DEAR DR. GOTT: In September my wife was diagnosed with epithelioid hemangioendothelioma in her liver. Because of the size and aggressiveness, the team at Yale-New Haven Hospital has recommended a liver transplant. Dr Gott, my wife is 43 years old, the mother for four and other than this, she is very healthy. We need help and direction.
DEAR READER: Epithelioid hemangioendothelioma, otherwise known as EHE, is an uncommon vascular tumor with variable-sized nodules that can be found on tissue or in any organ. It frequently occurs in patients from the ages of 10 to 73 with the lower extremities most commonly involved. Many patients have a slowly progressive, low-grade malignant tumor but survive for decades. Radiological diagnosis is generally difficult because of the presence of multiple nodules that simulate metastatic carcinoma. The disorder was first reported in medical journals some 27 years ago.
Clinical manifestations vary from a complete lack of signs and symptoms to portal hypertension and liver failure. When symptoms are present, they initially include right upper quadrant pain and weight loss. An overwhelming number of patients will be found to have increased alkaline phosphatase and bilirubin levels, coupled with other more complex laboratory abnormalities. Most tumor markers, to include a CA 19-9 may be found to be within normal range but testing allows researchers to rule out other primary or metastatic liver tumors.
Liver transplantation is now the first choice for curative treatment. In the past, liver resection was preferred; however, many patients cannot undergo such a procedure because of multiple lesions or anatomic difficulties and palliative options such as chemotherapy and radiotherapy aren’t particularly endorsed as a method of treatment. This particular type of tumor can respond aggressively following liver resection.
EHE is rare and the treatment strategy must be determined on a case by case basis. Considerations include the rate of progression and the severity of signs and symptoms a patient presents with. Therefore, if your wife’s surgeon is endorsing liver transplant, I am sure he is fully aware of the complexity and intensity of her condition and deems the procedure appropriate. She has the advantage of being young, has a caring husband and four children I am sure she loves dearly. The incentive for full recuperation is great. Any procedure, no matter how simple or complex, is difficult for me to endorse. Nonetheless, my guess is that you are teamed up with a good surgeon in a very reputable facility. Your wife has a great deal to look forward to and should do well. I wish you well.
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