Gastroenterology Hepatology and Digestive System

Primary Hepatic Lymphoma –An Enigmatic Diagnosis: A Case series

Kakodkar Pramath,

Kakodkar Pramath

Department of Pathology and Laboratory Medicine, College of Medicine, University of Saskatchewan, Canada.

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Shekari Nooshin,

Shekari Nooshin

Department of Anatomic, Physiology, and Pharmacology, University of Saskatchewan, Saskatoon, SK, Canada.

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Zhang Dan,

Zhang Dan

Department of Pathology and Laboratory Medicine, College of Medicine, University of Saskatchewan, Canada.

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Selliah Kanthan,

Selliah Kanthan

Department of Surgery, College of Medicine, University of Saskatchewan, Saskatoon, SK, Canada.

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Rani Kanthan

Rani Kanthan

Department of Pathology and Laboratory Medicine, College of Medicine, University of Saskatchewan, Canada.

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  10.33425/2833-4418.0010
Background: Primary hepatic lymphoma (PHL) is a rare presentation of a common hematological disease and is defined as lymphoma confined to the liver without extrahepatic involvement. PHL can mimic carcinoma, inflammatory or infectious conditions, posing a diagnostic challenge across clinical, radiological and pathological domains. Lab investigations and radiology often lack specificity, and early suspicion is limited. This case series highlights the importance of timely recognition and accurate diagnosis through a review of seven patients with PHL. Methods: An index case of PHL identified on a routine medical liver biopsy prompted a retrospective search of our laboratory information system for the diagnosis of ‘hepatic lymphomas’ since 2010 [14 years]. Inclusion criteria included identification of cases with absent lymphadenopathy, bone marrow involvement or peripheral compartment lymphoma at the time of the liver biopsy. Histopathological features were independently reviewed, and relevant clinical data, imaging and management parameter were compiled from the electronic medical records. All cases were deidentified. Results: Seven cases of PHL were included from a pathology caseload of 586,813 cases (prevalence: 1.2 per 100,000). Median age at diagnosis was 54 ± 12.3 years with a slight female predilection (F:M=1.3:1). Presentation ranged from acute liver dysfunction (14.2%, n=1) to nonspecific abdominal pain (85.7%, n=6). Radiology ranged from normal (14.3%, n=1), to solitary hepatic lesions (14.3%, n=1), multifocal perfusion abnormalities without discrete masses (14.3%, n=1), or heterogeneous, multiple, ill-defined hypodense liver lesions (57.1%, n=4). Diffuse large B-cell lymphoma (71.4%, n=5) was the predominant subtype, followed by Burkitt lymphoma (14.3%, n=1), and marginal zone lymphoma (14.2%, n=1). Remission was achieved in 57.1% of cases, whereas 42.8% died within 2 months of diagnosis, reflecting underlying biological heterogeneity. Conclusions: PHL is rare, and histopathology along with ancillary tests remains the gold standard for accurate diagnosis. Clinician awareness and early liver biopsy are essential for appropriate patient management.
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