Case Presentation: A 32-year-old female of Chinese descent with no past medical history presented with abdominal pain, nausea and bloating 1 month in duration. Initial labs remarkable for normal blood counts, positive CA 125 of 1118 U/mL and CA 19-9 of 53 U/mL. Abdominal CT demonstrated bilateral ovarian masses, peritoneal carcinomatosis with omental caking and a large amount of abdominal and pelvic ascites. Peritoneal fluid showed a neutrophil count of 15,920 with 20,000 red blood cells. Cytology was positive for large, rare CD 20 positive, atypical cells. Flow cytometry pertinent for monoclonal B cell lymphoproliferative disease (CD19+, CD 20+, CD 5, CD 10+ lambda light chain restricted) suggestive of Non-Hodgkin’s Lymphoma. FISH studies obtained demonstrated re-arrangement at BCL2 and MYC genes. Patient was started on Dexamethasone 40mg for 4 days, followed by induction chemotherapy consisting of the EPOCH regimen. The patient was later discharged and has continued to receive chemotherapy outpatient without further accumulation of ascites.
Discussion: Diffuse large B cell lymphoma (DLBCL) is the most common subtype of B cell non-Hodgkin lymphoma (NHL), accounting for approximately 30-40% of NHL worldwide. Typical presenting symptoms include rapidly enlarging, non-painful lymph nodes, fever, weight loss and night sweats. Extra-nodal disease with origins in the gastrointestinal tract can occur in approximately 40% of cases. Abdominal ascites as an initial presentation of DLBCL is rare but has been described. Peritoneal fluid accumulation may occur as a result of direct tumor invasion of the peritoneal cavity, or lymphatic obstruction. It has also been proposed that DLBCL can enhance cytokine mediated capillary permeability leading to “capillary leak syndrome.” Furthermore, mesothelial cell activation in response to cancer activity can further promote ascites development. Through these same mechanisms of mesothelial cell activation, the tumor marker CA 125 is produced. Elevations in CA 125 may indicate high tumor burden, extensive disease spread, advanced disease stages and poorer prognosis. Although preliminary workup was suggestive of ovarian cancer, further investigation revealed a diagnosis of lymphoma. Treatment rapidly resolved the ascites and remains ongoing. Although CA 125 and CA 19-9 are tumor markers associated with ovarian cancer, they are not specific and can also be evaluated in DLBCL with some prognostic utility.
Conclusions: Diffuse Large B Cell Lymphoma (DLBCL) can rarely present as ascites with peritoneal carcinomatosis. The diagnostic utility of tumor markers such as CA 125 and CA 19-9 are limited by low specificity but may have prognostic utility once a definitive diagnosis has been reached.