Adult primitive neuroectodermal tumor (PNET) of the orbit is an extremely rare malignant tumor. We report the case of a 37-year-old woman with PNET metastasis of the liver 3 years after treatment of the primary right intraconal orbital PNET with resection and chemoradiation adjuvant therapy. Literature review revealed eight previous cases of orbital PNET, but this is the first case report of liver metastasis arising from orbital PNET.

Epstein-Barr (EB) virus infection is common, with up to 90% of individuals demonstrating positive titers by age 20. Although elevated liver function tests commonly occur, severe hepatitis is rare. Only six cases of ascites complicating Epstein-Barr infection are reported, but none clearly demonstrate the absence of other causes of hepatic dysfunction. A 37-yr-old male presented with a 4-wk history of upper respiratory tract symptoms. Over 3 days before admission he developed jaundice and right upper quadrant pain. After hospitalization, the patient developed tense ascites requiring paracentesis. Serum-ascitic albumin gradient was 0.3 g/dL. Liver function tests peaked at the following values: prothrombin time of 24.5 s, total bilirubin of 18.0 mg/dL, and transaminases in excess of 5000 IU/L. EB Virus IgG and IgM titers were 1:640 and >1:40, respectively. Other viral serologies and polymerase chain reactions were negative. The patient experienced a complete clinical and laboratory recovery over the next 6 months. This represents the first documentation of ascites complicating Epstein-Barr infection without other sources of hepatic dysfunction. It demonstrates a narrow serum-ascitic albumin gradient in these patients, and that complete recovery can occur with supportive care.

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