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 Table of Contents  
Year : 2014  |  Volume : 39  |  Issue : 4  |  Page : 253-255

Primary hepatic lymphoma: an unusual hepatic malignancy: case report and review of the literature

1 Department of Hematology, All India Institute of Medical Sciences, Gautam Nagar, New Delhi, India
2 Department of Pathology, All India Institute of Medical Sciences, Gautam Nagar, New Delhi, India

Date of Submission10-Aug-2014
Date of Acceptance21-Nov-2014
Date of Web Publication25-Mar-2015

Correspondence Address:
Mukul Aggarwal
Department of Hematology, All India Institute of Medical Sciences, Gautam Nagar, 110029, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1110-1067.153973

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Primary hepatic lymphoma is a very rare malignancy representing ~0.016% of all cases of non-Hodgkin's lymphoma. It often presents in elderly persons with signs and symptoms of hepatitis, primary liver cancer, or metastatic tumor. In this case report, we present a 58-year-old lady with poor performance status who presented with obstructive jaundice, epigastric pain, and bilious vomiting. Abdominal imaging revealed a large hepatic mass that was pathologically confirmed primary hepatic diffuse large B-cell lymphoma without bone marrow involvement. Because of severe jaundice, she was managed with rituximab, cyclophosphamide, and prednisolone-based therapy but succumbed to neutropenic fever following chemotherapy. Egyptian J Haematol 39:-0 © 2014 The Egyptian Society of Haematology.

Keywords: Hepatic malignancy, liver mass, non-Hodgkin lymphoma, primary hepatic lymphoma

How to cite this article:
Purohit A, Aggarwal M, Venkatesan S, Mahapatra M, Dinda AK, Mishra P, Saxena R. Primary hepatic lymphoma: an unusual hepatic malignancy: case report and review of the literature. Egypt J Haematol 2014;39:253-5

How to cite this URL:
Purohit A, Aggarwal M, Venkatesan S, Mahapatra M, Dinda AK, Mishra P, Saxena R. Primary hepatic lymphoma: an unusual hepatic malignancy: case report and review of the literature. Egypt J Haematol [serial online] 2014 [cited 2022 Sep 30];39:253-5. Available from: http://www.ehj.eg.net/text.asp?2014/39/4/253/153973

  Introduction Top

Hepatic involvement by lymphoma is usually secondary to systemic involvement. Primary hepatic lymphoma (PHL) is a very rare malignancy, comprising 0.016% of all cases of non-Hodgkin's lymphoma and 0.4% of extranodal non-Hodgkin's lymphoma [1],[2]. We present a patient with a case of primary hepatic diffuse large B-cell lymphoma.

  Case report Top

A 58-year-old woman presented with complaints of intermittent high-grade fever, epigastric discomfort, heaviness in the abdomen, and nonbilious vomiting of 5 months duration and jaundice of 1 month duration. There was no history of weight loss, night sweats, pallor, or bleeding. On presentation, she had ECOG performance status of 3, was pale and icteric, with no palpable lymphadenopathy or stigmata of chronic liver disease. She had a tender hepatomegaly 10 cm below the right costal margin but no splenomegaly. On investigations, her hemoglobin, leukocyte count, and platelets count were 910 g/l, 11.2 × 10 9 /l, and 250 × 10 9 /l, respectively, with normal differential count. Her liver function tests were markedly deranged (serum bilirubin 15 mg% with direct component being 13 mg%, alkaline phosphatase 1938 U/l, albumin 2.9 g%, lactate dehydrogenase being 1830 IU/l, and transaminases being normal). Serology for hepatitis A, B, C, and E viruses was negative. Contrast-enhanced computed tomography of the abdomen [Figure 1] revealed a large hypoechoic lesion in the left lobe of liver measuring 14 × 12 × 7 cm without calcifications. Imaging confirmed absence of splenomegaly and significant lymphadenopathy. Biopsy of the mass revealed a diffuse large B-cell lymphoma; immunohistochemistry was positive for CD20 [Figure 2] and negative for CD3, ALK-1, cytokeratin, and epithelial membrane antigen. Bone marrow examination showed normal hematopoetic elements without lymphoma deposit. Hence, clinical diagnosis of PHL was considered and was planned for R-CHOP regimen. However, in view of elevated bilirubin, only rituximab (375 mg/m 2 ), cyclophosphamide (750 mg/m 2 ), and prednisolone (60 mg/m 2 for 5 days) were administered. Patient succumbed to febrile neutropenia and hypotension on 10th day of chemotherapy.
Figure 1: Contrast-enhanced computed tomographic of the abdomen. A large solid hypodense, heterogeneous hepatic lesion, measuring 14 × 12 × 7 cm with no calcification.

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Figure 2: Microphotograph of the liver biopsy showing (a) infiltration of intermediate and large-sized lymphoid cells (H&E, ×200); (b) tumor cells positive for CD20 (CD20 immunohistochemistry, ×200).

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  Discussion Top

PHL is defined as a lesion or lesions confined to the liver without involvement of other organ or lymph nodes. It can occur in any age group but is more common in middle-aged men, in organ transplant recipients, in patients receiving immunosuppressive therapy, and in individuals with AIDS. Studies have indicated that hepatitis C infection is strongly related to PHL (found in 20-60% of patients) [3],[4]. The present case had neither hepatitis C infection nor signs of immunodeficiency.

Lei [5] proposed the following criteria for the diagnosis of PHL:

  1. The symptoms are mainly caused by liver involvement at presentation;
  2. No clear evidence of superficial lymph node enlargement and distant lymph node metastasis; and
  3. No abnormalities in peripheral blood cells in blood smears, including spleen, lymph node, and bone marrow.

Abdominal pain or discomfort is the most common presenting symptom, occurring in 39-70% of patients, whereas B symptoms appear in 37-86%, jaundice in 10-20%, and hepatomegaly in 50% of patients [6]. b2-Microglobulin is elevated in up to 90% of patients. a-Fetoprotein and carcinoembryonic antigen are within normal limits in all cases. Radiologically, it can present as a solitary lesion (55-60%), multiple lesions (30-35%), or as diffuse hepatic infiltration. Lesions are hypoechoic on ultrasound and hypoattenuating lesions on computed tomographic scans, which may have a central area of low intensity indicating necrosis. Following the administration of intravenous contrast, 50% of PHL lesions do not enhance, 33% show patchy enhancement, and 16% show a ring of enhancement.

Histologically, majority of PHL are diffuse large B-cell lymphoma type (46-68%) and rarely lymphoblastic lymphoma, Burkitt lymphoma, and follicular lymphoma. The tumor has a nodular or diffuse growth pattern, in which the lymphoma cells expand into the liver parenchyma [7]. Differential diagnoses include hepatitis, primary hepatic tumors (hepatoma, hepatocellular carcinoma), carcinoma with hepatic metastases, and systemic lymphoma with secondary hepatic involvement.

The optimal treatment of PHL is not yet defined. Surgical treatment, radiotherapy, and chemotherapy were all reported as treatment modalities alone or in combination. Rituximab combination with anthracycline-based multiagent chemotherapy is preferred with modification based on underlying comorbidity. Options include R-CHOP, R-CVP, or R-CP. Radiotherapy is given as adjuvant or in individuals not fit for chemotherapy. The reported survival rates for PHL vary, largely depending on comorbidity and treatment used. In a series of 72 patients of PHL, Avlonitis and Linos [8] showed a median survival of 29 months (n = 8) when surgery alone was used for treatment, 20.7 months when both surgery and chemotherapy were used (n = 14), 14 months when chemotherapy alone (n = 40), and 12.5 months when both chemotherapy and radiotherapy were used as treatment modality (n = 8). Page et al. [9] described 24 cases with varying comorbidity, and treatment only with chemotherapeutics resulted in an overall 5-year survival of 83%. Poor prognostic factors include massive liver infiltration, high index of proliferation, advanced age (especially poor performance status), elevated lactate dehydrogenase, elevated levels of b2 microglobulin, and diffuse infiltration on liver biopsy. Emile et al. [10] showed 1 and 3-year survivals with nodular involvement 70 and 57% and 38 and 18%, respectively, with diffuse involvement (P = 0.0033).

  Conclusion Top

PHL, although a rare disease, should be considered in an elderly who presents with liver mass or infiltration. Although hepatocellular carcinoma or metastatic diseases are more common, absence of elevated levels of carcinoembryonic antigen and a-fetoprotein and hypoechoic liver mass may indicate PHL. New multiagent attenuated chemotherapy and addition of rituximab may offer an appropriate treatment with good response. Prognosis is better if diagnosed and treated early (probably before performance worsens and jaundice increases or appears).

  Acknowledgements Top

The authors M.A. and A.P. were involved in the patient diagnosis and management and preparation of the manuscript. M.M. critically analyzed the manuscript and managed the patient. A.K.D. helped in the pathological diagnosis.

Conflicts of interest

There are no conflicts of interest.

  References Top

Miyashita K, Tomita N, Oshiro H, Matsumoto C, Nakajima Y, Ito S, et al. Primary hepatic peripheral T-cell lymphoma treated with corticosteroid. Intern Med 2011; 50 :617-620.  Back to cited text no. 1
Ryan J, Straus DJ, Lange C, Filippa DA, Botet JF, Sanders LM et al. Primary lymphoma of the liver. Cancer 1988; 61 :370-375.  Back to cited text no. 2
Nasr BAC, Chaari N, Kochbati L, Besbes M, Maalej M Primary non-Hodgkin lymphoma of the liver: case report and review of the literature. Cancer Radiother 2006; 10 :595-601.  Back to cited text no. 3
Chowla A, Malhi-Chowla N, Chidambaram A, Surick B. Primary hepatic lymphoma in hepatitis C: case report and review of the literature. Am Surg 1999; 65 :881-883.  Back to cited text no. 4
Lei KI. Primary non-Hodgkin′s lymphoma of the liver. Leuk Lymphoma 1998; 29 :293-299.  Back to cited text no. 5
Santos ES, Raez LE, Salvatierra J, Morgensztern D, Shanmugan N, Neff GW. Primary hepatic non-Hodgkin′s lymphomas: case report and review of the literature. Am J Gastroenterol 2003; 98 :2789-2793.  Back to cited text no. 6
Rockey DC, Caldwell SH, Goodman ZD, Nelson RC, Smith AD. Liver biopsy. Hepatology 2009; 49 :1017-1044.  Back to cited text no. 7
Avlonitis VS, Linos D. Primary hepatic lymphoma: a review. Eur J Surg 1999; 165 :725-729.  Back to cited text no. 8
Page RD, Romaquera JE, Osborne B, Medeiros LJ, Rodriquez J, North L, et al. Primary hepatic lymphoma: favourable outcome after combination chemotherapy. Cancer 2001; 92 :2023-2029.  Back to cited text no. 9
Emile JF, Azoulay D, Gornet JM, Lopes G, Delvart V, Samuel D, et al. Primary non-Hodgkin′s lymphomas of the liver with nodular and diffuse infiltration patterns have different prognoses. Ann Oncol 2001; 12 :1005-1010.  Back to cited text no. 10


  [Figure 1], [Figure 2]


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