Burkitt Lymphoma in Gastrointestinal Tract: a Report of Two Cases

Abstract

SUMMARYBurkitt lymphoma, a type of non-Hodgkin B-cell lymphoma, is the fastest growing human cancer, presenting pathologically with a ‘starry sky’ pattern. It is most often found in the abdomen and the jaw, however, localization in the abdomen other than the ileocecal area is very rare and described only in a handful of cases. Standard treatment consists of initial tumor cytoreduction followed by intense chemotherapy. Most of the relapses occur within one year of the diagnosis, while the 5-year survival is around 80%. We present two cases which are specific for unusual location of Burkitt lymphoma in the colon and stomach, in immunocompetent patients with negative Epstein-Barr virus tests. Also, one of the patients presented is one of the oldest ever reported with abdominal Burkitt lymphoma, while the other patient is an example of diagnostic difficulties in distinguishing Burkitt lymphoma from similar lymphomas. Due to the rapidly growing tumors and urgent need for cytoreductive surgery, it is crucial to consider the diagnosis of Burkitt lymphoma even in atypical localizations or absence of the common risk factors associated with Burkitt lymphoma.

Primary Gastrointestinal Lymphoma

“…BL, a type of non-Hodgkin BCL, displays a diffuse, monotonous infiltrate of medium-sized neoplastic lymphoid cells with round nuclei and little cytoplasm showing finely clumped and dispersed, with multiple basophilic nucleoli, presenting pathologically with a "starry sky" pattern [15,29,109]. It is most often found in the abdomen and the jaw, however, localization in the abdomen other than the ileocecal area is very rare [15,109].…”

“…BL, a type of non-Hodgkin BCL, displays a diffuse, monotonous infiltrate of medium-sized neoplastic lymphoid cells with round nuclei and little cytoplasm showing finely clumped and dispersed, with multiple basophilic nucleoli, presenting pathologically with a "starry sky" pattern [15,29,109]. It is most often found in the abdomen and the jaw, however, localization in the abdomen other than the ileocecal area is very rare [15,109]. Mutations in the transcription factor 3 gene, TCF3 (19p13.3) or in its negative regulator ID3 (1p36.12) take place in about 70% of sporadic and immunodeficiency-related BL and less frequently (40%) in endemic cases.…”

“…The most common cytogenetic change is t(8;14)(q24;q32) and t(8;22)(q24;q11) translocations that are characterized by overexpression of c-MYC oncogene and can be detected in 90% of cases by classical karyotyping or FISH. The proliferation index Ki-67 of BL is very high, usually over 95%, so it is not surprising because BL is the fastest growing human cancer [29,109]. Cytogenetic analysis is recommended to allow a clear distinction between BL and other c-MYC-driven B-cell NHL, especially DLBCL [29].…”

“…The morphological distinction between BL and DLBCL has been problematic for pathologists [12,109]. Distinguishing between these two lymphomas, however, is critical, especially in adults (BL is rare in adults and rarely found in the stomach and colon), as the two diseases are treated differently [109].…”

An unusual cause of large bowel obstruction: are we aware of this?

“…Three distinct variants of BL have been recognized; endemic, sporadic and immunodeficient. Endemic usually appears in African countries usually in children, whereas sporadic form shows no predilection as far as geographical areas are concerned [ 2 ]. On the other hand, immunodeficient variant has been reported in patients suffering from human immunodeficiency virus (HIV), in solid organ recipients as well as in patients with congenital immunodeficiency diseases.…”