Am J Blood Res. 2013 Dec 18;3(4):265-70.
Granulocytic sarcoma: a systematic review.
American journal of blood research
Asu Fergun Yilmaz, Guray Saydam, Fahri Sahin, Yusuf Baran
Affiliations
Affiliations
- Department of Hematology, Medical School, Ege University Izmir, Turkey.
- Department of Molecular Biology and Genetics, Science Faculty, Izmir Institute of Technology Urla, Izmir, Turkey.
PMID: 24396704
PMCID: PMC3875275
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.
Keywords: Granulocytic sarcoma; chemotherapy; leukemia; treatment
References
- Radiology. 1994 Mar;190(3):698-702 - PubMed
- Leukemia. 2007 Feb;21(2):340-50 - PubMed
- Neurol Med Chir (Tokyo). 2009 May;49(5):221-4 - PubMed
- Ther Adv Hematol. 2011 Oct;2(5):309-16 - PubMed
- Ann Hematol. 1997 Nov-Dec;75(5-6):239-41 - PubMed
- Cancer. 2008 Sep 15;113(6):1370-8 - PubMed
- Cancer. 1986 Dec 15;58(12):2697-709 - PubMed
- Biomed Pharmacother. 2013 Feb;67(1):72-7 - PubMed
- Acta Haematol. 2009;122(4):238-46 - PubMed
- Bone Marrow Transplant. 1996 May;17(5):801-8 - PubMed
- Leuk Lymphoma. 2000 Oct;39(3-4):427-33 - PubMed
- Am J Clin Pathol. 2009 Sep;132(3):426-37 - PubMed
- Clin Nucl Med. 2011 May;36(5):365-6 - PubMed
- Oncol Lett. 2013 Apr;5(4):1229-1232 - PubMed
- Med Pediatr Oncol. 1998 Sep;31(3):144-9 - PubMed
- Diagn Cytopathol. 2001 Jan;24(1):53-7 - PubMed
- Leukemia. 2003 Jun;17(6):1100-3 - PubMed
- J Urol. 1994 Dec;152(6 Pt 1):2092-3 - PubMed
- Leuk Lymphoma. 2002 Mar;43(3):565-74 - PubMed
- J Clin Oncol. 2008 Oct 20;26(30):4940-3 - PubMed
- Blood. 2010 Jan 21;115(3):453-74 - PubMed
- Haematologica. 2011 Oct;96(10):1552-6 - PubMed
- Hematol Oncol. 2012 Mar;30(1):34-40 - PubMed
- Blood. 2011 Oct 6;118(14):3785-93 - PubMed
- Cancer. 2002 Mar 15;94(6):1739-46 - PubMed
- J Pediatr Hematol Oncol. 2003 Oct;25(10):760-8 - PubMed
- Ann Intern Med. 1995 Sep 1;123(5):351-3 - PubMed
- Yonsei Med J. 2004 Apr 30;45(2):330-3 - PubMed
- Cancer. 1981 Sep 15;48(6):1426-37 - PubMed
- Haematologica. 2011 Sep;96(9):1391-4 - PubMed
- J Low Genit Tract Dis. 2010 Apr;14(2):136-41 - PubMed
- Arch Pathol Lab Med. 1997 May;121(5):520-3 - PubMed
- Eur J Haematol. 2002 May;68(5):322 - PubMed
- Eur J Gynaecol Oncol. 2010;31(6):697-700 - PubMed
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