Diagnosis and treatment of a patient with isolated spinal granulocytic sarcoma: A case report.
ABSTRACT A previously healthy 34-year-old female presented with a 5-month history of progressive backache and weakness 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 findings. 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.
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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.American journal of blood research. 01/2013; 3(4):265-270.
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ABSTRACT: Introduction. Granulocytic sarcoma (GS), also known as chloroma or extramedullary myeloblastoma, is a solid tumor composed of primitive precursors of the granulocytic series that include myeloblasts, promyelocytes, and myelocytes. Granulocytic sarcoma is a rare tumor that may develop during acute myeloid leukemia (AML) but less frequently may precede its presentation. Although generalized lymph node enlargement is a presentation for malignant lymphoma, it can also rarely be the early presenting sign of GS. Methods. We present a case of GS mimicking lymphoma in a 45-year-old male. The patient presented with bilateral neck masses and had widespread, prominent lymphadenopathy secondary to AML as the first presenting manifestation of GS for the last 4 months with concurrent marrow AML. Result. A clinical diagnosis of lymphoma was suspected; fine needle aspiration cytology findings were also suggestive of lymphoma. However, peripheral blood and bone marrow examination reported as acute myeloid leukemia with monocytic differentiation and histopathology of excised lymph node confirmed it to be a GS not lymphoma. Conclusion. GS is often misdiagnosed as malignant lymphoma because of cytomorphologic and histologic similarities of the blasts to large cell lymphoma. A careful search for immature myeloid is a useful clue to the diagnosis accompanied with appropriate immunophenotyping.Case Reports in Medicine 01/2013; 2013:483291.
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.
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