Primary Hepatic Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue in a Patient with Chronic Hepatitis B Virus Infection: Case Report and Summary of the Literature

Abstract

Background: The incidence of extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is low, at 7–8% of all non-Hodgkin lymphoma cases. The most common site of MALT lymphoma occurrence is the stomach. Primary hepatic extranodal marginal zone lymphoma of MALT is classified as a type of non-gastric MALT lymphoma and is considered extremely rare, with no consensus on imaging study findings or treatment due to a limited number of reports. We herein describe a rare case of primary hepatic extranodal marginal zone lymphoma of MALT with underlying hepatitis B infection (HBV) and present useful diagnostic findings of various imaging modalities, including contrast-enhanced ultrasonography (CEUS) with Sonazoid. Case presentation: A 66-year-old woman was diagnosed as being a non-active carrier of HBV at 51 years of age at the time of total hysterectomy and bilateral adnexectomy for uterine cervical cancer. She was admitted to our hospital following the incidental detection of two focal liver lesions on computed tomography. The lesions were considered malignant based on clinical and other radiologic imaging findings. Her CEUS results of hypo-enhancement in the portal and late phases were consistent with those of previously reported cases of hepatic extranodal marginal zone lymphoma of MALT, and histological liver biopsy findings were compatible with the diagnosis. Conclusions: Primary hepatic extranodal marginal zone lymphoma of MALT is a rare condition that can appear in HBV carriers. Characteristic CEUS findings may help in disease diagnosis. Clinicians should bear primary hepatic extranodal marginal zone lymphoma of MALT in mind when encountering patients with focal liver lesions which exhibit image findings different from those of typical hepatocellular carcinoma.

The findings on the CEUS of diffuse large B cell lymphoma in abdomen: A case report and literature review

“…Primary liver lymphoma is often associated with HBV and HCV virus infections. HBV infection-related MALT was more common in primary liver lymphoma ( 29 ). More studies are needed to determine whether viral infection affects the progression of liver lymphoma.…”

Focal liver lesions other than hepatocellular carcinoma in cirrhosis: Diagnostic challenges

“…Histologic liver assessment is required for the accurate diagnosis of primary hepatic extranodal marginal zone lymphoma of MALT. [ 102 ] Preexisting liver disease, which may be etiologically important for the development of nongastric hepatic MALT lymphoma, is, in turn, a risk factor for the development of HCC. In a secondary liver manifestation of lymphoma, other organ and lymph node manifestations are already present.…”

“…Hypoenhancement on CEUS already occurs in the early portal venous phase. [ 102 ] Yamashita et al [ 102 ] compared the appearance of hepatic extranodal marginal zone lymphoma of MALT from published case reports with HCC on CEUS, CT, MRI, and positron emission tomography (PET). The appearance is very similar, and a reliable differential diagnosis based on imaging is not possible.…”

“…The appearance is very similar, and a reliable differential diagnosis based on imaging is not possible. [ 102 ] However, the “vascular penetration sign” was described as a special characteristic. The hepatic lymphomas showed penetrating vessels in the arterial phase.…”

“…This sign is sometimes also present in metastatic liver tumors and CCCs, but not in HCC. [ 102 ] In the study by Terzi et al [ 8 ] 0.2% (2/1006) lesions in liver cirrhosis corresponded to lymphoma infiltrations (without specifying primary or secondary). These showed CEUS-LIRADS pattern of LR-M. Histologic assignment by ultrasound-guided needle biopsy is necessary, taking into account anamnestic data ( Figure 7 ).…”