biopsy then showed hepatic epithelioid haemangioendothelioma (Figure 1). CT revealed several focal lesions, each up to 3 cm in diameter. She was referred to the regional liver unit, where orthotopic liver transplantation was performed nine months later. Figure 2 shows a slice of the explanted liver, with several discrete and coalescing pale lesions. Serum aspartate aminotransferase was normal by day 8 and the patient returned to work after ten weeks. Sixteen months postoperatively she was in good health, and on ultrasound scanning the liver was normal in shape and echotexture, with no focal lesions or biliary dilatation. There was no free fluid in the abdomen, the spleen and kidneys were of normal size and liver function tests were within the normal range.
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Hepatic epithelioid haemangioendothelioma is a rare multifocal vascular tumour1 that can develop at any age, with a female preponderance. The aetiology is unclear, although vinyl chloride, liver trauma and exogenous hormones1 have come under suspicion. Survival of affected individuals has ranged from a few months to many years. On the spectrum of malignancy haemangioendothelioma lies between benign haemangioma and malignant angiosarcoma.1,2 There are several diagnostic difficulties. First, the presentation is non-specific; second, liver function tests may not become abnormal until the disease is advanced; third, ultrasound scans of the liver can be inconclusive; and, finally, CT may lead to a misdiagnosis of metastatic disease because of the tumour's multifocal nature. On CT, multiple angiogenic lesions, 1–3 cm in diameter,3 are a common feature. The peripherally placed lesions are hypodense in the centre and cause enhancement of contrast media at the periphery. In a patient with an abnormal liver ultrasound or CT, without a history of malignant disease or chronic liver disease, a guided liver biopsy may be appropriate. The biopsy appearance in the present patient was typical, with spindle-shaped tumour cells infiltrating the sinusoids and blood vessels. Intracytoplasmic vacuoles may be found, some containing red blood cells.3 Over time the tumours become sclerotic and calcified, and may coalesce to form a large fibrotic mass. Endothelial markers include factor VIII antigen, CD34 and CD31.
There are few treatment options. Chemotherapy and radiotherapy yield no improvement in survival,1 and surgical excision is usually impossible because of the multifocal nature of the disease; indeed, attempted resection may provoke aggressive regrowth. Orthotopic liver transplantation offers a possibility of long-term cure,5 although some patients do well without any treatment. How these 'indolent' cases can be identified is unknown. Experience of transplantation for this tumour at the Liver Transplant Unit in Birmingham is as follows. The first patient, transplanted in 1984, lived for 7 years; she died of lymphoproliferative disease. The second patient, transplanted in 1996, has recently been shown to have lung metastases; there is uncertainty whether metastases affect overall survival, and so far no treatment has been given. Three patients transplanted in 2003 are alive at the time of writing. Out of nearly 40 000 liver transplants undertaken in Europe up to 2001, only 11% were for cancers6 and 66 of these were for epithelioid haemangioendothelioma. 5-year survival is estimated at 55–71%.7
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