Granulocytic sarcoma usually occurs in one of the following three clinical settings. The first is in patients with previous or current AML, and the second in patients as a sign of blast transformation of CML or of some other chronic myeloproliferative disorder. Third setting is in patients with no other clinical evidence of hematologic disease at the time of diagnosis. Neiman et al. (5
) reported 61 patients with granulocytic sarcoma, in which 48% of the cases were associated with myeloproliferative disorder, 30% presented with no overt leukemia, and 22% occurred as the initial presentation or during the course of AML. The most common sites of involvement were the periosteum, soft tissue and bone followed by the lymph nodes and skin, however it may be found in any location including gingiva, pancreas, breast, testis, spinal cord, or ovary (5
Isolated biliary involvement by granulocytic sarcoma is extremely rare. Only four cases of biliary granulocytic sarcoma have been reported in the English literature (). The first case (14
) was presented with a sign of AML relapse. The patient received chemotherapy because of AML M2 and reached complete remission. However, 29 months later, the patient developed jaundice and abdominal pain, and was diagnosed as having a granulocytic sarcoma in the common bile duct. The second case (15
) was of a 45-yr-old man who underwent allogenic bone marrow transplantation (BMT) because of chronic myelogeneous leukemia in blast crisis. On day 69 after BMT, he presented with anorexia and jaundice and was diagnosed as having a granulocytic sarcoma by open biopsy. Third case (16
) was of an 84-yr-old man presented with jaundice. A CT scan showed a low-density mass at the porta hepatis, and thickening of the gallbladder wall which suggests gallbladder carcinoma. Endoscopic retrograde biliary drainage was performed. Other therapies, such as laparotomy, chemotherapy, and radiotherapy were not performed because of old age. Fifty-one days after first admission, a peripheral blood examination showed a white cell count of 16,400/µL with 60% blast cells. A bone marrow examination revealed AML (FAB type M0), and he died 1 month after diagnosis. At autopsy, the suspected gallbladder carcinoma turned out to be granulocytic sarcoma associated with AML. The forth case (17
) was of a 75-yr-old woman who presented with severe obstructive jaundice. A bile duct carcinoma arising in the hilum of the liver (a so-called Klatskin tumor) was clinically suspected. A right hepatectomy was performed and revealed a granulocytic sarcoma. Subsequently, a bone marrow biopsy showed acute myelomonocytic leukaemia. The patient died of disease 1 month after surgery.
Summary of biliary granulocytic sarcomas reported
The present case also presented with abdominal pain and jaundice. Likewise to the case reported by Matsueda et al. (16
), the patient had no preceding leukemia. Neiman et al. (5
) reported that among 15 patients of granulocytic sarcoma without leukemia, 13 patients developed AML between 1 and 49 months (mean: 10.5 months) after diagnosis. Most nonleukemic granulocytic sarcomas left untreated will progress to acute nonlymphoblastic leukemia (ANLL), and patients initially administered chemotherapy for ANLL showed longer disease free intervals and overall survival (18
). The present patient did not receive chemotherapy until development of leukemia, which may have caused the early development of acute leukemia.
Granulocytic sarcoma occurs in only 2 to 8% of AML cases, which are mainly of the M2 morphology, although the disease has also been associated with M3, M4, M5, and M7 morphologies (21
). A patient having AML type M2 with t(8;21) showed a greater tendency to develop granulocytic sarcoma than others, as demonstrated by a study which found that 4.5 to 38% of patients with t(8;21)(q22;q22) developed granulocytic sarcoma (22
). The acute leukemia that developed in the current patient was FAB type M2 with chromosomal abnormality affecting chromosome 21(q22).
A diagnosis of biliary granulocytic sarcoma is difficult to make by radiographic examination only, and is virtually impossible in a nonleukemic patient. Biliary granulocytic sarcoma may be misdiagnosed as inflammatory lesion such as cholangitis or cholecystitis or as a malignancy. Biliary granulocytic sarcoma reported previously, and the current case commonly presented with obstructive jaundice with extrinsic bile duct compression. Therefore, biliary granulocytic sarcoma should be included in the differential diagnosis of patients with obstructive jaundice. Early diagnostic intervention with histologic evaluation is important, and may lead to a better prognosis.