Primary hepatic lymphoma

Last revised by Dr Eid Kakish ◉ on 11 Nov 2021

Citation, DOI & article data

Citation:

Weerakkody, Y., Kakish, E. Primary hepatic lymphoma. Reference article, Radiopaedia.org. (accessed on 14 Aug 2022) https://doi.org/10.53347/rID-7581

DOI:

https://doi.org/10.53347/rID-7581

Permalink:

https://radiopaedia.org/articles/7581

rID:

7581

Article created:

11 Nov 2009 by Dr Yuranga Weerakkody ◉

Revisions:

29 times by 15 users - see full revision history

Systems:

Hepatobiliary, Haematology, Oncology

Sections:

Tags:

cases, liver, lymphoma

Synonyms:

Primary hepatic lymphoma (PHL)

Primary hepatic lymphoma (PHL) is rare, with roughly 100 described cases. If it is being considered as a diagnosis, distal lymphadenopathy, splenomegaly, bone marrow disease, and leukemia should not be present for at least 6 months after the liver tumor has been detected (see: secondary hepatic lymphoma) ⁴.

The exact cause of this primary hepatic lymphoma is still unclear; however, it is primarily found in immunocompromised patients due to AIDS/HIV, congenital immunodeficiency, collagen vascular disease, and immunosuppressant therapy for organ transplant. Reports have also shown infections as a probable cause, including EBV, HIV, hepatitis C and hepatitis B with chronic hepatitis and cirrhosis.

Diffuse large B-cell lymphoma comprises the majority of cases of primary liver lymphoma. Other histologic types include peripheral T-cell leukemia/lymphoma not-otherwise-specified, follicular lymphoma, mantle cell lymphoma, and Burkitt lymphoma.

Radiographic features

Primary hepatic lymphoma is most commonly seen as a solitary mass (53-65%). It does not have characteristic radiographic features and can often be mistaken for other liver tumors. Liver biopsy is the most specific means of making the diagnosis.

Ultrasound

Ultrasound is usually the initial radiological test performed:

typically hypoechoic to the liver parenchyma, probably due to high cellularity and lack of background stroma
rarely, they may be so hypoechoic as to appear similar to a cyst
occasionally have a "target" appearance with a central hyperechogenicity and outer hypoechogenicity
increased peripheral vascularity on color Doppler

CT

frequently a single (~60%), solid, hypoattenuating (due to hypovascularity) hepatic lesion, as opposed to secondary lymphoma, which can be more diffusely infiltrating ^1,2
there can be a large variation in size, but primary hepatic lymphoma tends to have a dominant mass, as opposed to secondary lymphomatous involvement of the liver, which tends to be more diffuse
variable contrast enhancement on CT, but tend to be hypoenhancing relative to liver on all phases, with no enhancement in some cases
can present as an ill-defined mass at the porta hepatis
can have hemorrhage, necrosis, and rim enhancement

MRI

MRI findings depend on the extent of infiltration.

T1: typically hypointense relative to remainder of liver
T2: often shows variable intensity
T1 C+ (Gd): these lesions show poor enhancement but may show faint peripheral rim enhancement
DWI/ADC: restricts diffusion, even in comparison to liver parenchyma, as it is hypercellular

Signal characteristics are almost identical to secondary lymphomatous involvement but manifestation as a single solid mass may favor a primary hepatic lymphoma ¹.

Nuclear medicine

PET/CT

PET/CT is useful in making the diagnosis and can also be used for staging.

FDG-PET
- avid FDG uptake of the lymphomatous mass in the liver
- can show or exclude extrahepatic sites of lymphoma with a high degree of sensitivity

Treatment and prognosis

Primary hepatic lymphoma is primarily treated with chemotherapy. R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) is the most common chemotherapy regimen for advanced diffuse large B-cell lymphoma (DLBCL). In the United States, this regimen is generally given every three weeks for 6 to 8 cycles. Other treatment options include radiation therapy or surgery.