Gastroduodenal Burkitt's lymphoma in an immunocompetent patient
Linfoma gastroduodenal de Burkitt en un paciente inmunocompetente
Eduardo Rodrigues-Pinto1, Regina Gonçalves1, Elsa Fonseca2 and Guilherme Macedo1
Departments of 1Gastroenterology and 2Pathology. Centro Hospitalar São João. Porto, Portugal
Case report
]]> The stomach is the most frequent site of extranodal malignant lymphomas. The most common gastric lymphomas are mucosa-associated lymphoid tissue (MALT) and diffuse large B cell lymphomas. Burkitt's lymphoma is a highly aggressive non-Hodgkin lymphoma. In the sporadic form, patients usually present with abdominal disease, with involvement of the distal ileum, cecum and mesentery. Although the true incidence of gastroduodenal Burkitt lymphoma is not yet defined, non-endemic gastroduodenal Burkitt's lymphoma is rare in non-HIV adult population. We report a case of gastroduodenal involvement by Burkitt lymphoma in an immunocompetent patient, previously healthy, who presented with persistent epigastric pain, cervical swelling, horizontal diplopia and paresthesias. Viral markers were negative. On upper endoscopy, multiple raised ulcerated nodules, some with central depression were observed on the greater curvature of the gastric body and antrum, and numerous smaller nodules were present in the second and third part of the duodenum. Gastric and duodenal biopsies showed expansion of the lamina propria by a diffuse infiltrate of neoplastic lymphoid cells of intermediate/large size, predominantly regular nuclei with dispersed chromatin, high mitotic index and signs of apoptosis. On immunohistochemistry, tumour cells showed positive staining for CD20 and CD10. Helicobacter pylori infection was not detected. Induction chemotherapy with intrathecal chemotherapy was preformed with improvement of neurologic and digestive symptoms.
Discussion
The stomach is the most frequent site of extranodal malignant lymphomas (1-7% of all gastric malignancies). The most common gastric lymphomas are mucosa-associated lymphoid tissue (MALT) and diffuse large B cell lymphomas (1). Burkitt's lymphoma is a highly aggressive non-Hodgkin lymphoma. In the sporadic form, patients usually present with abdominal disease, with involvement of the distal ileum, cecum and mesentery (2). Although the true incidence of gastroduodenal Burkitt lymphoma is not yet defined, non-endemic gastroduodenal Burkitt's lymphoma is rare in non-HIV adult population (3,4).
We report here on a case of gastroduodenal involvement by Burkitt lymphoma in an immunocompetent patient.
A previously healthy 34-year old man presented with persistent epigastric pain, cervical swelling, horizontal diplopia and paresthesias. Examination showed cervical lymphadenopathy, epigastric pain with the remainder of physical examination being normal. Blood tests showed leukocytosis with immature cells in peripheral blood, thrombocytopenia and elevated transaminases and cholestasis. Viral markers were negative. Abdominal ultrasound showed multiple hypoechoic hepatic nodules. On upper endoscopy, multiple raised ulcerated nodules (2 to 3 cm in diameter), some with central depression were observed on the greater curvature of the gastric body and antrum (Fig. 1A and B), and numerous smaller nodules were present in the second and third part of the duodenum (Fig. 1 C).
Gastric and duodenal biopsies showed expansion of the lamina propria by a diffuse infiltrate of neoplastic lymphoid cells of intermediate/large size, predominantly regular nuclei with dispersed chromatin, high mitotic index and signs of apoptosis (Fig. 2 A). On immunohistochemistry, tumour cells showed positive staining for CD20 and CD10 (Fig. 2 B and C). Helicobacter pylori infection was not detected. These morphological and immunophenotypical features were consistent with Burkitt's lymphoma.
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Further evaluation revealed bone marrow, hepatic and central nervous system involvement. Induction chemotherapy was preformed with rituximab, cyclophosphamide, vincristine, prednisone, leucovorin, doxorubicin and methotrexate with intrathecal chemotherapy with improvement of neurologic and digestive symptoms.
References
1. Baumgaertner I, Copie-Bergman C, Levy M, Haioun C, Charachon A, Baia M, et al. Complete remission of gastric Burkitt's lymphoma after eradication of Helicobacter pylori. World J Gastroenterol 2009;15:5746-50. [ Links ]
2. Collins RH. Gastrointestinal lymphomas. In: Feldman M, Friedman LS, Sleisenger MH, editors. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 9th ed. Philadelphia: Saunders; 2003. [ Links ]
]]>3. Sharma A, Raina V, Gujral S, Kumar R, Tandon R, Jain P. Burkitt's lymphoma of stomach: A case report and review of literature. Am J Hematol 2001;67:48-50. [ Links ]
4. Collins J, Katon R, Harty-Golder B. Burkitt's lymphoma presenting with gastroduodenal involvement. Endoscopic description and review of the literature. Gastroenterology 1983;85:425-9. [ Links ]
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