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 Table of Contents  
Year : 2018  |  Volume : 51  |  Issue : 5  |  Page : 201-204

Burkitt's lymphoma presenting as ovarian mass and ileocecal intussusception in an adult

1 Department of Surgery, National Cheng Kung University Hospital, Tainan, Dou-Liou, Taiwan
2 Department of Pathology, National Cheng Kung University Hospital, Tainan, Dou-Liou, Taiwan
3 Department of Pediatric Surgery, National Cheng Kung University Hospital, Tainan, Dou-Liou, Taiwan

Date of Submission14-Nov-2017
Date of Decision17-Dec-2017
Date of Acceptance19-Mar-2018
Date of Web Publication17-Oct-2018

Correspondence Address:
Dr. Chung-Hsiung Liu
Department of Surgery, National Cheng Kung University Hospital, Dou-Liou Branch, No.345, Zhuangjing Rd., Douliu City, Yunlin County 640
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/fjs.fjs_168_17

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Burkitt's lymphoma (BL) is usually diagnosed in children and young adults. Adults with Burkitt's lymphoma, particularly those with advanced stage disease, present with poorer prognosis compared to children and very rarely as multiorgan tumors. Involvement of the gastrointestinal (GI) tract presents with GI tract symptoms and can cause intussusception. Herein, we report a 42-year-old, chronic schizophrenic woman, presenting with lower GI bleeding secondary to intussusception, and the radiologic study revealed multiple site organs involvement including terminal ileum, cecum, and bilateral ovaries. She underwent optimal cytoreduction procedure, bilateral salpingo-oophorectomy and radical right hemicolectomy with ileocolostomy, and adjuvant multiagents chemotherapy postoperatively with the regimen of CODOX-M/IVAC for four courses without radiologic evidence of tumor recurrence after a 6-year follow-up.

Keywords: Burkitt's lymphoma, ileocecal intussusception, ovary

How to cite this article:
Liu CH, Wu CL, Sy ED. Burkitt's lymphoma presenting as ovarian mass and ileocecal intussusception in an adult. Formos J Surg 2018;51:201-4

How to cite this URL:
Liu CH, Wu CL, Sy ED. Burkitt's lymphoma presenting as ovarian mass and ileocecal intussusception in an adult. Formos J Surg [serial online] 2018 [cited 2021 Mar 2];51:201-4. Available from: https://www.e-fjs.org/text.asp?2018/51/5/201/243581

  Introduction Top

Burkitt's lymphoma (BL) occurs most commonly in male than female during childhood and young adult and is an uncommon form of Non-Hodgkin's lymphoma in adults. The incidence accounts for 1%–2% of lymphoma in adults, while up to 40% of lymphoma in children.[1] It is a highly aggressive B-cell lymphoma genetically characterized by a chromosomal translocation and deregulation of the c-Myc oncogene. It often presents at extranodal sites and can be divided into three distinct clinic types: endemic, sporadic, and immunodeficiency associated, in which the sporadic BL most often presents as abdominal tumors with bone marrow involvement.[2]

Primary malignant tumors of small intestinal are very rare which are <2% of all GI malignancies. Lymphoma constitutes 15%–20% of all small bowel neoplasms, and ileum is the most common site involved (60%–65%) involving in small bowel lymphoma while colorectal lymphoma constitutes only 0.2% of GI lymphoma, in which cecum is the most common site involved.[3] The clinical presentations are nonspecific, such as colicky abdominal pain, nausea, vomiting, weight loss, bleeding, intussusceptions, or perforation. Involvement of the ovary, about 1.5% of ovarian neoplasms, can mimic a gynecologic malignancy in adults, and bilateral ovarian involvement is frequent.[4] In female adults, BL of the reproductive organ usually presents as pelvic mass with pain, which can be confused with a large leiomyoma, torsion of ovarian cyst, or ovarian tumor. Herein, we report a case with huge extranodal BL involving the ileum, cecum, and bilateral ovaries presenting with symptoms of intussusceptions, gastrointestinal (GI) bleeding, and giant intra-abdominal tumors.

  Case Report Top

A 42-year-old, G1P1, Indonesian woman, married to Taiwanese, who was diagnosed of chronic schizophrenia with stationary status, but poor medicinal compliance. She started to have the symptoms of abdominal pain, nausea, and vomiting with lower GI bleeding for one week. Physical examination revealed pale conjunctiva, palpable, and movable low abdominal mass of about 15 cm × 10 cm in size. Laboratory data showed hemoglobin: 9.2 g/dL and hematocrit: 28.0%. Colonoscopy revealed a protruding mass, about 4 cm × 3 cm in size, within the terminal ileum with mucosal hyperemia. Biopsy revealed granulation tissue with necrotic debris only. Tumor markers CEA, CA19-9, CA153, and CA125 were 0.77 ng/ml, 18.85 U/ml, 21.5 U/ml and 321.0 U/ml, respectively. Abdominal computed tomography (CT) showed a huge intraabdominal mass [Figure 1]a, occupied the whole lower abdomen and intussusception in the ileocecal region [Figure 1]b. Explorative laparotomy showed ileocolic intussusceptions during operation [Figure 2] with a protruding tumor 5 cm × 4 cm × 3 cm, pale-tan in color, in the terminal ileum, involving whole intestinal wall and serosal fat [Figure 3]a. A second tumor, which is not connected with the ileal tumor, of about 5 cm × 2 cm in size is located in the cecum [Figure 3]b. A third and fourth tumor, giant and blizzard lobulated mass, with size and weight of, 10 cm × 8 cm, 480 g and 12 cm × 10 cm, 670 g on the right and left ovary, respectively [Figure 4]. She underwent bilateral salpingo-oophorectomy and radical right hemicolectomy with ileocolostomy. Microscopically, the ileal [Figure 5]a and [Figure 5]d, cecal [Figure 5]b and [Figure 5]e, and bilateral ovarian tumors [Figure 5]c and [Figure 5]f showed diffuse infiltration of monomorphic and medium-size lymphoid cell involving the whole bowel wall with starry-sky pattern macrophages with ingested debris interspersed between tumor cells. These tumor cells have noncleaved round nuclei with coarse chromatin and occasionally prominent nucleoli [Figure 6]. There were 26 lymph nodes dissected from mesentery, in which 18 showed lymphoma cells infiltration. Immunohistochemically, these neoplastic cells were stained positive for CD20, CD10, and bcl-6 but negative for CD3 and bcl-2, and Ki67 index is more than 95% [Figure 7]a, [Figure 7]b, [Figure 7]c, [Figure 7]d, [Figure 7]e. Epstein-Barr encoding region (EBER) in situ hybridization is negative [Figure 8]. The morphologic features and immunoprofile were consistent with malignant BL. HIV titer was not elevated following definitive diagnosis of BL. According to Ann Arbor Staging System, the tumor spread widely into several organs outside the lymph system, which means stage IV disease. She underwent further adjuvant multiagents chemotherapy postoperatively with the regimen of CODOX-M/IVAC for four courses without evidence of tumor recurrence with abdominal CT after 6-year follow-up period.
Figure 1: Abdominal computed tomography shows multiple peritoneal tumors (a) with ileocolic intussusception (white arrow) (b)

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Figure 2: Burkitt's lymphoma presented as ileocolic intussusceptions during operation

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Figure 3: Burkitt's lymphoma in the ileum (A) and cecum (B)

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Figure 4: Giant Burkitt' lymphoma over bilateral resected specimen of ovaries

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Figure 5: The ileum (a and d), cecum (b and e), and ovary (c and f) were invaded by Burkitt lymphoma, composed of medium-sized atypical lymphoid cells with brisk mitoses and typical “starry-sky” pattern

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Figure 6: Starry-sky (Burkitt's lymphoma) in ovary, similar to the cecum and ileum (×400)

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Figure 7: The tumor cells were diffusely positive for CD20 (a), CD10 (b),bcl-6 (c) but negative for bcl-2 (d) and CD3 (e). The ki67 proliferative index was over 95% (f)(×400)

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Figure 8: Epstein–Barr encoding region in situ hybridization is negative (×400)

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  Discussions Top

Synchronous neoplasms can be based on the existence of primary cancers, involving two or more organs, detected either pre/intraoperative or during a 6-month period postoperatively. BL is an aggressive, highly malignant, and rapidly growing B-cell neoplasm which occurred in the abdomen. It usually involved the facial bones, especially the jaw, maxilla, and orbit in children, which is more common in male than female. BL is associated with Epstein–Barr virus (EBV),[5] monoclonal EBV infection, and rearrangement of the c-Myc gene,[6] and in approximately 20% of AIDS-related non-Hodgkin.[7] However, in our case, the EBER in situ hybridization shows negative for the detection of the EBV in tissue sections.

Sporadic Burkitt's lymphoma often presents as abdominal tumor and the isolated GI tract mass can present as intussusception, obstruction, or GI bleeding with accompanying symptoms of abdominal pain, vomiting, and anemia ranging from 2 weeks to year. Related intussusception is often difficult to diagnose or often mislead in adult with acute abdomen. The definitive diagnosis of intra-abdominal lymphoma is difficult preoperatively by routine blood examination, endoscopic examination, and abdominal CT unless the neoplasm is examined histologically. Laboratory values often show anemia due to chronic or acute GI bleeding. Endoscopic examination may reveal a protruding mass with or without sign of bleeding while abdominal CT often shows lesion with mass effect, sign of intussusception, or intestinal obstruction, which make BL difficult to differentiate from other solid neoplasm. As in our case, the abdominal CT revealed that ileocolic intussusception with intestinal obstruction and solid, dense masses, adenocarcinoma, lymphoma, leiomyoma, and leiomyosarcoma was taken into our differential diagnosis.

In general, the role for surgery was limited to obtaining tissue for histopathology and treatment of complications such as GI bleeding and obstruction. However, surgical intervention should include complete resection when it is possible or palliative debulking surgery, with sufficient tissue for histopathologic examination, to relieve abdominal symptoms and optimal cytoreduction. In our case, the patient suffered from lower abdominal pain with ileocolic intussusception, and surgery is the best first option rather than preoperative chemotherapy. It had been reported that survival rate was better with extensive surgical resection than those who had only partial or incomplete resection.

The mainstay and primary treatment of BL is multiagents chemotherapy aggressively, as in our case treated with a regimen of CODOX-M/IVAC for four courses, which improves the survival rate.[8] It is critical to closely monitor serum biochemistry data because of the high risk of tumor lysis syndrome and uric acid nephropathy. About 30% of BL patients suffered from the complications of tumor lysis syndrome, which is associated with excessive tumor load, and chemotoxicity can be reduced following successful surgical debulking.[9] In conclusion, synchronous extranodal BL involving multiorgans is rarely reported. The mainstay treatment of therapy is multiagents chemotherapy while optimal surgery is also necessary for correct histopathologic diagnosis, relief of clinical symptoms, and palliative cytoreduction.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Queiroga EM, Gualco G, Weiss LM, Dittmer DP, Araujo I, Klumb CE, et al. Burkitt lymphoma in Brazil is characterized by geographically distinct clinicopathologic features. Am J Clin Pathol 2008;130:946-56.  Back to cited text no. 1
Molyneux EM, Rochford R, Griffin B, Newton R, Jackson G, Menon G, et al. Burkitt's lymphoma. Lancet 2012;379:1234-44.  Back to cited text no. 2
Ghimire P, Wu GY, Zhu L. Primary gastrointestinal lymphoma. World J Gastroenterol 2011;17:697-707.  Back to cited text no. 3
Rochford R, Feuer G, Orem J, Banura C, Katongole-Mbidde E, Mwanda WO, et al. Strategies to overcome myelotoxic therapy for the treatment of Burkitt's and AIDS-related non-Hodgkin's lymphoma. East Afr Med J 2005;82:S155-60.  Back to cited text no. 4
Yildirim Y. Primary ovarian large B-cell lymphoma in patient with juvenile rheumatoid arthritis treated with low dose Methotrexate. Gynecol Oncol 2005;97:249-52.  Back to cited text no. 5
Brady G, MacArthur GJ, Farrell PJ. Epstein-Barr virus and Burkitt lymphoma. J Clin Pathol 2007;60:1397-402.  Back to cited text no. 6
Erikson J, ar-Rushdi A, Drwinga HL, Nowell PC, Croce CM. Transcriptional activation of the translocated c-myc oncogene in Burkitt lymphoma. Proc Natl Acad Sci U S A 1983;80:820-4.  Back to cited text no. 7
Stepniak A, Czuczwar P, Szkodziak P, Wozniakowska E, Wozniak S, Paszkowski T. Primary ovarian Burkitt's lymphoma: A rare oncological problem in gynaecology: A review of literature. Arch Gynecol Obstet 2017;296:653-60.  Back to cited text no. 8
Kemeny MM, Magrath IT, Brennan MF. The role of surgery in the management of American Burkitt's lymphoma and its treatment. Ann Surg 1982;196:82-6.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]


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