Abstract
Primary extramedullary myeloid tumors (PEMMT) are extramedullary proliferations of myeloid cells occurring in the absence of an antecedent myeloproliferative disorder. They have predilection for the skin, lymph nodes, central nervous system, and small intestine. Breast is an uncommon location for PEMMT and only a few cases have been reported so far in the medical literature. Many of these cases have been initially misdiagnosed. We reviewed all the reported cases of PEMMT of the breast in the English-language literature since 1965. In addition, we present a new case of PEMMT of the breast who presented with a mass in her right breast mimicking a breast malignancy and was initially misdiagnosed as non-Hodgkin's lymphoma. A careful histologic examination with immunohistochemical studies revealed the presence of PEMMT of the breast. Treatment with systemic chemotherapy and local radiotherapy rendered a complete remission. Seventeen cases of PEMMT of the breast were reported in the English literature from 1965 to 2003. Most of the cases were misdiagnosed initially as lymphomas, breast carcinomas, or malignant melanomas. PEMMT of the breast is a poorly recognized entity whose diagnosis frequently challenges both the pathologist and the oncologist. Given the small number of patients reported no optimal treatment has been defined, but systemic chemotherapy similar to that given for acute myeloid leukemia with or without local radiotherapy may result in long remissions and avoid the progression to overt acute myeloid leukemia.
Similar content being viewed by others
References
Davey FR, Olson S, Kurec AS, et al. (1988) The immunophenotyping of extramedullary myeloid cell tumors in paraffin-embedded tissue sections. Am J Surg Pathol 12:699–707
Imrie K, Kovacs M, Lipton J, et al. (1993) Localized granulocytic sarcoma: the benefit of early aggressive chemotherapy (abstract 494). Blood 82:127a
Eshghabadi M, Shojania AM, Carr I (1986) Isolated granulocytic sarcoma: report of a case and review of the literature. J Clin Oncol 4:912–917
Byrd JC, Edenfield WJ, Shields DJ, Dawson NA (1995) Extramedullary myeloid cell tumors in acute nonlymphocytic leukemia: a clinical review. J Clin Oncol 13:1800–1816
Ahrar K, McLeary MS, Young LW, et al. (1998) Granulocytic sarcoma (chloroma) of the breast in an adolescent patient: ultrasonographic findings. J Ultrasound Med 17:383–384
Ngu IWY, Sinclair EC, Greenaway S, Greenberg ML (2001) Unusual presentation of granulocytic sarcoma in the breast: a case report and review of the literature. Diagn Cytopathol 24:53–57
Chen J, Yanuck RR 3rd, Abbondanzo SL, Chu WS, Aguilera NS (2001) c-Kit (CD117) reactivity in extramedullary myeloid tumor/granulocytic sarcoma. Arch Pathol Lab Med 125:1448–1452
Breccia M, Petti MC, Fraternali-Orcioni G, et al. (2000) Granulocytic sarcoma with breast and skin presentation: a report of a case successfully treated by local radiation and systemic chemotherapy. Acta Haematol 104:34–37
Joo M, Lee HK, Kang YK, Kim JH (2000) Granulocytic sarcoma of the breast preceding acute myelogenous leukemia: a case report. J Korean Med Sci 15:457–459
Jung SM, Kuo TT, Wu JH, Shih LY (1998) Granulocytic sarcoma presenting as a giant breast tumor in a pregnant woman: a case report. Changgeng Yi Xue Za Zhi 21:97–102
Doval DC, Rao CR, Misra S, et al. (1996) Isolated granulocytic sarcoma of the breast. Ann Oncol 7:424–425
Quitt M, Elmalach I, Dar H, Aghai E (1997) Isolated chloroma of breast preceding acute nonlymphatic leukemia. Leukemia 11:1995–1996
Traweek ST, Arber DA, Rappaport H, Brynes RK (1993) Extramedullary myeloid cell tumors. An immunohistochemical and morphologic study of 28 cases. Am J Surg Pathol 17:1011–1019
Menasce LP, Banerjee SS, Beckett E, Harris M (1999) Extra-medullary myeloid tumor (granulocytic sarcoma) is often misdiagnosed: a study of 26 cases. Histopathology 34:391–398
Meis JM, Butler JJ, Osborne BM, et al. (1986) Granulocytic sarcoma in nonleukemic patients. Cancer 58:2697–2709
Liu K, Stern RC, Rogers RT, et al. (2001) Diagnosis of hematopoietic processes by fine-needle aspiration in conjunction with flow cytometry: a review of 127 cases. Diagn Cytopathol 24:1–10
Lin Y, Govindan R, Hess JL (1997) Malignant hematopoietic breast tumors. Am J Clin Pathol 107:177–186
Warnke RA, Weiss LM, Chan JKC, et al. (1995) Extramedullary leukemia and mastocytosis. In: Warnke RA, Weiss LM, Chan JKC, Cleary ML, Dorfman RF (eds) Tumors of the lymph nodes and spleen. Armed Forces Institute of Pathology, Washington, D.C, pp 385–410
Neiman RS, Barcos M, Berard C, et al. (1981) Granulocytic sarcoma: a clinicopathologic study of 61 biopsied cases. Cancer 48:1426–1437
Roth MJ, Medeiros LJ, Elenitoba-Johnson K, Kuchnio M, Jaffe ES, Stetler-Stevenson M (1995) Extramedullary myeloid cell tumors. An immunohistochemical study of 29 cases using routinely fixed and processed paraffin-embedded tissue sections. Arch Pathol Lab Med 119:790–798
Hutchinson RE, Kurec AS, Davey FR (1990) Granulocytic sarcoma. Clin Lab Med 10:889–901
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Quintas-Cardama, A., Fraga, M., Antunez, J. et al. Primary extramedullary myeloid tumor of the breast: a case report and review of the literature. Ann Hematol 82, 431–434 (2003). https://doi.org/10.1007/s00277-003-0668-3
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00277-003-0668-3