J Gastrointest Canc DOI 10.1007/s12029-014-9633-8

CASE REPORT

Primary Hepatic Lymphoma: A Case Report Meryem Aitelhaj & Safa Akaaboun & Siham Lkhouyaali & Meryem Ait Ali & Salma Benhmida & Rachida Latib & Imane Chami & Najib Boujida & Hind M’rabti & Hassan Errihani

# Springer Science+Business Media New York 2014

Abbreviations PHL Primary hepatic lymphoma CT Computed tomography MRI Magnetic resonance imaging PETFluorine-18 fluorodeoxyglucose-positron emisFDG sion tomography

M. Aitelhaj (*) : S. Lkhouyaali : S. Benhmida : H. M’rabti : H. Errihani Medical Oncology Department, National Institute of Oncology, Rabat, Morocco e-mail: [email protected] S. Lkhouyaali e-mail: [email protected]

Introduction Extranodal lymphomas are responsible for 10 to 25 % of nonHodgkin’s lymphoma cases [1, 2]. Primary hepatic lymphoma (PHL) is an extremely rare and poorly characterized disease that accounts for 0.016 % of these patients [3]. The most common subtype is the diffuse large B cell lymphoma [4, 5]. Caccamo and colleagues outlined the traditional definition of PHL as lymphoma that is confined to the liver (stage IE) [6]. The medical literature consists primarily of case reports. There have been no large studies describing the treatments against this tumour; chemotherapy is usually considered to be the mainstay of treatment. Misdiagnosis is frequent, and cases of unnecessary resection have been reported. Present case highlights the importance of an accurate diagnosis and the benefit of combination chemotherapy and rituximab.

S. Benhmida e-mail: [email protected] H. M’rabti e-mail: [email protected]

Case Report

H. Errihani e-mail: [email protected]

We present a 66-year-old Moroccan woman with no relevant antecedents who presented for 2 months right upper quadrant abdomen pain. She had no other symptoms, including fever, weight loss, or anorexia. Physical examination revealed hepatomegaly and soft abdomen, without lymphadenopathy or splenomegaly. Computed tomography (CT) of the abdomen showed a large hypodense area in the segment V and VI of the liver with a size of 110×78 mm, and enhanced heterogeneously after injection (Fig. 1). There was no lymphadenopathy involvement on CT. Magnetic resonance imaging (MRI) findings showed a hypointense lesion on T1-weighted images sized 98 mm×70 mm, and hyperintense on T2weighted images enhanced after injection, with necrosis zones (Fig. 2). Radiographic characteristics were read as

S. Akaaboun : M. Ait Ali : R. Latib : I. Chami : N. Boujida Radiology Department, National Institute of Oncology, Rabat, Morocco S. Akaaboun e-mail: [email protected] M. Ait Ali e-mail: [email protected] R. Latib e-mail: [email protected] I. Chami e-mail: [email protected] N. Boujida e-mail: [email protected]

J Gastrointest Canc

Fig. 3 Immunohistochemical staining reveals diffuse and strongly positive reactions with CD20 Fig. 1 CT image showing a large hypodense area in the segment V and VI of the liver

consistent with malignant lesion, probably hepatic metastasis. The results of the complete blood count, liver function and β microglobulin were normal. Lactate dehydrogenase (LDH) level was found to be 391 U/L, slightly upper normal limit (300 U/l). Results of serologic tests for hepatitis B virus, hepatitis C virus and human immunodeficiency virus were negatives. Tumour marker levels carcinoembryonic antigen (CEA), carbohydrate antigen 19–9 (CA 19.9) and alphafetoprotein (AFP) were also negative. Because of her symptoms and the radiological findings, a percutaneous CT-guided liver biopsy was performed. The histopathological examination showed a diffuse infiltration of large-sized lymphoid cells, with large nuclei and high mitotic activity. Immunohistochemistry revealed a lack of reactivity to pan cytokeratin AE1/AE3. Neoplastic cells were positive for membranous CD20 expression (Fig. 3). These findings were consistent with the diagnosis of diffuse large B cell lymphoma. Subsequent CT of the brain, chest and pelvis was performed and did not show lymphadenopathy or visceral

involvement. Bone marrow biopsy examination showed no evidence of diffuse large B cell lymphoma. The patient received R-CHOP treatment (cyclophosphamide 750 mg/m2 on day 1, doxorubicin 50 mg/m2 on day 1, vincristine 1.4 mg/2 on day 1, rituximab 375 mg/m2 on day 1 and prednisone 100 mg for 5 days) every 3 weeks for 8 cycles. In this case, the patient showed a good response to treatment with mild side effects. After 8 cycles, the lesion decreased in size to 22 mm×13 mm. After 36 months of follow-up, the patient remained asymptomatic. However, imaging studies have shown the persistence of a small stellar lesion sized 8 mm of the largest diameter (Fig. 4). Unfortunately, we could not perform PET-FDG to confirm the complete response due to a lack of resources. The patient has been regularly seen in an outpatient.

Discussion Although secondary involvement of the liver by nonHodgkin’s lymphoma is relatively common, PHL is a rare

Fig. 2 MRI image sowing a large solitary mass in the liver. hypointense on T1 and hyperintense on T2, enhanced after injection; with necrosis zones

J Gastrointest Canc

Fig. 4 CT scan showing the hepatic lesion before treatment and after treatment during the follow-up

disorder that accounts for 0.016 % of extranodal nonHodgkin’s lymphoma [3]. Misdiagnosis is frequent, and cases of unnecessary resection have been reported. Present case highlights the importance of an accurate diagnosis, because PHL has a good response to chemotherapy, and may have a better prognosis than other hepatic lesions such as hepatocellular carcinoma or hepatic metastasis. It is more frequent in men, and the usual age at presentation is the fifth decade [4]. Presentations vary from the incidental discovery of hepatic abnormalities in asymptomatic patients to a fulminant hepatic failure. Symptoms are usually nonspecific including right upper quadrant and epigastric pain such as in our patient, fatigue, weight loss, fever, anorexia and nausea. Hepatomegaly is very common, and jaundice may be found on physical examination [7]. Imaging means can objectify a single nodule in 42 % of cases, multiple masses in 50 % of patients or more rarely diffuse hepatic infiltration [8]. Laboratory findings usually include abnormal aminotransferases, alkaline phosphatase, lactate dehydrogenase and β microglobulin [4]. Relation between PHL and hepatitis B, hepatitis C, HIV and immunosuppression has been reported in several small case series [9]. The main tool of the diagnosis of PHL is biopsy with histopathology examination. The radiological findings and fluorine-18 fluorodeoxyglucose-positron emission tomography (FDG-PET) positivity remain non-specific and have not been very successful at distinguishing hepatic lymphoma from other liver pathologic processes. Biopsy is also indispensable for identifying the immunophenotype of the PHL. More than half of all hepatic lymphomas are diffuse large B cell lymphoma [9]. This type of lesion is highly chemosensitive, and early aggressive chemotherapy may result in sustained remission [9]. Most patients are treated with chemotherapy, with some physicians employing a multimodality approach that also incorporates surgery and radiotherapy [3, 10]. The standard treatment for patients with diffuse large B cell lymphoma is the CHOP. The addition of rituximab, a chimeric monoclonal

antibody to the CHOP regimen, increases the response rate and prolongs event-free and overall survival in patients with nodal diffuse large B cell lymphoma [10, 11]. The role of chemotherapy in PHL is supported by the two largest series. Twenty-four patients were treated at the M. D Anderson Cancer Center with multiagent chemotherapy between 1974 and 1995. Most patients in this series were given CHOP-based chemotherapy. The complete response rate to chemotherapy was 83.3 %, and only 12.5 % had primary refractory disease. The 5-year failure-free survival rate of patients in this series was 70 %, which compares favourably with treatment outcomes in non-hepatic lymphomas [12]. The prognosis of PHL is considered poor with a median survival of 8 to 16 months [13]. In this case, we obtain long survival more than 3 years. Clinical, biological and histological prognostic factors have been reported: age, a history of cirrhosis, constitutional symptoms, bulky disease, elevated levels of LDH, unfavourable histological subtype, and a high proliferation rate [14, 11].

Conclusion Although the primary liver lymphoma is rare, this diagnosis should be considered in a symptomatic single liver injury without other known primary tumour or haematological disease. In addition to the rarity of its occurrence, our observation highlights the importance of an accurate diagnosis that allows adequate treatment and the excellent response to chemotherapy plus rituximab. Acknowledgments We would like to thank Dr kettani the head department of united nations pathology laboratory, for his cooperation and help in making the slides and the printing thereof, and also the patient and her family. Competing Interests The authors declare that they have no competing interests.

J Gastrointest Canc Consent Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. Authors’ Contributions M.A and S.L were involved in the analysis of the data and the literature research, and also wrote the manuscript. S.A and M.A helped with the patient management and revision of the manuscript. S.B helped with the literature research. H.M and HE approved the treatment and analyzed the literature data. All authors read and approved the final manuscript.

References 1. Freeman C, Berg JW, Cutler SJ. Occurrence and prognosis of extranodal lymphomas. Cancer. 1972;29:252–60. 2. Rudders RA, Ross ME, DeLellis RA. Primary extranodal lymphoma: response to treatment and factors influencing prognosis. Cancer. 1978;42:406–16. 3. Yang X, Tan W, Yu W. Diagnosis and surgical treatment of primary hepatic lymphoma. World J Gastroenterol. 2010;16: 6016–9. 4. Agmon-Levin N et al. Primary hepatic lymphoma: a case report and review of the literature. Age Ageing. 2004;33:637–40.

5. Haider F et al. Primary hepatic lymphoma presenting as fulminant hepatic failure with hyperferritinemia: a case report. J Med Case Rep. 2008;2:279. 6. Caccamo D, Pervez NK, Marchevsky A. Primary lymphoma of the liver in the acquired immunodeficiency syndrome. Arch Pathol Lab Med. 1986;110:553–5. 7. Masood A, Kairouz S, Hudhud KH. Primary non-Hodgkin lymphoma of liver. Curr Oncol. 2009;16:74–7. 8. Maher MM et al. Imaging of primary non Hodgkin lymphoma of the liver. Clin Radiol. 2001;56:295–301. 9. Salmon JS, Thompson MA, Arildsen RC, Greer JP. Non-Hodgkin’s lymphoma involving the liver: clinical and therapeutic considerations. Clin Lymphoma Myeloma. 2006;6:273–80. 10. Serrano-Navarro I, Rodríguez-López JF, Navas-Espejo R. Primary hepatic lymphoma-favorable outcome with chemotherapy plus rituximab. Rev Esp Enferm Dig. 2008;100:724–8. 11. Doi H, Horiike N, Hiraoka A. Primary hepatic marginal zone B cell lymphoma of mucosa-associated lymphoid tissue type: case report and review of the literature. Int J Hematol. 2008;88:418–23. 12. Page RD, Romaguera JE, Osborne B, et al. Primary hepatic lymphoma: favorable outcome after combination chemotherapy. Cancer. 2001;92:2023–9. 13. Lettieri CJ, Berg BW. Clinical features of non-Hodgkin’s lymphoma presenting with acute liver failure: a report of five cases and review of the published experience. Am J Gastroenterol. 2003;98:1641–6. 14. Ma Y, Chen E, Chen X. Primary hepatic diffuse large B cell lymphoma: a case report. Hepat Mon. 2011;11:203–5.

Primary hepatic lymphoma: a case report.

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