Primary hepatic lymphoma: A rare form of extra-nodal Non-Hodgkin Lymphoma

A 54-year-old male presented with a 2-week history of right upper quadrant pain, low-grade fever, and weight loss. Liver function tests were recorded as ALT- 36, AST- 66, total bilirubin- 86, conjugated bilirubin- 71, GGT- 568, Alkaline Phosphatase- 607, and Albumin- 22. AFP level was not raised (2.1 IU/ml).

Ultrasound of the abdomen showed heterogeneous liver texture with ill-defined hypoechoic nodules. On triphasic computed tomography of the liver, non-enhancing hypodense nodules were seen throughout both the hepatic lobes of the enlarged liver. Subsequent magnetic resonance imaging also demonstrated non-enhancing liver lesions scattered throughout the liver, which were hyperintense on T2W FAT SAT imaging, with restricted diffusion [Fig. 1-4]. No biliary dilatation was noted. Porta hepatis lymph nodes were not significantly enlarged. Vascular invasion was not seen. It was decided to go for a liver biopsy for a conclusive diagnosis. Biopsy revealed primary hepatic lymphoma (DLBCL; diffuse large B-cell lymphoma).

Primary hepatic lymphoma (PHL) is a rare form of non-Hodgkin lymphoma (NHL), that is more common in adolescents and young adults and has a rapidly progressive clinical course, and poor outcome due to its refractory behaviour to conventional chemotherapy regimens [1]. Patients commonly present with constitutional symptoms, hepatic and liver enlargement, and cytopenias. The rarity of this disease, along with the lack of nodal involvement and presenting symptoms that mimic different entities including infectious etiologies [2]. PHL can present as solitary or multiple nodules, raising suspicion for carcinoma on imaging, or may mimic benign inflammatory conditions, posing a diagnostic challenge for both hepatologists and radiologists [3]. In our case, multifocal hepatocellular carcinoma was excluded because of non-arterial enhancement of liver lesions, and normal AFP levels. Micro-abscesses, lymphoma, and metastases remained in the differential possibilities. Tissue biopsy was therefore opted for a definitive diagnosis that revealed diffuse large B-cell lymphoma (DLBCL). Radiologists should be aware of the radiological features of liver lymphoma and keep it in differential possibilities of multiple liver lesions with no evidence of vascular invasion or contrast enhancement, and no associated lymphadenopathy or spleen involvement [4]. The patient was referred to a specialist centre where he was offered a chemotherapy regimen (CHOP; cyclophosphamide, doxorubicin, vincristine, and prednisone). He had mild clinical improvement when contacted last week on the phone. Follow-up imaging could not be obtained due to hospital policy from where treatment was ongoing due to confidentiality.

Written informed patient consent for publication has been obtained.