Burkitt’s Lymphoma of the Stomach: A Case Report and Review of the Literature

    

    

    Figure 4:  OGD 30/4/2013. Ongoing GI bleed.
    
    

At the moment the patient is undergoing an IV° cycle of chemotherapy with peripheral blood stem-cell collection in case an autologous transplantation becomes necessary. The possibility of performing an allogeneic transplantation is being evaluated. Gastrectomy was not considered because the bleeding settled after the second OGD.

Discussion

Burkitt´s lymphoma is an aggressive form of B-cell NHL. The US incidence is 0.3 per 100,000 [3]. While BL is the commonest NHL in childhood, it accounts for >5% of adult lymphomas, and is thought to be under-researched in adults [4]. The sporadic (non-endemic) form encountered outside of Africa usually presents with an abdominal mass, most commonly localized at the ileocaecal region; it is rare for BL to manifest primarily in the stomach or duodenum [1,5]. The prevalence of this is unknown due to a paucity of published literature, consisting mostly of case reports and small case series (summarized in Table 1). Reviews are outdated by two decades. Cases display much heterogeneity in disease presentation, gastric site, patient age, and associated features such as infection. Gastric BL is prone to developing complications such as perforation and haematemesis, with some patients requiring surgery [6-8].

The aetiology of sporadic BL is unclear. A number of reports have demonstrated BL arising in association with H. Pylori infection [7-10]. Notably, one group demonstrated concomitant remission of gastric BL with HP eradication alone (no chemotherapy was initiated) [9]. In our case no HP was detected. HP has been established as a causative agent in MALT lymphoma where its eradication leads to lymphoma regression [11-13], and has also been associated with DLBCL, both through evolution from MALT, and spontaneous DLBCL; here eradication also promotes healing [14]. The low incidence of gastric BL means that insufficient data exists to establish any causative association. Such a link might lead to improved management of BL in a similar manner as in MALT and DLBCL.

Our patient´s serology showed prior resolved acute HBV infection. This is significant as HBV has been implicated in studies as an oncogenic agent in various subtypes of NHL [15-17] and cases have reported BL associated with HBV infection [18,19]. Causative links have not been firmly established. It has been shown that HBV can persist in tissues, undetectable by serology, and therefore the presence of ongoing HBV infection in NHL cases may be underrecognized [15,20]. Further exploration of the role of the virus in NHL development is necessary to establish a causative role. EBV is the causative agent in endemic BL, and is present in a minority of sporadic BL cases. Our patient´s pathology samples were negative for EBV suggesting this was not a causative agent.

Chemotherapy is highly effective in BL and is the mainstay of management. The role of surgery is restricted to managing complications. Three of the cases of gastric BL previously reported required surgical resection of the stomach, and other published cases required management of GI perforation [21,22]. Our case presents a dilemma, as radical surgery on a BL patient has risks due to the chemotherapy-induced neutropaenia, and the damaged oozing mucosa. Furthermore, gastrectomy carries several complications and impairs the quality of life of a young patient, so where possible we believe that a conservative approach restricted to chemotherapy should be followed. Published studies on this condition are lacking, therefore more reports should be encouraged to identify patients’ characteristics and dictate the best management. Serological screening including HP should be carried out and, if positive, eradication therapy should be prescribed.

Conclusion

BL affecting the stomach remains rare and likely under-reported. No up to date review articles exist in the literature and while sporadic reports show associations with EBV, HP and HBV, no causal relationship is established (Table 1). Such a link would improve understanding of pathogenesis, and potentially offer additional treatment options as has been shown for DLBCL and MALT tumours. Finally, there is no clear guidance on when to perform surgery for gastro duodenal BL. Published cases vary; generally operating in emergency circumstances (for instance, several had perforations). We believe that this is an appropriate role, as data are lacking on curative resections. Further case reports are warranted to improve our understanding and management of this disease.

  

    
Study
    
Age
    
Gender
    
Site
    
Appearance
    
Notable features 
    
Surgery
    
Outcome
  
  

    
Angotti et al 2012 [23]
    
4
    
M
    
Stomach anterior and posterior walls
    
Ulcerated masses
    
 
    
No
    
Undisclosed
  
  

    
Ziade et al 2012 [24]
    
15
    
F
    
Stomach, duodenum, jejunum
    
Raised, ulcerated tumours
    
EBV +ve; Krukenberg tumour
    
No
    
Remission
  
  

    
Colovic et al 2011 [6]
    
30
    
M
    
Antrum
    
Ulcero-infiltrative lesion
    
 
    
Subtotal distal gastrectomy
    
Remission
  
  

    
Ergun Met et al 2011 [25]
    
42
    
F
    
Mid portion of ant wall
    
Large Ulcer
    
Subsequent perforation 
    
Closure
    
Remission 
  
  

    
Kesik et al 2010 [7]
    
7
    
M
    
Antrum
    
Ulcerated masses
    
HP positive.

    EBV/HBV -ve
      
Perforation
    
Semi-total gastrectomy for perforation;    oesophago-jejunostomy
    
Remi­­ssion
  
  

    
Baumgartner et al 2009 [9]
    
30
    
F
    
Lesser curvature of antrum
    
Ulcer 5x5cm
    
Remission with HP eradication
    
No
    
Remission
  
  

    
Chogle et al 2009 [26]
    
11
    
M
    
Body of somach
    
Deep ulcers
    
HIV positive
    
No
    
Death
  
  

    
Chieng et al 2009 [27]
    
9
    
M
    
Greater curve of gastric body, duodenum
    
Multiple raised ulcerated tumours
    
Presented as protein losing enteropathy
    
No
    
Remission
  
  

    
Grewal et al 2007 [28]
    
12
    
M
    
Proximal lesser curvature
    
Ulceration, thickened walls
    
HP infection
    
No
    
 
  
  

    
Sharma et al 2001 [29]
    
35
    
M
    
Fundus, body and pylorus
    
Thick folds, lack of distensibility
    
Nil
    
No
    
Death
  
  

    
Shannon et al 2000 [30]
    
53
    
M
    
anterior wall of duodenum
    
Ulcer, perforation
    
HP +ve, perforation
    
Partial gastrectomy
    
 
  

Table 1:  Previous case reports on Gastric Burkitt’s Lymphoma.