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.