CASE 17572 Published on 11.01.2022

Discerning the diagnostic quandary of primary hepatic lymphoma


Abdominal imaging

Case Type

Clinical Cases


Ashwini Bellamkondi, Juhi Agrawal, Prama Dubey, Bhavyansh Rathi, Meenu Bagarhatta, Kuldeep Mendiratta, Naima Mannan

Department of Radiodiagnosis, SMS Medical College and Hospital, Jaipur, Rajasthan, India


63 years, male

Area of Interest Abdomen ; Imaging Technique CT-Colonography
Clinical History

A 63-year-old male presented with complaints of right upper quadrant abdominal pain since 1 month, low-grade fever, yellowish discoloration of sclera.

Lab parameters suggested direct bilirubinemia. Mildly elevated lactic dehydrogenase enzyme, positive for hepatitis B virus. Carcinoembryonic antigen (CEA) and alphafetoprotein (AFP) were within normal limits.

Imaging Findings

The patient was referred to the department of radiodiagnosis to evaluate the cause of obstructive jaundice. The patient initially underwent ultrasonography which revealed presence of ill-defined lobulated hypoechoic lesion in periportal location extending along the right porto-biliary radicles with encasement of the right portal vein and right branch of hepatic artery without causing obstruction of the same. The lesion was seen compressing the common bile duct (CBD) causing intrahepatic biliary radicles (IHBR) dilatation which was confirmed on triple-phase abdominal computed tomography (TPCT) and magnetic resonance imaging(MRI). There is no evidence of periportal lymphadenopathy or splenomegaly. The lesion showed minimal post-contrast enhancement on TPCT in comparison with rest of liver parenchyma suggesting periportal infiltrating mass. Differentials of cholangiocarcinoma, hepatocellular carcinoma, hepatic lymphoma was kept after extensive research. Then the patient was advised biopsy for confirmation of diagnosis which revealed small round cell tumor followed by immunohistochemistry confirmed B-cell type of Non-Hodgkin’s lymphoma. The patient succumbed to death within 15 days of diagnosis.



Liver is the largest reticuloendothelial organ which is involved in many hematologic malignancies. Liver is one of the extranodal site of non-Hodgkins lymphoma.

Hepatic lymphoma can be classified into primary and secondary forms. Primary hepatic lymphoma (PHL) is a rare form of non-Hodgkin lymphoma (NHL) which is confined to liver and hilar lymph nodes and accounts for <1% of all NHL cases. In contrast, multifocal lesions or diffuse infiltration is the commonest pattern of secondary hepatic lymphoma

Involvement of liver secondary to systemic NHL is seen in  40% of patients.

Diagnosis of PHL depends on a liver biopsy that is compatible with the lymphoma and the absence of extrahepatic lymphoproliferative involvement.

Clinical Perspective

Clinical presentation is variable ranging from fulminant onset of disease to death[1]. The common presenting symptom is right upper quadrant pain, jaundice, weight loss, and fever [2]. Secondary hepatic lymphoma presents with systemic symptoms like fever, night sweats and weight loss along with hepatosplenomegaly and generalised lymphadenopathy.

Lei et al. [3] proposed the following diagnostic criteria for PHL: symptoms at presentation caused mainly by liver involvement, absence of both clinical and radiological distant lymphadenopathy, and absence of leukemic blood involvement in the peripheral blood smear.

Laboratory findings associated with PHL include abnormal liver tests results, with nonspecific elevations of AST, alanine aminotransferase, alkaline phosphatase, and bilirubin levels. LDH elevation is common, being present in 30–80% of patients. Tumour markers such as AFP, CA 19-9, and CEA are within normal range in almost 100% of cases, helping to differentiate PHL from hepatocellular carcinoma or metastatic disease.

Imaging Perspective

Radiologically, hepatic lymphoma can present in three forms:

  • Solitary lesion
  • Multifocal lesions
  • Diffuse hepatic infiltration

Periportal mass can be seen in both primary and secondary hepatic lymphoma

At CT, periportal mass type of hepatic lymphoma shows ill-defined hypoenhancing soft tissue mass encasing portal and bilary tracts without causing its obstruction (vessel penetration sign).

At MR imaging, the lesion tend to be hypo- or isointense on T1-weighted images and moderately hyperintense on T2-weighted images with diffusion restriction.

Take-Home Message

PHL is an exceedingly rare disease, having no pathognomonic imaging/clinical features. It should be part of the differential diagnosis in a patient presenting with nodular/diffuse hepatic lesions, especially in the presence of normal tumour markers and/or elevated LDH levels/history of immunosuppression. Histology is mandatory for definitive diagnosis. It is important to recognize PHL because it is a chemosensitive disorder, allowing early treatment and improved overall prognosis.

Differential Diagnosis List
Periportal mass type of primary hepatic lymphoma
Periductal infiltrating type of cholangiocarcinoma
Hepatocellular carcinoma
Secondary hepatic involvement of the liver from non-Hodgkin lymphoma
Acute hepatitis
Final Diagnosis
Periportal mass type of primary hepatic lymphoma
Case information
DOI: 10.35100/eurorad/case.17572
ISSN: 1563-4086