Case studyPrimary anorectal Hodgkin lymphoma: report of a case and review of the literature
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.
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