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  Table of Contents    
CASE REPORT  
Year : 2017  |  Volume : 60  |  Issue : 3  |  Page : 412-414
Primary classical Hodgkin lymphoma of rectum: Report of an extremely rare case and review of the literature


1 Division of Pathology, Regional Cancer Centre, Thiruvananthapuram, Kerala, India
2 Division of Medical Oncology, Regional Cancer Centre, Thiruvananthapuram, Kerala, India

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Date of Web Publication22-Sep-2017
 

   Abstract 

Hodgkin lymphoma (HL) commonly presents as nodal disease, but in a subset of cases, the disease primarily develops in extranodal sites. Primary classical HL of the gastrointestinal (GI) tract is an extremely rare occurrence. Primary nature of the disease is confirmed after a complete lymphoma work up including chest radiograph, computed tomography scan, peripheral blood, and bone marrow studies. Only a few cases of primary GI lymphomas with limited immunohistochemical or molecular confirmation have been reported in literature. We report the case of a 64-year-old immunocompetent woman with primary rectal HL. She presented with constipation, and on sigmoidoscopy examination, she was detected to have an ulceroproliferative circumferential growth in the rectum. Considering the possibility of rectal carcinoma, a low anterior resection was done. Histology was suggestive of mixed cellularity classical HL. She was started on combination chemotherapy, and she responded well to treatment. However, she developed pulmonary complication after the fourth cycle of chemotherapy and succumbed to the illness. Primary rectal HL is extremely rare, and to the best of our knowledge, only 16 cases have been reported previously. We believe that reporting this case will add to the scarce data about this unusual presentation in immunocompetent patients.

Keywords: Classical Hodgkin lymphoma, extranodal, primary, rectum

How to cite this article:
Vasudevan JA, Nair RA, Nambiar K R. Primary classical Hodgkin lymphoma of rectum: Report of an extremely rare case and review of the literature. Indian J Pathol Microbiol 2017;60:412-4

How to cite this URL:
Vasudevan JA, Nair RA, Nambiar K R. Primary classical Hodgkin lymphoma of rectum: Report of an extremely rare case and review of the literature. Indian J Pathol Microbiol [serial online] 2017 [cited 2019 Dec 10];60:412-4. Available from: http://www.ijpmonline.org/text.asp?2017/60/3/412/215397



   Introduction Top


Hodgkin lymphoma (HL) was first recognized by Thomas Hodgkin and Samuel Wilks in the 19th century.[1] They usually arise in lymph nodes in young adults and the neoplastic tissue contains several large mononucleate and binucleate cells with prominent nucleoli (Hodgkin and Reed–Sternberg cells [HRS] cells) in a background of abundant nonneoplastic inflammatory infiltrate.[1] Extranodal forms account for <1% of HLs.[2] Gastrointestinal tract (GIT) is the most common site followed by lung, thyroid, skin, genitourinary system, and central nervous system.[2] Herein, we describe a case of 64-year-old female patient diagnosed as primary mixed cellularity classical HL in the rectum which is an extremely rare occurrence.


   Case Report Top


A 64-year-old postmenopausal female patient presented with constipation, reduced appetite and increased the frequency of micturition of 1-year duration. She was evaluated in a local hospital. Sigmoidoscopic examination revealed ulceroproliferative circumferential growth in the rectum measuring around 15 cm. Computed tomography (CT) abdomen revealed irregular rectal wall thickening with perirectal stranding. She underwent anterior resection in a local hospital and slides and blocks were brought to our center for review. Histopathology revealed the ulceration of lining epithelium with granulation tissue and an underlying neoplasm composed of mixed population of small lymphocytes, histiocytes, several eosinophils, and several large mononucleate and binucleate cells with nucleoli. Atypical mononucleate and binucleate cells were CD 30 positive, CD 20 negative, PAX 5 positive, CD 15 negative, leukocyte common antigen negative, and Epstein-Barr virus-encoded RNA (EBER) positive and a diagnosis of mixed cellularity classical HL was given [Figure 1]. She was a hypertensive on medication for 4 years. Her sleep and appetite were normal. Her blood routine examinations, renal function, and liver function tests were within normal limits. CT scan revealed the absence of lymphadenopathy. She was in stage 1AE. She was started on combination chemotherapy with doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) and received four cycles of chemotherapy. She responded well to therapy; however, after the fourth cycle, she developed breathing difficulty. CT of the chest showed septal thickening with interspersed areas of ground glass attenuation predominantly in basal and pleural aspects with an impression of interstitial lung disease. She was managed on conservative measures; however, her symptoms worsened, and she succumbed to the illness.
Figure 1: (a and b) Dense small lymphoid infiltrate admixed with few eosinophils and large atypical mononucleate and binucleate cells infiltrating the muscularispropria (H and E, ×100). Inset showing the submucosal granuloma formation (H and E, ×100). (c) Hodgkin and Reed–Sternberg cells (H and E, ×200). (d) Hodgkin and Reed–Sternberg cells admixed with several eosinophils (H and E, ×400). (e) Hodgkin and Reed–Sternberg cells are CD 30 positive (IHC, ×400) and inset showing the CD 20 negative Hodgkin and Reed–Sternberg cells (IHC, ×400). (f) PAX5 positive (IHC, ×400). (g) Epstein-Barr virus-encoded RNA positive (IHC, ×400)

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   Discussion Top


Primary colorectal lymphomas account for <1% of colorectal malignancies.[3] The criteria for primary gastrointestinal (GI) lymphomas include the absence of superficial lymphadenopathy at the time of diagnosis, absence of retroperitoneal and mediastinal lymphadenopathy, normal complete blood counts and white blood cell differential counts, predominance of GI lesion with or without adjacent lymph nodes, absence of hepatic, and splenic infiltrates at the time of diagnosis.[4] The most common histologic subtype affecting the colorectal region is diffuse large B cell lymphoma followed by mucosa-associated lymphoid tissue lymphoma.[5] The most common presenting symptom is abdominal pain and weight loss. Palpable abdominal mass can be present in half of the patients. Obstruction is a rare occurrence as compared to adenocarcinoma possibly due to more pliable nature of colorectal lymphoma and absence of desmoplastic response. The majority of colorectal lymphomas occur in cecum and ascending colon.[3] More than 70% of lymphomas of colorectum occur proximal to hepatic flexure. Descending colon and rectosigmoid account for around 25% of lymphomas of colorectal region. Diagnosis of colorectal lymphoma requires a high index of suspicion due to its rarity and nonspecific clinical and radiological signs. Preoperative diagnosis can be done by colonoscopic biopsy. The presence of numerous lymphoid follicles in ileum makes it the most common site in the intestine. Although HL can rarely present with colonic and rectal localization, primary rectal HL is extremely rare, and only 16 cases have been reported previously. We reviewed the 16 published cases of primary rectal HL and summarized five cases of HL which have mentioned follow- up details also [Table 1].[4],[6],[7],[8],[9]
Table 1: Clinical and treatment detailsof 5 published cases of HL which have mentioned outcome details also and the present case

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We report the case of a 68-year-old female patient with primary rectal HL. Our patient was seronegative for HIV and had neither inflammatory bowel disease nor any other condition indicative of immune deficiency. The diagnosis was confirmed by histology and immunohistochemistry. Our patient was treated with surgical resection followed by four cycles of ABVD; however, she succumbed to pulmonary complications. This adds to the scarce data about this unusual presentation in immunocompetent patients. Primary HL of the rectum in immunocompetent patients is uncommon, and including this case, 17 cases of HL arising in the rectum are reported in the literature. A close differential diagnosis, in this case, is EBV-positive mucocutaneous ulcer (EBVMCU). This is a recently described distinct clinicopathologic entity with HL-like features, characterized by shallow, and sharply demarcated MCUs involving the mucosa of oropharynx, GIT, and skin.[10] EBVMCU is associated with immunosuppression either due to immunosuppressive drugs or immunosenescence related to aging. It is characterized histopathologically by a polymorphous cellular infiltrate, including several HRS-like cells. HRS-like cells will be strongly positive for CD 30 and EBER often with downregulation of CD 20. CD 15 is also reportedly positive in around 43% of cases in one study.[10] EBVMCU generally has an indolent clinical course and responds well to conservative treatment. Our patient had an ulceroproliferative growth with obstruction which is outside the currently described clinical spectrum of EBV-positive MCU. To conclude, primary extranodal classical HL in the rectosigmoid being a rare entity, high degree of suspicion is needed for the correct diagnosis and management. Preoperative diagnosis by colonoscopy will aid in proper diagnosis and correct management of these extraordinarily rare occurrences.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al., editors. Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). In: World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues. 4th ed. Lyon: IARC Press; 2008. p. 214-7.  Back to cited text no. 1
    
2.
Gandhi JS, Mehta A, Sharma A, Kamboj M. Primary Hodgkin lymphoma of the ileum. J Cancer Res Ther 2010;6:342-3.  Back to cited text no. 2
    
3.
Fan CW, Changchien CR, Wang JY, Chen JS, Hsu KC, Tang R, et al. Primary colorectal lymphoma. Dis Colon Rectum 2000;43:1277-82.  Back to cited text no. 3
    
4.
Dawson IM, Cornes JS, Morson BC. Primary malignant lymphoid tumours of the intestinal tract. Report of 37 cases with a study of factors influencing prognosis. Br J Surg 1961;49:80-9.  Back to cited text no. 4
    
5.
Bairey O, Ruchlemer R, Shpilberg O. Non-Hodgkin's lymphomas of the colon. Isr Med Assoc J 2006;8:832-5.  Back to cited text no. 5
    
6.
Shapiro HA. Primary Hodgkin's disease of the rectum. Arch Intern Med 1961;107:270-3.  Back to cited text no. 6
    
7.
Sapp M, Perez-Ordonez B, Brenneman F, Imrie K, Morava-Protzner I, Lim MS. EBV-associated perianal Hodgkin's disease in an HIV-positive individual. Am J Hematol 2001;66:42-5.  Back to cited text no. 7
    
8.
da Costa AA, Flora AC, Stroher M, Soares FA, de Lima VC. Primary Hodgkin's lymphoma of the rectum: An unusual presentation. J Clin Oncol 2011;29:e268-70.  Back to cited text no. 8
    
9.
Ambrosio MR, Rocca BJ, Barone A, Mastrogiulio MG, Costa A, Bellan C, et al. Primary anorectal Hodgkin lymphoma: Report of a case and review of the literature. Hum Pathol 2013 ;45:648-52.  Back to cited text no. 9
    
10.
Dojcinov SD, Venkataraman G, Raffeld M, Pittaluga S, Jaffe ES. EBV positive mucocutaneous ulcer – A study of 26 cases associated with various sources of immunosuppression. Am J Surg Pathol 2010;34:405-17.  Back to cited text no. 10
    

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Correspondence Address:
Rekha A Nair
Division of Pathology, Regional Cancer Centre, Thiruvananthapuram - 695 011, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_767_16

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