Splenic cyst deroofing complicated with B lymphoma

Abstract Background: Splenic cysts are uncommon and very rarely malignant therefore their treatment isn’t standardised. In case of symptomatic cysts different surgical approaches have been suggested. Primary malignant lymphoma of the spleen comprises less than 1% of non-Hodgkin’s lymphomas. To our knowledge, only 203 cases of splenic large B-cell lymphoma (LBCL) have been reported to date and only 2 of them were fibrin-associated splenic cysts. Case presentation: 27-year-old model with a 19x13cm splenic cyst without data of malignancy in the preliminary study and therefore treated with laparoscopic deroofing. After histological diagnosis of LBCL with a fibrin/EVB-associated splenic pseudocyst the patient received 4 cycles of Rituximab and a laparoscopic splenectomy was performed due to resurgence of the pseudocyst. No evidence of malignancy has been found in controls performed after the splenectomy. Discussion and Conclusions: the value of tumour markers and radiology for diagnosis of splenic cysts is put into question. Only 60 cases of Fibrin-associated LBCL (FA-LBCL) have been described in the literature therefore there are no treatment guidelines for them even though surgery together with systemic treatment has been the prevalent route with good results in most cases.