Successful Surgical Removal of the Largest Known Spleen

BACKGROUND: Splenic marginal zone lymphoma (SMZL) is a rare subtype of non-Hodgkin lymphoma that typically presents with symptomatic splenomegaly. The optimal treatment of SMZL not well established. Case Presentation. A 44-year-old man with a history of low-grade B-cell lymphoproliferative disorder previously treated with chemotherapy presented with a three-month history of rapidly enlarging abdominal girth. He was found to have large symptomatic splenomegaly by computed tomography. After workup, he underwent preoperative angioembolization of the splenic artery followed by successful splenectomy. The spleen measured 45 cm in greatest dimension and weighed 12.14 kg, more than 12% of the patient's total body weight, making this both the largest spleen on record as well as the largest spleen ever surgically removed. Pathology was consistent with splenic marginal zone lymphoma. The patient did well and was discharged home on postoperative day 3. CONCLUSIONS: SMZL is an infrequent condition that leads to progressive symptomatic splenomegaly which can be managed surgically providing symptomatic improvement and an overall satisfactory oncologic outcome. A multidisciplinary approach to complex cases of SMZL splenomegaly is imperative to achieving optimal outcomes.