Chin Med J 2008;121(18):1854-1856
Synchronous granulocytic sarcoma of the breast and spine: a case
report and review of the literature
CUI Yan, ZHOU Jin-lian, WU Ji-hua and ZHANG Jian-zhong
Keywords: granulocytic sarcoma; chloroma; breast tumor; thoracic epidural tumor
ranulocytic sarcoma or chloroma is a rare tumour
derived from myeloid cell precursors. It is generally
seen before or after or together with the onset of
myelocytic leukaemia. Immunohistochemical staining of
myeloperoxidase is necessary for a definite diagnosis of
granulocytic sarcoma. Recognition of this entity ensures
an early aggressive chemotherapy that causes regression
of the tumour.1-3 We present an unusual case of a
40-year-old woman who presented with back pain,
numbness in the legs, and fatigue for 2 weeks. She was
diagnosed as having synchronous granulocytic sarcomas
of the breast and spine, confirmed surgically and
histopathologically. After a subsequent systemic
chemotherapy the patient was completely asymptomatic
during a 20-month follow-up. We also reviewed the
literature about the clinical manifestations, diagnosis,
treatment, and prognosis of this condition.
A 40-year-old female patient was admitted to our hospital
in August 2004 because of back pain, numbness in the
legs, and fatigue for 2 weeks. She had been diagnosed
with acute myelocytic leukaemia 10 years ago and had
achieved complete remission after a systemic
chemotherapy and an all-trans retinoic acid induction
therapy. Physical examination on admission revealed a
palpable mass on the right side of the breast that was
approximately 2.5 cm× 2.5 cm in size and not tender.
Hypoaesthesia was present under the ninth thoracic level.
The clonic Achilles test produced negative results; deep
tendon reflexes were hyperactive; and the Babinski test
was positive bilaterally. Magnetic resonance imaging
(MRI) revealed a solid intraspinal lesion that was located
extradurally between the ninth and eleventh thoracic
vertebral levels and measured approximately 4.0 cm × 1.5
cm× 0.8 cm in size (Figure 1). The results of the
peripheral blood smear and biochemical tests were within
the normal limits. The breast mass was removed, then the
spinal tumor. The postoperative period was uneventful.
The mass resected from the breast measured 5.0 cm× 5.0
cm× 2.5 cm. Upon opening the specimen, a round solid
tumor with a diameter of 2.0 cm was observed in the middle;
it was green and had a clear boundary. The tumor excised
from the spine was irregular, 4.0 cm× 2.5 cm× 1.0 cm in size,
and its cut surface appeared to be grey and green.
Figure 1. MRI (sagittal T2WI) revealed a solid intraspinal
lesion (arrow) located between T9–11 extradurally.
Microscopically, the breast mass revealed massively
infiltrated, middle-sized tumor cells with prominent
nucleoli (Figure 2). The tumor cells of the thoracic spine
showed the same features as those of the breast tumor
(Figure 3). Immunohistochemically, the tumor cells were
positive for myeloperoxidase (MPO) (Figure 4). They
were also positive for CD68, CD43, CD99, and lysozyme,
while negative for leukocyte common antigen (LCA),
CD20, CD3, CD5, and CD34.
As confirmed histopathologically,
granulocytic sarcomas (GSs) of the breast and spine were
diagnosed finally. Bone marrow aspirate was analyzed,
and the results revealed a mild hypercellularity but no
evidence of increase in blast count.
After recovery from surgery, the patient was discharged in
September 2004 and was subjected to a systemic
chemotherapy with harringtonine plus cytosine arabinoside
Department of General Surgery (Cui Y), Department of Pathology
(Zhou JL, Wu JH and Zhang JZ), 306 Hospital of People’s
Liberation Army, Beijing 100101, China
Correspondence to: Dr. CUI Yan, Department of General Surgery,
306 Hospital of People’s Liberation Army, Beijing 100101, China
Chinese Medical Journal 2008; 121(18):1854-1856
Figure 2. A specimen of breast mass showing immature myeloid blastic cell infiltration (HE, original magnification ×200).
Figure 3. A specimen of thoracic epidural lesion showing the same features as in the breast mass (HE, original magnification ×200).
Figure 4. Positive staining for myeloperoxidase in granulocytic sarcoma cells of the breast (EnVision, original magnification ×200).
(HA). The patient responded well to the treatment, and
complete remission was achieved. Clinical and laboratory
findings were normal during 20 months of follow-up.
GSs are rare extramedullary tumors that consist of
immature granulocytic cells. The cut surfaces of these
specimens usually appear to be green because of MPO
enzyme within the cells; hence, they are also called
chloromas. The term ‘chloroma’ is derived from the
Greek word chloros (green). This tumor was first
described by Burns in 1811 and originally called
chloroma by King in 1853. Its association with leukemia
was established by Dock in 1893.
GSs occur in 3%–8% of patients with acute myelogenous
leukemia but without sex predilection, and can arise
before, during, or after the onset of acute myeloblastic
leukemia. Rarely, they can also occur as a primary
isolated mass or can be associated with other myeloid
disorders such as chronic myelogenous leukemia and
myeloid metaplasia.1-3 GSs are generally seen in the ribs,
sternum, pelvis, and orbital bones as well as in the soft
tissues, lymph nodes, skin, and gums. There are some
cases of GSs originating from the breast or spine, whereas
those of synchronous GSs in the breast and the thoracic
epidural region are rare.4,5 The present case presented
with back pain, numbness, and weakness of the legs for 2
weeks. Physical examination detected a solid mass in the
right side of the breast. MRI revealed a solid intraspinal
lesion between the ninth and eleventh thoracic vertebral
levels, resulting in thoracic spinal cord compression.
Before surgery, the diagnosis of the lesion was uncertain
even though the patient had had a history of acute
myelocytic leukemia 10 years ago.
Not suspected clinically, GS is misdiagnosed most
frequently as a lymphoma or sarcoma. According to the
World Health Organization classification, the tumor is
histologically classified into 3 types: well differentiated,
poorly differentiated, and blastic.3,6,7 Clinically and
histologically, GS should be distinguished from Hodgkin
lymphoma, Burkitt's lymphoma, large cell lymphoma,
and small round cell tumor, including neuroectodermal
tumor. Hematoxylin-eosin-stained slides show various
morphological changes in the cells of GS. In
well-differentiated tumors, a diffuse infiltration of
granulocytic cells can be seen. In poorly differentiated
tumors, most of the cells form a large and monotonous
population. The nucleoli of the cells may or may not be
prominent. The cytoplasm however is often scanty and a
high mitotic index can be seen. The dissection of collagen
by strings of the neoplastic cells is also a common
Immunohistochemical studies, especially staining of
MPO, are extremely helpful to make a correct diagnosis.
Traweek et al6 suggested that an immunohistochemical
panel including CD20, CD43, CD68, and MPO can
successfully identify 96% of extramedullary myeloid cell
tumors via paraffin sections. In the present case, the
synchronous GSs of the breast and thoracic spine were
diagnosed by positive staining of MPO, CD68, CD43,
CD99, and lysozymes. These molecular analyses provide
a precise method of diagnosis.3,6,8
Optimal therapy for patients with GSs is not yet well
defined. Reports have emphasized the use of combined
local and systemic radical options, including surgical
resection, chemotherapy, radiotherapy, and bone marrow
transplantation. The strategy for the management of GS
should be individualized. The protocols of standard
chemotherapy for acute myelocytic leukemia are
moderately effective in all suitable cases. Radiotherapy
and/or stem cell transplant is also beneficial to the
survival. The early systemic chemotherapy with HA after
surgery is beneficial and may induce complete remission
as in our case.2,4
The prognosis of GS is still controversial. The mean
survival time of patients with GS ranges from 2.5 to 22
months. In patients with leukemia, the discovery of a GS
heralds an imminent downturn in the clinical course. The
onset of GS is almost always followed by bone marrow
relapse after 1–19 months (mean 7 months), and the
outcome is poor. In patients without myeloproliferative
disorders, GS is usually an ominous sign, and most cases
proceed to overt leukemia within 2–4 years. Uncommonly,
however, cases of no progression have also been reported.
Chin Med J 2008;121(18):1854-1856
Because aggressive induction chemotherapy may prolong
the survival of patients or lead to complete remission,
early diagnosis and strict follow-up of these patients are
essential to a better outcome in GS.2,4
Neiman RS, Barcos M, Berard C, Bonner H, Mann R, Rydell
RE, et al. Granulocytic sarcoma: a clinicopathologic study of
61 biopsied cases. Cancer 1981; 48: 1426-1437.
Park HJ, Jeong DH, Song HG, Lee GK, Han GS, Cha SH, et al.
Myeloid sarcoma of both kidneys, the brain, and multiple
bones in a nonleukemic child. Yonsei Med J 2003; 44:
Liu YH, Zhuang HG, Liao XB, Luo XL, Cai XL, Luo DL.
Diagnosis and differential diagnosis of granulocytic sarcomas.
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Shea B, Reddy V, Abbitt P, Benda R, Douglas V, Wingard J.
Granulocytic sarcoma (chloroma) of the breast: a diagnostic
dilemma and review of the literature. Breast J 2004; 10: 48-53.
Kalayci M, Sümer M, Yenidünya S, Ozdolap S, Açikgöz B.
Spinal granulocytic sarcoma (chloroma) presenting as acute
cord compression in a nonleukemic patient. Neurol India 2005;
Traweek ST, Arber DA, Rappaport H, Brynes RK. Extra-
medullary myeloid cell tumors. An immunohistochemical and
morphologic study of 28 cases. Am J Surg Pathol 1993; 17:
Valbuena JR, Admirand JH, Gualco G, Medeiros LJ. Myeloid
sarcoma involving the breast. Arch Pathol Lab Med 2005; 129:
Sekiguchi N, Watanabe T, Kobayashi Y, Inokuchi C, Kim SW,
Yokota Y, et al. The application of molecular analyses for
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(Received January 23, 2008)
Edited by QIAN Shou-chu and HAO Xiu-yuan
In the Medical Progress entitled “Phosphatases of regenerating liver: a novel target in human solid tumors” published in
August 2008, issue 15 of the journal [2008; 121 (15): 1469-1474], the authors’ affiliation should be changed into
“Department of Hematology, Provincial Hospital Affiliated to Shandong University, Jinan, Shandong 250021, China”.