Granulocytic sarcoma is a rare extramedullary tumor composed of myeloid progenitor cells. Primary involvement of the biliary tract without evidence of leukemia is exceedingly rare. Here, we report an isolated biliary granulocytic sarcoma in a 30-yr-old man who presented with jaundice, fever, and chill without any evidence of leukemia. However, five months after the diagnosis, he developed acute myelogenous leukemia with multilineage dysplasia and chromosomal abnormality. A rare possibility of biliary granulocytic sarcoma should be considered as a differential diagnosis in patients with obstructive jaundice. A histologic evaluation by aggressive diagnostic intervention is important and may improve prognosis.

A 30-yr-old man was admitted to our hospital because of jaundice and mild fever with a chill. The patient had been well until 3 months earlier, when he began to develop right upper quadrant pain. One month before admission, the pain worsened and at times radiated into his back. On admission, a physical examination was unremarkable except for icteric sclera. An abdominal examination revealed right upper quadrant tenderness but did not disclose hepatosplenomegaly or a palpable mass. Laboratory tests on admission showed elevated total bilirubin (6.9 mg/dL; normal range 0-0.5), serum aspartate aminotransferase (201 µ/L; normal range 0-40), serum alanine aminotransferase (814 mg/dL; normal range 0-40), and alkaline phosphatase (158 µ/L; normal range 53-128). Complete blood cell counts were within normal limits, with no blast cells. Ultrasonography (US) revealed thickening of the gallbladder wall and multiple tiny stones with some sludge in the gallbladder. Endoscopic retrograde cholangiopancreatography (ERCP) revealed bile duct strictures (Fig. 1). Endoscopic nasobiliary drainage (ENBD) was performed using a 5-Fr. plastic stent. Abdominal computed tomography after ENBD disclosed a low density rim around the ENBD tube, but no definitive abnormal enhancing lesion of the wall or mass was recognized (Fig. 2). The jaundice was temporally relieved after ENBD and the patient received open cholecystectomy under the impression of cholelithiasis with bile duct stricture. The histologic diagnosis of the gallbladder was chronic cholecystitis with cholelithiasis. Because jaundice had not resolved after cholecystectomy, right lobectomy with hepaticojejunostomy was performed. During the operation a diffuse stricture from the middle common bile duct extending to right and left hepatic ducts with wall thickening was observed. A pathologic examination showed a monomorphous and discohesive infiltration of tumor cells along the bile duct wall (Fig. 3A, B). Tumor cells were of medium size with large nuclei showing dispersed chromatin, and one or more distinct but not prominent nucleoli. The cytoplasm was deeply eosinophilic and granular reminiscent of metamyelocytes, and indicative of myeloid differentiation (Fig. 3B). Immunohistochemistry showed positivity for myeloperoxidase (1: 20,000, Dako, Denmark)(Fig. 3C), CD34 (1:100, Dako, Denmark), and lysozyme (1:100, Zymed, New Zealand) but negativity for CD3 (1:200, Novocastra, U.K.) and CD20 (1:500, Novocastra, U.K.). The liver was not involved by the tumor. The histologic diagnosis was granulocytic sarcoma involving the right and left hepatic ducts, superior bile duct, and cystic duct. A bone marrow biopsy taken at the time was normal, and the patient had no symptoms or signs of leukemia. The patient was then followed without other therapies such as chemotherapy or radiotherapy. Five months after the initial diagnosis of granlocytic sarcoma, peripheral blood smear showed normochromic normocytic anemia (Hb 11.4 g/dL, Hct 33.1%) with aniso- and poikilo-cytosis, neutropenia (3,700/µL) with 10% blasts, and thrombocytopenia (73,000/µL) with giant forms. Bone marrow aspiration and biopsy showed leukemic blasts with abnormal nuclear folding, and cytoplasmic blebs which were counted up to 40.1% of all nucleated cells and infiltrated between normal and dyshematopoietic erythoid and myeloid cells(Fig. 4). Those blasts were positive for myeloperoxidase, Sudan-black B, and peroxidase in cytochemical stain and expressed CD13, CD33, myeloperoxidase, and CD34 in flow cytometric analysis. Megakaryocytes with dysplasia were hardly observed in aspiration smear but increased in number in biopsy. A diagnosis of acute myelogeneous leukemia with multilineage dysplasia was made according to the WHO classification or M2 by the French-American-British (FAB) classification. A chromosomal study showed 45,XY,del(3)(q21q27),-16,add (21)(q22) [18]/46,XY[2]. The patient received induction chemotherapy with idarubicin and cytarabine. A follow up bone marrow examination 21 days after the induction chemotherapy showed persistent acute myelogenous leukemia (AML) with multilineage dysplasia (Blasts: up to 4.3% of all nucleated cells).