| Abstract|| |
Primary non-Hodgkin lymphoma of liver is a very rare malignancy. Here we report the case of a 50 year old female who presented with dull ache in the right hypochondrium and decreased appetite since 1 month. CT scan of abdomen and pelvis showed an enlarged liver with an ill- defined soft tissue lesion arising from left lobe measuring 13 × 9 cm suggestive of primary hepatic neoplasm. CT scan of chest, abdomen, and pelvis and whole body positron emission tomography showed no involvement of bone marrow, lymph nodes, spleen, or any other organ. Her liver function tests, alpha fetoprotein and carcinoembryonic antigen levels were normal. Serology was negative for viruses. Pathological examination favored diagnosis of Burkitt's lymphoma. Cytogenetic studies for MYC translocation t (8;14) is suggested for confirming the diagnosis since Ki 67 index is > 70% and not nearly 100% which is characteristic of Burkitt's lymphoma.
Keywords: Burkitt's lymphoma, non- Hodgkin lymphoma, primary hepatic lymphoma
|How to cite this article:|
Reddy P, Vernekar SS, Sneha P. Primary hepatic lymphoma- A diagnostic dilemma. Indian J Pathol Microbiol 2022;65:925-7
| Introduction|| |
The liver is most commonly involved in non-Hodgkin lymphomas (NHL) next to lymph nodes, spleen, and bone marrow. In rare instances, lymphomas may also involve the liver exclusively (primary hepatic lymphomas). The diagnosis of primary hepatic lymphoma may be challenging because of low index of suspicion, nonspecific presentations and often nonspecific radiologic presentations. PHL represents 0.016% of all non-Hodgkin's lymphomas and 0.4% of all extranodal lymphomas. Here we are presenting an interesting case of primary non-Hodgkin's lymphoma originating in liver showing unique presentation on pathological evaluation.
| Case History|| |
A 50 year old female admitted in our hospital with history of dull ache in the right hypochondrium and decreased appetite since 1 month. There was no history of fever, vomiting, weight loss, diarrhea, blood in stools, or jaundice. There was no significant past history. On physical examination, per abdomen showed hepatomegaly 15 cm from costal margin. There was no superficial lymphadenopathy. Laboratory results included hemoglobin levels 12 g/dl and white cell count of 8 × 109/L, with normal differentials and platelet count of 3.56 lakhs/cumm3. Liver function test and levels of serum alpha- fetoprotein and carcinoembryonic antigen were within normal limits. Serology was negative for HIV hepatitis B and C viruses. CT scan of abdomen and pelvis showed an enlarged liver with an ill-defined soft tissue lesion arising from left lobe measuring 13 × 9 cm suggestive of primary hepatic neoplasm. Pancreas, spleen and biliary tract were normal. CT scan of chest did not reveal any mediastinal lymphadenopathy [Figure 1]. Imaging by Positron emission tomography showed increase FDG concentration in the soft tissue mass lesion possibly arising from the left lobe of liver with infiltration.
|Figure 1: CT SCAN of abdomen and pelvis showed an enlarged liver with an ill-defined soft tissue lesion arising from left lobe|
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Ultrasound guided fine needle aspiration of liver was done which showed cellular smear comprising of small round discohesive cells, having coarse chromatin and scant amount of cytoplasm. Few lymphoglandular bodies were seen. Features were suggestive of non-Hodgkins lymphoma [Figure 2]. Liver biopsy was done and on histopathology, section studied showed small round cells in sheets. These cells were mildly pleomorphic having coarse chromatin and scant amount of cytoplasm. Occasional tangible body macrophages were seen. Features were suggestive of malignant small round cell tumor [Figure 3]. On immunohistochemistry, tumor cells were positive for CD20, CD45, CD10, Bcl6, Cmyc and negative for Bcl2, MUM1, Tdt and cyclin D1. Ki67 index was >70% [Figure 4] and [Figure 5]. Diagnosis on IHC favored more for Burkitt's lymphoma. Cytogenetic studies for MYC translocation t (8; 14) was suggested for confirmation of diagnosis since in this case Ki67 index was >70% and not nearly 100% which is characteristic of Burkitt's lymphoma.
|Figure 2: (40 × wrights) FNAC of liver suggestive of non-Hodgkins lymphoma|
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|Figure 3: (40 × H & E) section studied from liver biopsy suggestive of malignant round cell tumour|
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|Figure 5: (40×) Cmyc positivity- favouring diagnosis of Burkitts lymphoma|
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| Discussion|| |
PHL is a rare form of extranodal lymphoma accounting for less than 1% of all extranodal lymphomas. The criteria for diagnosis as given by Lei et al. are: a) clinical signs and symptoms due to liver involvement b) no evidence of distant lymphadenopathy (clinical or radiological) c) peripheral blood smear showing absence of leukemic picture. The age of presentation is more common in 5th decade with male predominance. Etiological factors that are proposed to be associated with PHL are Epstein-Barr virus (EBV) prior infection, hepatitis B and hepatitis C (HBV, HCV) and cirrhosis. Hepatitis C is found in 40-60% of patients with PHL. Our patient was negative for hepatitis viruses. Right upper quadrant pain or dullness, hepatomegaly, and palpable liver are the main presenting features as in our case. Elevated LDH, with normal alpha-fetoprotein and CEA remains a valuable biologic features, but in our case all were within normal limits. Pathologically, most PHL are diffuse large B cell type (46-68%), less than 5% of cases include diffuse mixed large and small cell, diffuse immunoblastic, lymphoblastic and small non- cleaved or Burkitt's lymphoma.
On immunohistochemistry in our case, CD45 and CD20 were positive suggesting that the tumor was a mature B cell lymphoma. Further typing of lymphoma was done, in which the tumor cells were positive for CD10 and Bcl6 suggesting lymphoma of germinal center origin. Germinal center origin lymphomas include diffuse large B cell lymphoma, follicular lymphoma and Burkitts lymphoma. Bcl2 and MUM1 were negative ruling out follicular and diffuse large B cell lymphoma. Cmyc showed nuclear positivity in >40% of cells and Ki 67 index was >70%. Diagnosis on IHC favored more towards Burkitt's lymphoma. Primary hepatic Burkitt lymphoma is very rare and only 11 cases are reported in world literature.
Modi et al. reported a case of primary hepatic Burkitt lymphoma in 21 year old female with clinical history similar to our case. Lionaki et al. reported a case in 51 year old female who developed primary hepatic lymphoma post kidney transplantation. She was a HCV carrier. Both the cases had immunohistochemical features similar to our case where in the tumor cells were positive for CD10, Bcl6, CD20 and negative for Bcl2 and Tdt. Ki67 index in both the cases were nearly 100%, whereas in our case it was >70%. Cytogenetic studies for MYC translocation t (8;14) was suggested to confirm the diagnosis.
Fine needle aspiration and biopsy of liver is a valuable tool in the diagnosis of primary hepatic lymphoma. Immunohistochemistry, cytogenetic studies help in further characterization of tumor. PHL should be considered in the differential diagnosis of space occupying lesions of liver in the presence of normal levels of alpha fetoprotein. Burkitt lymphoma is an aggressive B cell non-Hodgkin lymphoma with rapid clinical course and can be fatal within months if left untreated. Early diagnosis is crucial for disease management.
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The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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Department of Pathology, Karnataka Institute of Medical Sciences, Hubli, Karnataka
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]