Am J Blood Res 2013;3(4):265-270
Review Article
Granulocytic sarcoma: a systematic review
Asu Fergun Yilmaz, Guray Saydam, Fahri Sahin, Yusuf Baran
Department of Hematology, Medical School, Ege University, Izmir, Turkey; Department of Molecular Biology and Genetics,
Science Faculty, Izmir Institute of Technology, Urla, Izmir, Turkey
Received September 21, 2013; Accepted November 8, 2013; Epub December 18, 2013; Published December 30, 2013
Abstract: Granulocytic sarcoma also called myeloid sarcoma is an extramedullary tumor of immature granulocytic cells. It is a
rare entity, and mostly accompanied by acute myeloid leukemia. It is observed during the course of myeloproliferative
disorders especially in chronic myeloid leukemia and myelodysplastic syndromes. In some rare circumstances, it is detected
before clinical signs of leukemia or other diseases. When the bone marrow biopsy reveals no other hematologic
malignancies, the granulocytic sarcoma is described as nonleukemic, primary or isolated. It is observed at any part of the
body but the most common locations are soft tissues, bone, peritoneum and lymph nodes. Presenting signs or symptoms
are mainly due to mass effect of the tumor and dysfunction of the organ, or the tissue that is affected. The diagnosis is
performed by biopsy of the tumor. The tumor consists of immature granulocytic cells, which could be documented by H&E,
immunohistochemistry, and flow cytometric methods. Fluorescence in-situ hybridization and molecular analysis are also
performed. The optimal time and type of treatment is not clear. Surgery could be an option especially for tumors, which cause
organ dysfunction and/or obstruction. Systemic treatment should be considered in all patients because without systemic
treatment, relapses and progression to acute myeloid leukemia is the ultimate fate of the disease in many cases.
Cytarabine-containing remission-induction chemotherapies have been the most applied therapeutic strategies, but it is not
clear whether the consolidation therapies are required or not, and what kind of regimens are appropriate. The role of
hematopoietic stem cell transplantation (HSC) as a consolidation regimen is not clear, but, after the relapse of the disease
with or without bone marrow involvement, HSC transplantation should be considered in suitable patients after the reinduction
performed by AML chemotherapies. There is only limited data about the role of radiotherapy in these patients. It could be used
in patients with relapsed disease, organ dysfunction which should be quickly relieved and inadequate response to
chemotherapy. The effect of radiotherapy on overall survival is not known. New prospective studies and clinical trials are
needed to generate guidelines for the treatment of primary granulocytic sarcomas. (AJBR1309001).
Keywords: Granulocytic sarcoma, treatment, chemotherapy, leukemia
Address correspondence to: Dr. Yusuf Baran, Department of Molecular Biology and Genetics, Science Faculty, Izmir Institute
of Technology, Urla, 35430, Izmir, Turkey. Tel: +90 232 7507515; Fax: +90 232 7507509; E-mail: yusufbaran@iyte.edu.tr; Dr.
Fahri Sahin, Department of Hematology, Medical School, Ege University, 35100, Bornova, Izmir, Turkey. Tel: +90 232 3903530;
Fax: +90 232 3903530; E-mail: fahri.sahin@ege.edu.tr
Review Article
Granulocytic sarcoma: a systematic review
Asu Fergun Yilmaz, Guray Saydam, Fahri Sahin, Yusuf Baran
Department of Hematology, Medical School, Ege University, Izmir, Turkey; Department of Molecular Biology and Genetics,
Science Faculty, Izmir Institute of Technology, Urla, Izmir, Turkey
Received September 21, 2013; Accepted November 8, 2013; Epub December 18, 2013; Published December 30, 2013
Abstract: Granulocytic sarcoma also called myeloid sarcoma is an extramedullary tumor of immature granulocytic cells. It is a
rare entity, and mostly accompanied by acute myeloid leukemia. It is observed during the course of myeloproliferative
disorders especially in chronic myeloid leukemia and myelodysplastic syndromes. In some rare circumstances, it is detected
before clinical signs of leukemia or other diseases. When the bone marrow biopsy reveals no other hematologic
malignancies, the granulocytic sarcoma is described as nonleukemic, primary or isolated. It is observed at any part of the
body but the most common locations are soft tissues, bone, peritoneum and lymph nodes. Presenting signs or symptoms
are mainly due to mass effect of the tumor and dysfunction of the organ, or the tissue that is affected. The diagnosis is
performed by biopsy of the tumor. The tumor consists of immature granulocytic cells, which could be documented by H&E,
immunohistochemistry, and flow cytometric methods. Fluorescence in-situ hybridization and molecular analysis are also
performed. The optimal time and type of treatment is not clear. Surgery could be an option especially for tumors, which cause
organ dysfunction and/or obstruction. Systemic treatment should be considered in all patients because without systemic
treatment, relapses and progression to acute myeloid leukemia is the ultimate fate of the disease in many cases.
Cytarabine-containing remission-induction chemotherapies have been the most applied therapeutic strategies, but it is not
clear whether the consolidation therapies are required or not, and what kind of regimens are appropriate. The role of
hematopoietic stem cell transplantation (HSC) as a consolidation regimen is not clear, but, after the relapse of the disease
with or without bone marrow involvement, HSC transplantation should be considered in suitable patients after the reinduction
performed by AML chemotherapies. There is only limited data about the role of radiotherapy in these patients. It could be used
in patients with relapsed disease, organ dysfunction which should be quickly relieved and inadequate response to
chemotherapy. The effect of radiotherapy on overall survival is not known. New prospective studies and clinical trials are
needed to generate guidelines for the treatment of primary granulocytic sarcomas. (AJBR1309001).
Keywords: Granulocytic sarcoma, treatment, chemotherapy, leukemia
Address correspondence to: Dr. Yusuf Baran, Department of Molecular Biology and Genetics, Science Faculty, Izmir Institute
of Technology, Urla, 35430, Izmir, Turkey. Tel: +90 232 7507515; Fax: +90 232 7507509; E-mail: yusufbaran@iyte.edu.tr; Dr.
Fahri Sahin, Department of Hematology, Medical School, Ege University, 35100, Bornova, Izmir, Turkey. Tel: +90 232 3903530;
Fax: +90 232 3903530; E-mail: fahri.sahin@ege.edu.tr
| AJBR Copyright © 2011-present, All rights reserved. Published by e-Century Publishing Corporation, Madison, WI 53711, USA |
 |  |  |  |  |  |  |