Background. Primary hepatic non-Hodgkin lymphoma (PHL) is a rare and diffic
ult to diagnose lymphoproliferative disorder of unknown etiology. It is bel
ieved that the prognosis in affected patients is dismal, consisting of earl
y recurrence and 2 short survival.
Methods. A retrospective cohort review of patients with PHL diagnosed betwe
en 1974 and 1995 at a university cancer center was performed.
Results. Twenty-four patients with PHL were identified. Typically, the dise
ase occurred in middle-aged men (median age, 50 years). The primary present
ing complaint was right upper quadrant abdominal pain, with hepatomegaly fo
und at physical examination. Serum liver enzymes, lactate dehydrogenase, an
d beta -2-microglobulin levels all were elevated, but a-fetoprotein and car
cinoembryonic antigen levels were within normal range. Hypercalcemia was fo
und in 6 of 15 patients who were tested. Six of 10 patients who were tested
were positive for the hepatitis C virus (HCV). Liver scans demonstrated ei
ther a solitary lesion or multiple lesions. Pathologic examination revealed
diffuse large cell lymphoma in 23 patients (96%). Combination chemotherapy
was the mainstay of treatment; surgery consisted of diagnostic biopsy. The
complete remission rate was 83.3%, and the 5-year cause specific and failu
re free survival rates were 87.1% and 70.1%, respectively. HCV infection di
d not appear to influence the outcome of therapy.
Conclusions. The outcome of patients with PHL who are treated with combinat
ion chemotherapy may be more favorable than that reported elsewhere. The fr
equent association of PHL with HCV infection observed in this series warran
ts further investigation. Cancer 2001;92:2023-9. (C) 2001 American Cancer S
ociety.