Case Presentation

A 51-year-old lady was admitted to hospital with chronic abdominal pain, weight loss (5% of body weight), history of undulant fever and nocturnal sweating. In physical examination, orthostatic hypotension, conjunctival pallor and abdominal right upper quadrant tenderness were noticed. Laboratory tests showed elevated Erythroid Sedimentation Rate (ESR=87mm/h) and normocytic normochromic anemia (hemoglobin=9.3 g/dl). Abdominal ultrasound revealed a 37mm×49mm cyst-like mass in the left lobe of liver (4A segment) without internal septation, and no other abnormality in abdominopelvic cavity was identified. Computed tomography showed a non-enhanced hypodense mass with regular margin (sized: 4×5cm) in the left lobe of liver (Figure 1) without any further abnormal findings.
We re-reviewed patient’s past medical history for any diagnostic clues for the solitary liver mass. Personal and family history was negative for chronic liver disease and viral hepatitis risk factors. No stigmata for cirrhosis were identified. Viral hepatitis panel, alpha fetoprotein and CA19-9 were undetectable. Screening colonoscopy for colorectal cancer was normal 6 months before symptoms start.
Finally, she was admitted to Ghazi Hospital, affiliated to Tabriz University of Medical Sciences, for diagnostic mass resection and underwent partial hepatectomy surgery. In pathological study, the liver tissue contained a cystic lesion composed of proliferated histiocytes, with eosinophilic nucleolus, some double or multinucleated, occasionally showing lymphophagocytosis (emperipolesis). The histiocytes were mixed with a polymorph infiltration composed of mononuclear and plasma cells. Histiocytes stained positively for CD68 and protein S-100, and plasma cells were positive for CD138, kappa and lambda. Hodgkin’s lymphoma was ruled out by negative CD15 and CD30 staining. Peripheral blood smear and bone marrow aspiration were normal. Bone marrow biopsy showed lymphoplasmacytosis with slight megakaryocytosis. The liver histological pattern was consistent with Rosai-Dorfman disease (RDD). The patient was consequently referred to the hematology service and we decided to start prednisone, 30mg/day. She was followed conservatively with repeat imaging after 3 months. The follow-up CT revealed no recurrence and she responded clinically to steroids. She continues to be followed with yearly imaging and 5mg/day prednisone and will likely remain on steroids for the long term.