Elsevier

Human Pathology

Volume 45, Issue 3, March 2014, Pages 648-652
Human Pathology

Case study
Primary anorectal Hodgkin lymphoma: report of a case and review of the literature

https://doi.org/10.1016/j.humpath.2013.09.015Get rights and content

Summary

Primary colorectal lymphomas are very rare. They are mostly B-cell non-Hodgkin lymphomas. Only 2 cases of anorectal Hodgkin lymphoma have been described so far, both affecting HIV-infected males and showing Epstein-Barr virus infection. We report an unusual case of primary Hodgkin lymphoma of the anorectal region in an HIV-negative, Epstein-Barr virus–infected patient and in the absence of inflammatory bowel disease. The importance of distinguishing Hodgkin lymphoma from Epstein-Barr virus–induced lymphoproliferative disorders and from Epstein-Barr virus–positive diffuse large B-cell lymphoma of the elderly is stressed in particular in non-immunocompromised patients and in the absence of history of inflammatory bowel disease.

Introduction

Colorectal lymphomas represent 6% to 12% of all gastrointestinal lymphomas [1]. Usually, they are secondary B-cell non-Hodgkin lymphoma [1]. Primary colorectal lymphomas account for only 0.2% of all malignant tumors in this site [1], [2]. In the gastrointestinal tract, primary Hodgkin lymphoma (HL) has been reported only in 1% to 3% of all cases, and the stomach and small intestine are the sites most commonly involved [3]. Primary HL of the rectum is rare [3], mainly described in HIV-infected patients [4] or associated with inflammatory bowel disease (IBD) [1]. Epstein-Barr virus (EBV) is present in a significant proportion of cases of HL [3]. Only 2 cases of anorectal HL have been described so far, both affecting HIV- and EBV-infected males [3], [5], [6] and presenting with a concomitant lymphadenopathy. According to Dawson's criteria for classifying primary gastrointestinal lymphomas [1], they represent a secondary involvement by a nodal HL.

We report a case of primary HL of the anorectal region in an HIV-negative, EBV-infected patient and in the absence of IBD and any other localization. A review of the literature is also presented, and the differential diagnosis with EBV-induced lymphoproliferative disorders (LPDs) and EBV-positive diffuse large B-cell lymphoma (DLBCL) of the elderly is discussed.

Section snippets

Clinical history

An 83-year-old man presented to the Surgery Unit of Valdichiana Hospital (Siena) complaining of tenesmus and mucous bloody diarrhea that lasted for 1 month. Medical history was unremarkable. Immune suppression conditions (primary immunodeficiency, autoimmune disorders, and drugs) were excluded by clinical history and biochemical evaluation. HIV-1 and HIV-2 serology as well as hepatitis B virus (HBV) and hepatitis C (HCV) tests were negative. EBV serology showed immunoglobulin G (IgG) anti-VCA

Discussion

Primary colorectal lymphomas are rare, and most cases are non-Hodgkin lymphomas [1]. In an extensive review of the literature, Thomas et al [7] identified 26 cases of HL arising in the colon, of which 6 cases (23%) involved the rectum [3]. However, some of these previously reported cases may be examples of secondary involvement of the large bowel in patients with nodal disease [3]. We identified 11 sufficiently documented cases of primary HL of the rectum, all published as single case reports

Conclusion

We report a very rare case of HL arising in the anorectal region of an 83-year-old man. The association of clinical, histologic, and radiologic findings was necessary to lead us to a correct diagnosis. The importance to distinguish HL from EBV-LPDs and from EBV-positive DLBCL of the elderly particularly in organs not commonly affected by HL and in non-immunocompromised patients with no history of IBD is underlined [17].

Acknowledgment

The authors would like to thank Professor Piero Tosi (University of Siena) for his expert review of the manuscript.

References (25)

  • M. Sapp et al.

    EBV-associated perianal Hodgkin's disease in an HIV-positive individual

    Am J Hematol

    (2001)
  • D.B. Thomas et al.

    Primary Hodgkin's disease of the sigmoid colon: a case report and review of the literature

    Arch Pathol Lab Med

    (1997)
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