Sporadic Burkitt lymphoma presenting with peritoneal lymphomatosis

Abstract

Burkitt lymphoma (BL) is a highly aggressive non‐Hodgkin B‐cell lymphoma. It has a doubling time of 24 h. Fortunately, it is highly sensitive to aggressive chemotherapy. Sporadic BL often affects the mesenteric and retroperitoneal lymph nodes. Extra‐nodal involvement includes the ileocecal area, stomach, kidneys, gonads, and central nervous system. Peritoneal lymphomatosis is a rare presentation. We report a case of BL presenting with peritoneal lymphomatosis to emphasize the importance of early histological diagnosis of any peritoneal thickening.

Burkitt lymphoma should be considered as a rare differential diagnosis in patients presenting with peritoneal thickening. It is a highly aggressive non‐Hodgkin lymphoma with a doubling time of 24 h but it is highly sensitive to chemotherapy. Thus, early histological diagnosis is needed to initiate rapid treatment.

1. INTRODUCTION

Burkitt lymphoma (BL) is a rare and very aggressive non‐Hodgkin B‐cell lymphoma. It is a fast‐growing neoplasm that rarely affects adults. There are three clinical types: endemic, sporadic, and immunodeficiency‐associated. ¹ Sporadic BL often affects mesenteric and retroperitoneal lymph nodes. Extra‐nodal involvement includes ileocecal area, stomach, kidney, gonads, and central nervous system. In the non‐endemic form, patients typically present with an abdominal mass and its consequences. ² This abdominal mass can be associated with ascites, and the diagnosis of BL can often be made by paracentesis. Peritoneal lymphomatosis is rare and is usually associated with diffuse B‐cell lymphoma. It incites to consider other etiologies such as peritoneal carcinomatosis or tuberculosis. ³ We report a case of BL presenting with peritoneal lymphomatosis confirmed by histological evaluation.

2. CASE REPORT

The patient is a 16‐year‐old Caucasian male born in Tunisia, with no relevant medical or surgical history. He presented to the emergency department with progressive increase in abdominal volume. No other symptoms were reported. In particular, there was no history of fever. He denied nausea, vomiting, or changes in bowel habits. On physical examination, there was marked abdominal distension and shifting dullness. No palpable masses or lymphadenopathies were noted. Laboratory findings were as follows: hemoglobin was normal (12.3 g/dl), WBC count was 10.05 × 10³ U/L (N: 4–10 × 10³ μl), total bilirubin was 7 μmol/L (N: <22 μmol/L); alanine aminotransferase was 38 U/L (N: <50 U/L); alkaline phosphatase was 151 U/L (N: 38–126 U/L); creatinine was 47 μmol/L (N: 30–110 μmol/L). There was a slight elevation in aspartate aminotransferase: 58 U/L (N: <50 U/L) and urea: 3.8 mmol/L (N: 2–7.5 mmol/L) and a very high lactate dehydrogenase (LDH): 2596 U/L (N: 313.0–618.0 U/L). Later laboratory investigations, including tumor markers: carcinoembryonic antigen (CEA), carbohydrate antigen 19–9 (CA 19.9), alpha‐fetoprotein (AFP), were within normal levels. Abdominal computed tomography scan showed peritoneal effusion and nodular thickening of the peritoneum with a hypodense and heterogenous appearance of the mesentery and the omentum (Figure 1). Peritoneal fluid analysis revealed chylous (Figure 2) and exudative ascites (protein level 45 g/L). Triglyceride level in peritoneal fluid was 0.45 g/L. Exploratory laparoscopy showed large volume chylous ascites, micronodular omentum, associated with multiple adhesions of the small bowel. Peritoneal and omental biopsies were performed. Macroscopically, a fish flesh appearance was noted (Figure 3). Histology revealed medium‐sized tumor cells with many mitotic figures and apoptotic cells. At immunohistochemistry, the tumor cells are all positive for MIB1, compatible with the diagnosis of Burkitt's lymphoma. Ki‐67 proliferation index is 100% (Figure 4). HIV serology was negative. Hbs Ag was also negative. A chemotherapy protocol (intravenous adriamycine, cyclophosphamide, vincristine associated with oral prednisone) was started. The patient was then transferred to the Department of Hematology at a local oncological institution. Unfortunately, the disease progressed under chemotherapy and the patient died within 6 months of diagnosis.

FIGURE 1.

Abdominal CT scan: Nodular thickening of the peritoneum (red arrow) with a hypodense and heterogenous appearance of the mesentery (green arrow) and the omentum (Blue arrows).

FIGURE 2.

Chylous ascites

FIGURE 3.

Macroscopic examination: Fish flesh appearance.

FIGURE 4.

Histology: Burkitt lymphoma: Medium‐sized tumor cells with many mitotic figures and apoptotic cells (H&E staining 400×) immunohistochemistry shows strong and homogeneous positivity (100%) for Ki‐67 by MIB1 antibody staining (400×).

3. DISCUSSION

Burkitt lymphoma is a rare and highly aggressive non‐Hodgkin B‐cell lymphoma. ¹ There are three clinical types: endemic, sporadic, and immunodeficiency‐associated. ⁴ The endemic variant occurs in specific regions (equatorial Africa, Papua New Guinea) and in young males (4–7 years old). ¹ Immunodeficiency‐associated BL affects HIV‐infected patients. Sporadic BL occurs worldwide. ³

The pathogenesis of BL involves a chromosomal translocation (t (8;14), t (8;22) or t (8;2)) deregulating the expression of the c‐MYC proto‐oncogene on chromosome 8q24. ⁴ , ⁵ The pathogenesis involves in some cases an oncogenic virus, the Epstein–Barr Virus (EBV). EBV infection is present in 100% of endemic cases and in 25%–40% of sporadic and AIDS‐associated cases. ¹

Sporadic BL is one of the least common non‐Hodgkin lymphomas. ² The incidence of sporadic BL is 2–3 cases per million population. ¹ The median age of diagnosis is 30 years. ⁴ The male‐to‐female ratio is 3–4/1. ⁴ Our patient was a 16‐year‐old boy.

Sporadic BL often affects the mesenteric and retroperitoneal lymph nodes. Extra‐nodal involvement includes ileocecal area, stomach, kidneys, gonads, and central nervous system. ¹ , ⁴ Patients mostly present with abdominal pain, an abdominal mass, or ascites associated with fever, night sweats, and weight loss. ⁴ , ⁶ , ⁷ Peritoneal effusions in lymphomas have multiple mechanisms. These include impaired lymphatic drainage due to lymph node involvement, thoracic duct obstruction, venous obstruction, or serous involvement by the lymphoma. ⁸ In our case, ascites is probably explained by two mechanism: (1) Peritoneal lymphomatosis, which is the intra‐peritoneal spread of the lymphoma, and (2) obstruction of lymphatic drainage, which is why the ascites was chylous.

Peritoneal lymphomatosis is a rare presentation. ⁴ In our case, BL affected the mesentery and the omentum. Involvement of the omentum, which is a fibrofatty tissue devoid of lymphoid tissue, is rare. ⁹ Alternative causes of peritoneal thickening include peritoneal carcinomatosis, peritoneal mesothelioma, and tuberculous peritonitis. ³ , ⁹ Radiological features that may favor lymphomatosis include omental caking with bulky homogeneous masses, homogeneous smooth thickening, non‐compartmentalized ascites, and enlarged lymph nodes. ⁶ , ⁹

Peritoneal lymphomatosis, especially if secondary to BL, has a rapid progression with a doubling rate of 24 h. ⁷

Besides, it has a high responsive rate to chemotherapy. ⁶

Thus, an early and precise histological diagnosis is very important. Biopsy with morphology, immunophenotyping, and genetic analysis are used for the diagnosis. ⁷ Biopsy could be CT‐guided or surgical. On gross examination, BL presents as a hemorrhagic, necrotic mass with a fish‐flesh appearance. Microscopically, there is a high proliferation and apoptosis index which explains the classic starry‐sky appearance. ¹ Tumor cells are intermediate in size, non‐pleomorphic with basophilic cytoplasm. They express CD 10, BCL6, CD20, CD79a, and CD45. ¹⁰ The proliferation index measured by ki67 approaches 100%. EBV expression is detected in up to 40% of sporadic cases. Cytogenetic studies demonstrate a MYC translocation, which is highly characteristic of the disease. ¹ , ¹⁰ Serous effusions (SE) cytology could be another way to confirm the diagnosis. SE cytology combined with ancillary tests (flow cytometry, polymerase chain reaction, fluorescence in situ hybridization) is effective in the diagnosis of BL (concordance rate of cytological‐histological diagnosis of 62.5%). ¹¹ It could be an option in cases where biopsy is unavailable.

Work‐up should include routine hematology and biochemistry panels, lactate dehydrogenase (LDH), uric acid, testing for HIV, and hepatitis B virus. ¹ The staging is based on computed tomography imaging with or without positron emission tomography, bone marrow biopsy, and cerebrospinal fluid analysis by cytology and flow cytometry. ¹ The modified Ann Arbor is used for staging (adult burkitt lymphoma). Treatment for young adults is based on standard regimens such as CODOX‐M/IVAC (cyclophosphamide, vincristine, doxorubicin, and high‐dose methotrexate with ifosfamide, cytarabine, etoposide, and intrathecal methotrexate). ¹ , ⁷ Our patient received intravenous adriamycine, cyclophosphamide, vincristine associated with oral prednisone. Aggressive chemotherapy regimens with central nervous system prophylaxis have good success rates with complete remission in 75–90% of cases and overall survival up to 50%–70%. ¹² In older and less fit patients, less toxic strategies should be considered. ¹

4. CONCLUSION

Burkitt lymphoma should be considered as a rare differential diagnosis in patients presenting with peritoneal thickening. It is a highly aggressive non‐Hodgkin lymphoma with a doubling time of 24 h but it is highly sensitive to chemotherapy. Thus, early histological diagnosis is needed to initiate treatment in time.

AUTHOR CONTRIBUTIONS

Sabbah Meriam and Nakhli Abdelwahab wrote the case report. Bellil Nawel was the direct manager of the patient. Khanchel Fatma gave us the histopathological images. Chammakhi Chiraz was the radiologist who made the CT scan and gave us the images. Dalila gargouri is the head of department of the gastroenterology. Ben Farhat Fatma helped us to revise the article and to collect the missed data especially the evolution of the patient.

FUNDING INFORMATION

No fundings were obtained for this article.

CONFLICT OF INTEREST

No conflicts of interest are reported for this submission.

CONSENT

Written informed consent was obtained from the patient to publish this report in accordance with the journal's patient consent policy.

Sabbah M, Nakhli A, Bellil N, Khanchel F, Haddad D, Chammakhi C. Sporadic Burkitt lymphoma presenting with peritoneal lymphomatosis. Clin Case Rep. 2022;10:e06727. doi: 10.1002/ccr3.6727

DATA AVAILABILITY STATEMENT

None.