Final Diagnosis -- Primary Hepatic Lymphoma

FINAL DIAGNOSIS:

MALIGNANT LYMPHOMA, FOLLICULAR CENTER CELL TYPE,

LARGE NONCLEAVED CELL PREDOMINANT, DIFFUSE (WORKING FORMULATION: MALIGNANT LYMPHOMA, DIFFUSE LARGE CELL TYPE).

CONTRIBUTOR'S NOTE:

The histopathologic sections demonstrate a diffuse large noncleaved follicular center cell lymphoma. There is sclerosis. The flow cytometric immunophenotypic studies support the diagnosis of a follicular center cell lymphoma. Due to the lack of lymphadenopathy or other organ involement, this was determined to be a primary hepatic lymphoma. Genotypic studies performed on the liver biopsy in the Molecular Diagnostics Laboratory support the diagnosis of a B-cell neoplasm but do not help to further subclassify this lymphoma.

Primary malignant lymphoma of the liver accounts for only 0.4% of all extranodal lymphomas in the US. A recent published series of cases revealed a median patient age of 55 years and a male-to-female ratio of 3.1:1¹. The most common presenting signs and symptoms were epigastric and right upper quadrant pain, weight loss, fever, night sweats, and hepatomegaly. The majority of patients had elevated liver function tests. All patients in the series had no elevation of either alpha-fetoprotein or carcinoembryonic antigen.

While Hodgkin's disease, non-Hodgkin's lymphoma and various leukemias may secondarily involve the liver, virtually all primary hepatic lymphomas are non-Hodgkin's lymphomas. The majority (88.2%) are classified as Diffuse Type, with just over half (57.4%) further identified as Large Cell Type. Seventy-nine percent of the cases are B-cell lymphomas, 6.9% T-cell lymphomas, and the remainder phenotypically unclassified. The overall two-year survival rate is approximately 66%.

REFERENCES

1. Ohsawa M, et al. Malignant Lymphoma of the Liver. Digestive Diseases and Sciences, 37:1105-1109, 1992.
2. Zafran ES, Gauland P. Primary Lymphoma of the Liver. Liver 13:57-61, 1993.
3. Scoazec J-Y, Degott C, Brouss N, et al. Non-Hodgkin's Lymphoma Presenting as Primary Tumor of the Liver: Presentation, Diagnosis and Outcome in Eight Patients. Hepatology 13:870-875, 1991.

Contributed by Steven LaTulippe, MD and N. Paul Ohori, M.D.