Clinical History
A 50-year-old male immunocompromised with a low CD4 count of 19, complaining of severe abdominal pain and worsening septic markers.
Imaging Findings And Discussion
Unlike the visceral hepatic extension of stage IV lymphomas (15% of cases), primary lymphomas of the liver are rare (< 1% of extranodal lymphomas) [1].
They are mainly large B-cell Non Hodgkin Lymphomas. The other types described (immunoblastic, lymphoblastic, Burkitt’s, MALT lymphomas) represent less than 5% of cases.
Etiological factors reported include HCV (21% of cases in France), EBV and HIV infection, as well as auto-immune diseases for MALT lymphomas [2], [3].
They are typically discovered at a mean of 55 years (5–87 years), with a male/female ratio of 2.3/1, in a context of abdominal pain or discomfort [4]. Jaundice is uncommon (10–20% of cases).
The often large, solitary nodular form, which can measure more than 10 cm, is the most common (50–60% of cases), followed by the multinodular form in 40% of cases (like presented below), the diffuse infiltrating form being exceptional [5], [6].
With ultrasound, the nodules are usually hypoechoic (arrow), sometimes anechoic.
In a CT scan, these spontaneously hypodense lesions (arrows) exhibit variable behavior after injection (absence of enhancement in half of the cases, in patches for one third, possibly with a necrotic center, ring enhancement in 16–29% of cases, with a trend towards isodensity in the late stage). After chemotherapy, calcification is possible.
In MRI, there is spontaneous T1 hypointensity (Image 1), often marked T2 hyperintensity (Image 2), as with diffusion weighting (Image 3, multinodular), enhancement being again variable (Image 4). Forms of diffuse infiltration are possible (Image 3) [5].
Multiple infiltrative bone lesions were noted on MRI (white spots with arrows) and not clearly depicted on the CT scan.
Given its rarity, the most frequent diagnoses suggested are hepatocellular carcinoma, particularly when there is hepatitis C, and hepatic metastases.
The final diagnosis was made by percutaneous biopsy of the liver as the clinical manifestation not consistent with common liver diseases.
Pathology report, sections show diffuse involvement of the cores submitted by a high-grade B-cell lymphoma. Extensive necrosis is present. The tumor shows brisk apoptosis and mitotic activity (Ki-67 – virtually 100%). The morphology combined with immunohistochemistry is that of diffuse large B-cell lymphoma (DLBCL).
Median survival time in the literature is 15.3 months [4], [7].
After liver transplantation, two forms are described: a rapidly progressing precocious form, mainly involving the pedicular region and which may locally invade the liver in connection with EBV and respond favourably to a reduction in or discontinuation of immunosuppressants, and a later, EBV-negative form with a poorer prognosis.
Conclusion
Special case of DLBCL multinodular form of diffuse infiltration with bone metastasis.
Rare disease lacking specific clinical manifestations and biochemical indicators.
Its diagnosis is difficult clinically, needing to exclude organs or tissues lymphoma outside of the liver. When confronted with multiple space-occupying lesions of the liver but there is no any other organ or tissue invasion, primary liver lymphoma should be suspected on imaging and liver biopsy should be performed.
References:
- K.I. Lei Primary non-Hodgkin’s lymphoma of the liver Leuk Lymphoma, 29 (3–4) (1998), pp. 293-299. View Article
- J.P. Bronowicki, C. Bineau, P. Feugier, O. Hermine, N. Brousse, F. Oberti, et al. Primary lymphoma of the liver: clinical-pathological features and relationship with HCV infection in French patients Hepatology, 37 (4) (2003), pp. 781-787. View Article
- D. Chatelain, C. Maes, T. Yzet, M. Brevet, D. Bounicaud, J.P. Plachot, et al. Primary hepatic lymphoma of MALT-type: a tumor that can simulate a liver metastasis Ann Chir, 131 (2) (2006), pp. 121-124. View Article
- V. Noronha, N.Q. Shafi, J.A. Obando, S. Kummar Primary non-Hodgkin’s lymphoma of the liver Crit Rev Oncol Hematol, 53 (3) (2005), pp. 199-207. View Article
- K. Kaneko, A. Nishie, F. Arima, T. Yoshida, K. Ono, J. Omagari, et al. A case of diffuse-type primary hepatic lymphoma mimicking diffuse hepatocellular carcinoma Ann Nucl Med, 25 (4) (2011), pp. 303-307. View Article
- M.M. Maher, S.R. McDermott, H.M. Fenlon, D. Conroy, J.C. O’Keane, D.N. Carney, et al. Imaging of primary non-Hodgkin’s lymphoma of the liver Clin Radiol, 56 (4) (2001), pp. 295-301. View Article
- V.S. Avlonitis, D. Linos Primary hepatic lymphoma: a review Eur J Surg, 165 (8) (1999), pp. 725-729. View Article
Sources:
- Header Image by cnicholsonpath via flickr.
