Peripheral T-cell lymphoma arising in the liver.
We report 3 cases of primary hepatic peripheral T-cell lymphoma (PTCL). All patients were men, 50 to 57 years of age, who sought care because of systemic symptoms including fever, fatigue, and weight loss. Physical examination revealed hepatomegaly in 2 patients, associated with jaundice in 1. Two patients had abnormal serum liver enzyme levels and coagulation profiles. Imaging studies demonstrated marked hepatomegaly without focal lesions in 1 patient and multiple discrete tumor masses in 2 patients. Tumor infiltrates in biopsy specimens were heterogeneous with a large cell component in 2 cases. An inflammatory background was present in all cases, complicating the histologic recognition of PTCL Immunohistochemical studies showed that all tumors were of T-cell lineage, and 2 cases had monoclonal T-cell receptor gamma chain gene rearrangements. One patient died of disease shortly after diagnosis, and 2 patients treated with multiagent chemotherapy are in clinical remission with 12 and 84 months of clinicalfollow-up, respectively. PTCL may rarely arise in the liver. These neoplasms respond to chemotherapy, suggesting that this disease is curable if diagnosed at an early stage.
Stancu
Mirela
M
Division of Pathology and Laboratory Medicine, University of Texas M.D. Anderson Cancer Center, Houston 77030, USA.
Jones
Dan
D
Vega
Francisco
F
Medeiros
L Jeffrey
LJ
D019070:Cell Lineage; D004273:DNA, Neoplasm / Q000032:analysis; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor / Q000276:immunology; D006505:Hepatitis / Q000473:pathology; D006529:Hepatomegaly; D006801:Humans; D007150:Immunohistochemistry; D008099:Liver / Q000473:pathology; D008113:Liver Neoplasms / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D016411:Lymphoma, T-Cell, Peripheral / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D016133:Polymerase Chain Reaction; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000235:genetics; D013601:T-Lymphocytes / Q000276:immunology / Q000378:metabolism / Q000473:pathology
D004273:DNA, Neoplasm; D016692:Receptors, Antigen, T-Cell, gamma-delta