ONCOLOGY LETTERS 5: 1229-1232, 2013
Abstract. A previously healthy 34-year-old female presented
with a 5-month history of progressive backache and weak-
ness in the left fingers. Magnetic resonance imaging (MRI)
showed soft tissue masses in the spinal canal distributed along
the nerve course. The patient's baseline laboratory data were
normal. Surgical intervention was performed and histological
examination identified isolated spinal granulocytic sarcoma
(GS). A bone marrow biopsy also presented normal find-
ings. However, the patient developed numbness and pain in
the right lower limb two months later. Fluorodeoxyglucose
(FDG)-positron emission tomography (PET) showed FDG
uptake in the left trapezius muscle, cervix uteri, iliac bone,
lymphadenectasis of the pelvic wall and left axillary fossa.
Cerebrospinal fluid (CSF) examination allowed a diagnosis
of central nervous system leukemia (CNSL). The patient
underwent chemotherapy and intrathecal injection, resulting
in the elimination of the residual lesion. Correct diagnosis and
adequate treatment are essential to achieve optimal results in
patients with isolated spinal GS.
Granulocytic sarcoma (GS), also referred to as myeloid
sarcoma or chloroma, is a rare malignant tumor caused by
the extramedullary proliferation of myeloblasts or immature
myeloid cells (1‑3). GS usually occurs concomitantly with or
following the diagnosis of acute myeloid leukemia (AML) (2).
GS may also be a symptom of a myeloproliferative disorder
or leukemic transformation in myelodysplastic syndrome (4).
Isolated GS has occasionally been reported to initially present
in the skin, bone, pancreas, conjunctiva, gastrointestine,
cervix, vagina and mediastinum. However, isolated spinal
GS, particularly with the involvement of the central nervous
system (CNS), is extremely rare.
The present study describes a case of isolated spinal
subdural GS and a further diagnosis of CNS leukemia (CNSL)
which was successfully treated with surgery, intensive chemo-
therapy and intrathecal injection.
A previously healthy 34-year-old female exhibited a 5-month
history of progressive anesthesia and weakness in the left hand
fingers. In March 2012, magnetic resonance imaging (MRI)
showed that the neck and thoracic portions of the spine were
involved. Soft tissue masses were observed in the spinal canal
distributed along the course of the nerve root, at the C6-T1
level (Fig. 1). Blood tests showed a white blood cell count
(WBC) of 6.39x109/l, hemoglobin count of 119 g/l and platelet
count of 200x109/l. The patient immediately underwent
surgical intervention with the resolution of the neurological
symptoms. The pathological evaluation of the vertebral canal
mass showed homogenous malignant infiltration containing
round nuclei, dispersed chromatin and ill‑defined eosinophilic
cytoplasm (Fig. 2A). Immunohistochemical study showed
the vertebral canal mass to be positive for myeloperoxidase
(MPO) (Fig. 2B), partly positive for terminal transferase (TdT)
(Fig. 2C), positive for Ki67 (35%, Fig. 2D) and negative for
CD20, CD79a, CD138, CD15, CD3 and CD5. Bone marrow
aspiration revealed a normal result. Based on these findings,
the final histological diagnosis was isolated GS. The patient
developed numbness and pain in the right lower limb two
months later. Fluorodeoxyglucose (FDG)-positron emission
tomography (PET) showed FDG uptake in the left trapezius
muscle with a maximal standardized uptake value (SUV)
of 2.4. The proliferation of hypermetabolic lesions was also
observed in the cervix uteri, iliac bone, lymphadenectasis of
the pelvic wall and left axillary fossa with maximal SUVs
of 4.2, 3.0, 1.5 and 1.3, respectively (Fig. 3A). Laboratory
studies revealed a hemoglobin level of 113 g/l, platelet level
of 295x109/l and WBC level of 9.06x109/l. A bone marrow
biopsy yielded a normocellular specimen. A cytogenetic
study of the bone marrow cells revealed a normal karyotype.
A lumbar puncture was performed and revealed elevated
opening pressure (>140 mm H2O). Biochemical analysis of
the cerebrospinal fluid (CSF) showed that the CSF WBC was
Diagnosis and treatment of a patient with isolated
spinal granulocytic sarcoma: A case report
RUO‑ZHI XIAO1,2, ZI‑JIE LONG1,2, MU‑JUN XIONG1,2, WEN‑WEN WANG1,2 and DONG‑JUN LIN1,2
1Department of Hematology, Third Affiliated Hospital; 2Sun Yat‑sen Institute of Hematology,
Sun Yat‑sen University, Guangzhou, P.R. China
Received October 15, 2012; Accepted January 29, 2013
Correspondence to: Professor Ruo‑Zhi Xiao, Department of
Hematology, Third Affiliated Hospital, Sun Yat‑Sen University,
600 Tianhe Road, Guangzhou 510630, P.R. China
Key words: isolated spinal granulocytic sarcoma, diagnosis,
chemotherapy, intrathecal injection
XIAO et al: DIAGNOSIS AND TREATMENT OF ISOLATED SPINAL GRANULOCYTIC SARCOMA PATIENT
220x106/l and protein was 1.19 g/l. Cytological examination of
the CSF revealed a predominance of myeloid cells, including
myeloblasts. The final histological diagnosis was CNSL.
Systemic induction chemotherapy was started following
diagnosis and consisted of daunorubicin [90 mg/day intra-
venous (i.v.) on days 1, 2 and 3] and cytarabine (200 mg/day
continuous i.v. on days 1‑7) for 1 course, followed by piraru-
bicin (30 mg on day 1, 30 mg on day 2 and 40 mg on day 3) and
Ara‑C (200 mg/day continuous i.v. on days 1‑7). During the
chemotherapy, the patient also received 6 intrathecal injections
containing 15 mg MTX, 50 mg Ara‑C and 10 mg DXM each
time. At follow‑up 2 months later, the CSF WBC had disap-
peared and protein was 0.24 g/l. Cytological examination of
the CSF did not reveal any clear myeloid tumor cells.
A visual representation of the disease site and metabolic
remission was achieved by FDG‑PET. The maximal SUV of
the FDG uptake in the left trapezius muscle was 1.2, much
lower than pre‑treatment value. The maximal SUV decreased
from 4.2 to 2.1 in the cervix uteri, while FDG uptake disap-
peared in the iliac bone, lymphadenectasis of the left axillary
fossa and pelvic wall (Fig. 3B). Bone marrow examination
revealed a normocellular specimen. At present, a further cycle
of chemotherapy in addition to the first course is being admin-
GS is a localized tumor formed by primitive myeloid cells
at an extramedullary site. GS was first described by Burns
in 1811 and named chloroma in 1853 due to the infrequent
greenish appearance observed as a result of myeloperoxydase
granules in the malignant myeloid cells (5,6). GS may involve
any organ system, including the skin, bone, soft tissues and
lymph nodes. Spinal GS is extremely rare. It has been reported
Figure 1. (A) MRI of the sagittal plane and (B) cross‑section of the patient's spine. T2‑weighted MRI showed a large mass infiltrating the spinal canal (arrows).
MRI, magnetic resonance imaging.
Figure 2. Microscopic analysis of the vertebral canal mass. (A) H&E staining. (B) Positive staining for MPO. (C) Partly positive staining for TdT. (D) Positive
staining for Ki67. Magnification, x200. HE, hematoxylin and eosin; MPO, myeloperoxidase; TdT, terminal transferase.
ONCOLOGY LETTERS 5: 1229-1232, 2013
that the prevalence of GS in the spine is 1.0% among all
patients with myeloid leukemia (7). GS in the absence of clini-
cally detectable leukemia is not common and only a few cases
of GS in patients without leukemia have been observed with
spinal involvement (8,9). Among these, CNS involvement has
been reported in 19% of non‑leukemic GS patients (10).
Pathologically, the variable morphology may be
misleading in GS. The correct diagnosis is sometimes
challenging and is obtained in only ~50% of non‑leukemic
patients due to the histological and radiological similarities
to malignant lymphoma (11). The definitive diagnosis of GS
requires positive immunostaining for at least 1 of the myeloid
associated antigens (CD68, MPO, CD43, CD45, CD117,
CD99, CD33, CD34 and CD13), as well as negative staining
for the lymphoid lineages CD3 and CD20 (2,12). Bone
marrow sampling is also necessary for the diagnosis of GS
to assess the absence of AML. In the present case, immuno-
histochemical studies showed positivity for MPO and Ki67
and partly positive results for TdT, but negative results for
CD20, CD79a, CD138, CD15, CD5 and CD3, indicating GS.
The immunohistochemical findings were compatible with a
monoblastic or myelomonoblastic variant of myeloid sarcoma.
In addition, bone marrow aspiration showed a normal result,
indicating no involvement of the bone marrow.
An early and precise diagnosis of spinal GS with MRI
evaluation facilitates appropriate treatment with further
therapy (7). However, MRI is unable to evaluate the metabo-
lism. FDG‑PET is reported to be more sensitive for the
detection of malignant tumors with increased glucose metabo-
lism (13). In the present case, FDG‑PET was used to estimate
the malignancy of the tumor and the treatment efficacy. It was
observed that FDG‑PET successfully identified the active
lesion and demonstrated the malignancy. A decrease in FDG
uptake was observed 2 months after treatment. The follow‑up
FDG‑PET suggested that adequate treatment contributed to
the reduction in the cellularity of the tumor.
The prognosis of patients with GS depends on the initial
context in which it occurs. Out of all isolated GS patients,
66‑88% develop AML within 9‑11 months of diagnosis (3,14).
In the present case, the patient developed CNSL 2 months
after the diagnosis of GS. The optimal treatment for GS has
not been fully established, partially due to the variety of
Figure 3. (A) FDG‑PET showed hypermetabolic lesions (arrows) in the (a) left trapezius muscle, (b) cervix uteri, (c) lymphadenectasis of the left axillary
fossa and (d) pelvic wall. (B) FDG‑PET showed a decrease in FDG uptake following chemotherapy (a‑d). FDG, fluorodeoxyglucose; PET, positron emission
XIAO et al: DIAGNOSIS AND TREATMENT OF ISOLATED SPINAL GRANULOCYTIC SARCOMA PATIENT
clinical presentations. Chemotherapy, radiation therapy, bone
marrow transplantation, surgical resection or a combination of
approaches are employed in various cases. Surgery is gener-
ally reserved for patients with acute spinal cord compression
or neurological symptoms. However, surgery is not always
required and may worsen the prognosis due to the delayed
administration of induction chemotherapy. Treating GS
in the same manner as AML, even in the absence of clini-
cally detectable leukemia has been previously reported (8).
Combination treatment with radiotherapy and chemotherapy
resulted in improved survival (3,10). However, isolated CNS
GS and meningeal myeloid leukemia may be successfully
treated without radiotherapy (16).
In accordance with the previously mentioned studies, the
present patient was successfully treated using surgery and
intensive anti-leukemic chemotherapy accompanied by intra-
thecal injections. The present case highlighted the importance
of a correct diagnosis. Pre‑therapeutic examinations should be
the basis for the diagnosis of a mass with an atypical clinical
presentation. Notably, treating GS in the same manner as
AML may benefit patients with isolated spinal GS.
1. Balleari E, Panarello S, Capello E, et al: Granulocytic sarcoma:
an unusual cause of spinal cord compression. Int J Clin Oncol
12: 234‑237, 2007.
2. Swerlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H,
Thiele J and Vardiman JW (eds): WHO Classification of Tumours
of Haematopoietic and Lymphoid Tissues. 4th edition. IARC
press, Lyon, France, 2008.
3. Neiman RS, Barcos M, Berard C, et al: Granulocytic sarcoma: a
clinicopathologic study of 61 biopsy cases. Cancer 48: 1426‑1437,
4. Byrd JC, Edenfield WJ, Shields DJ and Dawson NA:
Extramedullary myeloid cell tumors in acute non lymphocytic
leukemia: a clinical review. J Clin Oncol 13: 1800‑1816, 1995.
5. Burns A: Observations on the Surgical Anatomy of the Head
and Neck. 2nd edition. Wardlaw and Cunninghame, Glasgow,
Scotland, pp386‑387, 1824.
6. King A: A case of chloroma. Monthly J Med 17: 97, 1853.
7. Seok JH, Park J, Kim SK, Choi JE and Kim CC: Granulocytic
sarcoma of the spine: MRI and clinical review. AJR Am J
Roentgenol 194: 485‑489, 2010.
8. Antic D, Verstovsek S, Elezovic I, et al: Spinal epidural granulo cytic
sarcoma in non‑leukemic patient. Int J Hematol 89: 95‑97, 2009.
9. Serefhanoglu S, Goker H, Aksu S, et al: Spinal myeloid sarcoma
in two non‑leukemic patients. Intern Med 49: 2493‑2497, 2010.
10. Tsimberidou AM, Kantarjian HM, Estey E, et al: Outcome in
patients with nonleukemic granulocytic sarcoma treated with
chemotherapy with or without radiotherapy. Leukemia 17:
11. Williams MP, Olliff JF and Rowley MR: CT and MR findings in
parameningeal leukaemic masses. J Comput Assist Tomogr 14:
12. Audouin J, Comperat E, Le Tourneau A, et al: Myeloid sarcoma:
clinical and morphologic criteria useful for diagnosis. Int J Surg
Pathol 11: 271‑282, 2003.
13. Go KG, Pruim J, Que TH, Vaalburg W and Haaxma‑Reiche H:
Evaluation of dissemination studies with FDG whole‑body
positron emission tomography in patients with suspected meta-
static tumours of brain and spine. Acta Neurochir (Wien) 142:
14. Imrie KR, Kovacs MJ, Selby D, et al: Isolated chloroma: the
effect of early antileukemic therapy. Ann Intern Med 123:
15. Lee JM, Song HN, Kang Y, et al: Isolated mediastinal myeloid
sarcoma successfully treated with chemoradiotherapy followed
by unrelated allogeneic stem cell transplantation. Intern Med 50:
16. Stepensky P, Revel‑Vilk S, Yehuda‑Gafni O, Mali B, Resnick IB
and Weintraub M: Isolated central nervous system granulocytic
sarcoma and meningeal myeloid leukemia: successful treatment
without radiotherapy. Isr Med Assoc J 11: 569‑570, 2009.