Primary lymphoma of the liver is a very rare malignancy with a frequency of about 0.016% of all cases of NHL. The most frequent form of primary liver lymphoma is diffuse large B-cell NHL which occurs primarily in immunodeficient patients. To determine the primary nature of a hepatic lesion, systemic lymphoproliferative disease should first be ruled out.
Secondary liver involvement as a result of Hodgkin's lymphoma and NHL is more frequent. In advanced cases the incidence varies from 25-50%.
Typical symptoms include weight loss, fever and night sweats, while physical examination often reveals asthenia, hepatomegaly, jaundice and ascitis. Obstructive jaundice may occur as a late manifestation of NHL resulting from encasement of the common bile duct by the tumor. Imaging studies usually reveal a solitary mass in the liver although multiple masses may occur, albeit less frequently.
Primary lymphoma is usually hypoechoic or anechoic on US and hypodense on CT. On pre-con-trast T2-weighted MR imaging, most lesions are homogeneously hyperintense with a SI that is comparable to or higher than that of the spleen (Fig. 2l). Other lesions, however, may be isointense on T2-weighted images but appear as slightly hy-pointense on Tl-weighted images (Fig. 22). Generally, the SI of lymphoma is comparable on "inphase" and "out-of-phase" Tl-weighted images. Lymphomas typically do not show significant enhancement on arterial phase images after the administration of Gd contrast agents, although a slight increase in SI may sometimes be seen in the late portal-venous phase. However, most lesions are isointense with the normal liver parenchyma on Tl-weighted images acquired during the equilibrium phase. The SI of lymphomas that have a peri-
portal distribution is non-specific, which may lead to an initial misdiagnosis of metastasis [17, 31] (Fig. 23).
Most patients are treated with chemotherapy, although some physicians employ a multimodal approach involving additional surgery and radiotherapy. Although the prognosis is variable, a good response may be achieved if aggressive combination chemotherapy is performed early after onset [18,39,56,67,76].
Burkitt's lymphoma is a mature B-cell lymphoma associated with Epstein-Barr-virus that is characterized by rapid proliferation and a propensity for extranodal sites of involvement such as the gastrointestinal tract and central nervous system. It affects primarily children and young adults. Since sonography is often the first imaging procedure performed in these patients, knowledge of the wide range of sonographic appearances is helpful for the recognition of Burkitt's lymphoma.
Burkitt's lymphoma appears to be curable in a high proportion of cases if treated with aggressive multiagent chemotherapy regimens. The use of au-tologous stem cell transplantation appears to benefit patients who have had chemotherapy-sensitive relapses [6,106].
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