Case Report
Granulocytic sarcomas (chloromas) are rare extramedullary tumors consisting of primitive granulocytic cells. They arise de novo, or are associated with other hematologic disorders such as acute myeloid leukemia, myelodysplastic syndrome, or myeloproliferative disorders. We report here on a case of a 62-year-old woman who presented with a large swelling in her right groin and leg. The mass was confirmed by biopsy to be a granulocytic sarcoma. Bone marrow examination showed mild hypercellularity but no evidence of increase in blast count. However, cytogenetic examination of the marrow showed t(9;22), indicating an unexpected diagnosis of chronic myeloid leukemia.
A 62-year-old female patient presented with a history of stiffness in both legs for 1 year, plus pain in the right groin for 3 months. On examination, a large mass was palpable in the right groin, and the right leg was swollen throughout. Blood count results were: Hb 10.1, WBC 10.4, plat 331, neut 8.2. A computed tomography (CT) scan of the pelvis showed a large soft tissue mass involving the anterior aspect of the acetabulum and hip joint on the right. It extended into the inguinal region and into the thigh with invasion of the right abdominal muscles. There was also invasion into the perineum, plus symphysis pubis and rectus sheath. The largest diameter is 12.5 x 10.3 cm.
Anterior abdominal mass at the level of the bladder.
Anterior abdominal mass at the level of the bladder.
Anterior abdominal mass at the level of the bladder.
Anterior abdominal mass at the level of the bladder.
Anterior abdominal mass at the level of the bladder.
Mass at the level of the femoral heads.
Mass at the level of the femoral heads.
Mass at the level of the femoral heads.
Mass at the level of the femoral heads.
Mass at the level of the femoral heads.
Central/right pelvic mass, crossing the midline, with involvement of the pelvic bones.
Central/right pelvic mass, crossing the midline, with involvement of the pelvic bones.
Central/right pelvic mass, crossing the midline, with involvement of the pelvic bones.
Central/right pelvic mass, crossing the midline, with involvement of the pelvic bones.
Central/right pelvic mass, crossing the midline, with involvement of the pelvic bones.
Upper right thigh.
Upper right thigh.
Upper right thigh.
Upper right thigh.
Upper right thigh.
Mass extending further down the thigh.
Mass extending further down the thigh.
Mass extending further down the thigh.
Mass extending further down the thigh.
Mass extending further down the thigh.
Mid right thigh region.
Mid right thigh region.
Mid right thigh region.
Mid right thigh region.
Mid right thigh region.
The diagnosis at this stage was thought to be a non-hematologic malignancy, most likely an ovarian tumor, sarcoma, or a metastatic lesion.
Further investigations were performed to establish the diagnosis. These included:
A biopsy was performed under CT guidance. The histology showed a tumor with nests and sheet of regular large cells (Figure 2). Immunohistochemistry was positive for CD 33, 34, 43, 45, and 68. Ki67 was 80%. This was consistent with a myeloid malignancy - an extramedullary myeloid tumor.
Tru-cut biopsy histology of the tumor.
Bone marrow aspirate was performed to look for any evidence of an increased myeloblast population. Results showed only mild myeloid and eosinophilic hyperplasia (Figure 3). There was no evidence of acute myeloid leukemia. (BM immunophenotyping - CD34 blasts < 1%).
Bone marrow aspirate morphology.
However, bone marrow cytogenetics subsequently demonstrated an unsuspected abnormality, t(9;22) (q34;q11) in 18/20 cells (Figure 4). This is the Philadelphia chromosome translocation associated with chronic myeloid leukemia (CML).
Cytogenetics karyotype.
Fluorescent in-situ hybridization (FISH) investigation was performed on the marrow sample (Figure 5). This involves utilizing specific fluorescently labeled DNA probes, and adding them to the denatured DNA from the patient"s sample. The probe hybridizes with the sample DNA and the probe signal can be seen through a fluorescent microscope. The marrow sample test used the Bcr-Abl dual color probe and confirmed the Bcr-Abl translocation fusion product in 92% of cells.
Fluorescent in-situ hybridization.
The final diagnosis was confirmed as bone marrow CML, with the presenting feature of the disease as unifocal extramedullary granulocytic sarcoma transformation. The blood and marrow met the criteria for chronic phase CML (ie, blasts < 10%, peripheral blood basophils < 20%, normal platelet count, no evidence of cytogenetic clonal evolution). However, the extramedullary myeloblast proliferation classifies the CML as blastic phase.
© 2005 Medscape
Cite this: Case Report: A Large Extramedullary Granulocytic Sarcoma as the Initial Presenting Feature of Chronic Myeloid Leukemia - Medscape - Oct 25, 2005.
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