Successful treatment of a granulocytic sarcoma of the uterine cervix in complete remission at six-year follow-up.
ABSTRACT Background. Localized granulocytic sarcoma of the uterine cervix in the absence of acute myelogenous leukemia (AML) at presentation is very rare, its diagnosis is often delayed, and its prognosis almost always ominous evolving into refractory AML. Case. We present the case of a 30-year-old woman with vaginal bleeding and a large cervical mass. Further evaluation confirmed the presence of a granulocytic sarcoma but failed to reveal systemic involvement. Results. AML type chemotherapy followed by radiotherapy of the uterus led to a durable complete remission. She remains in complete remission six years after diagnosis. Conclusion. Granulocytic sarcoma of the cervix is a rare entity for which early intensive AML type therapy is effective.
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ABSTRACT: Granulocytic sarcoma (GS) is a rare manifestation of leukemia and has been reported in 3-5% of acute myelogenous leukemia (AML) patients. GS in the uterine cervix is very rare and is also called chloroma because of its greenish appearance. We present the case of a patient whose disease relapsed as chloroma of the uterine cervix after bone marrow transplantation (BMT). She remained in continuous complete remission for 2 years after allogeneic BMT. However, she visited the hospital because of painless vaginal bleeding. She was diagnosed as having chloroma by cervical smear and colposcopically directed biopsy of the cervix. Systemic chemotherapy was administered on the presumption that myelogenous leukemia had recurred as chloroma, and a good clinical response was achieved. We describe the first case of AML that relapsed as chloroma of the uterine cervix after complete remission of the AML, which had complete response to only systemic chemotherapy.International Journal of Gynecological Cancer 14(3):553-7. · 1.65 Impact Factor
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ABSTRACT: Extramedullary relapse of acute myeloid leukaemia may occur in sites such as the central nervous system, testes, and skin. Presentations in the female genital tract are uncommon and usually asymptomatic. In contrast, symptomatic uterine myeloid sarcoma is very rare. Treatment of this is generally unsuccessful, but is improved when systemic therapies are used. We study a case of a uterine relapse of acute myeloid leukaemia presenting as vaginal bleeding and successfully managed by local irradiation. The mechanism of preferential infiltration of uterine tissue requires further study.Internal Medicine Journal 11/2006; 36(10):669-71. · 1.54 Impact Factor
Article: Isolated extramedullary relapse of acute myelogenous leukemia as a uterine granulocytic sarcoma in an allogeneic hematopoietic stem cell transplantation recipient.[show abstract] [hide abstract]
ABSTRACT: We report an unusual case of acute myelogenous leukemia in a patient who showed an extramedullary relapse in her uterus, without bone marrow recurrence, two years after an allogeneic bone marrow transplant. She complained of irregular vaginal spotting, and magnetic resonance imaging demonstrated a uterine mass. A biopsy revealed a massive infiltration of immature myeloid cells. A variable number of tandem repeats (VNTR) based on an examination of peripheral blood cells showed full donor chimerism. After receiving chemotherapy, her uterine mass had completely resolved. She has remained in complete remission for more than 6 months. This case suggests that physicians should be aware of the possibility of a uterine relapse in female bone marrow transplant recipients with acute myelogenous leukemia.Yonsei Medical Journal 05/2004; 45(2):330-3. · 1.14 Impact Factor
Hindawi Publishing Corporation
Journal of Oncology
Volume 2010, Article ID 812424, 3 pages
SuccessfulTreatmentof a GranulocyticSarcoma of theUterine
CervixinCompleteRemissionat Six-Year Follow-Up
1Department of Hematology and Oncology, Centre des Hˆ opitaux Universitaires de Strasbourg, BP 67000, Strasbourg, France
2Department of Pathology, Centre des Hˆ opitaux Universitaires de Strasbourg, Hautepierre 67200, Strasbourg, France
Correspondence should be addressed to Stefano C. H. Kim, firstname.lastname@example.org
Received 10 October 2009; Accepted 23 February 2010
Academic Editor: Janet S. Rader
Copyright © 2010 Stefano C. H. Kim et al. This is an open access article distributed under the Creative Commons Attribution
License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly
Background. Localized granulocytic sarcoma of the uterine cervix in the absence of acute myelogenous leukemia (AML) at
presentation is very rare, its diagnosis is often delayed, and its prognosis almost always ominous evolving into refractory AML.
Case. We present the case of a 30-year-old woman with vaginal bleeding and a large cervical mass. Further evaluation confirmed
the presence of a granulocytic sarcoma but failed to reveal systemic involvement. Results. AML type chemotherapy followed by
radiotherapy of the uterus led to a durable complete remission. She remains in complete remission six years after diagnosis.
Conclusion. Granulocytic sarcoma of the cervix is a rare entity for which early intensive AML type therapy is effective.
Granulocytic sarcoma (GS) is a rare solid tumor consisting
of immature myeloid cells first described as a chloroma by
staining [1, 2]. Its association with acute leukemia was
reported by Dock in 1893 . GS is most commonly
diagnosed as part of the systemic manifestations of acute
myelogenous leukemia (AML), predominantly after the
diagnosis of AML, but it may also be the presenting
symptom. Less commonly, they signal an AML relapse
or blast transformation of chronic myelogenous leukemia
prior evidence of leukemia, but in this situation, patients
usually develop AML within 8 days to 28 months [4–6].
The incidence of GS is estimated at 0.7 per million in
children and 2 per million in adults . It only occurs
in 32.3% of patients with granulocytic leukemia, being
clinically evident in less than 1% .
It most commonly occurs in bones, the periosteum,
soft tissues, lymph nodes, and skin but can occur virtually
anywhere.The most commonlyinvolved visceralorgan is the
GS has a poor prognosis. The overall 2-year survival rate
for all patients is 6%. No patient surviving more than 5 years
has been reported in the literature .
Involvement of the female genital tract in women dying
of leukemia is frequent but clinically significant involvement
is rare. The most commonly involved organ is the ovary
presenting symptoms of GS of the cervix or uterus include
vaginal or postcoital bleeding followed by abdominal pain
and systemic manifestations including fever, night sweats,
and weight loss .
Only a few cases have been reported in which localized
GS of the cervix was the only manifestation. Pathak et al.
described in their review of 25 patients with GS of the
cervix only two cases of confirmed isolated initial cervical
involvement. In both cases an AML was subsequently
diagnosed. One patient died of the disease, and the other
was in remission after acute leukemia chemotherapy in less
than 2 years of follow-up .
We present here a 30-year-old female with isolated
granulocytic sarcoma of the cervix without evolution into
AML and in complete remission after 6 years of follow-up.
2 Journal of Oncology
A 30-year-old, (gravida 1, para 1) white female visited her
gynecologist with complaints of irregular vaginal bleeding.
Four months earlier, she had had an episode of abnormal
vaginal bleeding which lasted two weeks. There was no
history of abdominal pain or systemic symptoms. Her last
PAP smear, one year prior to diagnosis, was normal. A pelvic
examination revealed hypertrophy of the cervix, and cervical
biopsy showed an infiltration of small, immature myeloid
cell. Laboratory studies were unremarkable. A pelvic CT
scan and MRI revealed a mass at the level of the cervix
measuring 8 × 5cm. The mass was homogeneous, well
defined, infiltrating, but spared the rectum and the bladder
wall. No lymph node enlargement was seen. Ovaries, liver,
spleen, pancreas, and kidneys were normal in appearance.
There was no ascites and no thoracic involvement. The
patient was subsequently referred to our department.
She was a social worker with a 2-year-old daughter who
had been delivered by cesarean section. The rest of the
patient’s medical history was unremarkable.
On examination, she was in perfect physical condition,
no history of weight loss, fever, or night sweats, and the
blood pressure was 110/70mmHg. Gynecologic examina-
tion revealed an enlarged cervix, which was firm, elastic
and bruised easily without parametrial or vaginal cul-de-sac
involvement. There were no palpable lymph nodes and no
hepatosplenomegaly. The rest of the physical examination
was normal. Laboratory studies revealed the following
values: hemoglobin 13.3g/dL, platelet 218 × 103/mL, and
white-cell count 7300 per cubic millimeter, with 62.5% neu-
trophils, 30.2% lymphocytes, and 5.3% monocytes. Blood
chemistry was unremarkable. Cytological examination of
cervical smears was composed of 50% of hematopoietic cells,
ratio, fine chromatin, and fine granulations in the cytoplasm
of myeloid origin. The blasts were myeloperoxidase positive.
A biopsy was performed (two fragments of 1.3 × 0.5 × 0.2
and 1.1 × 0.7 × 0.2cm) which showed extensive infiltration
of the squamous epithelium wall by small-sized blast cells.
Multiple immunohistochemical analyses were obtained.
Myeloperoxidase, Common leucocytic antigen, and CD34
were strongly positive. (Figures 1 and 2) Lymphoid markers,
epithelial markers, and neuroendocrine markers were all
negative (CD20, CD3, CD23, CD5, CD10, CD68, CD45
RO, Kappa, Lambda, cytokeratin, EMA, synaptophysin, and
chromogranin). The Ki67 was expressed in 80% of the cells.
These findings were compatible with the diagnosis of a GS
of the myeloblastic type. Bone marrow biopsy (BMB) and
aspirate were normal as were cytogenetics.
As most patients with a localized GS evolve into an AML,
cytosine arabinoside (200mg/m2/day for seven days) and
idarubicin (8mg/m2/day for five days). A postchemother-
apy examination of the cervix was normal, and a biopsy
performed one month after chemotherapy confirmed the
pregnancies in the future and so the following treatment
strategy was adopted: one cure of consolidation chemother-
Figure 1: Hematoxylin and eosin stain of granulocytic sarcoma of
the uterine cervix.
Figure 2: Myeloperoxidase stain of granulocytic sarcoma of the
apy with high-dose cytosine arabinoside (3g/m2× 2/day
for 4 days) and idarubicin (12mg/m2/day for 2 days)
followed by laparoscopic protective ovariopexy and external
beam radiotherapy of 30Gy limited to uterus. This was
followed by a second cycle of consolidation chemotherapy
with high dose cytosine arabinoside (3g/m2× 2/day for 4
At the last follow-up in December 2008, more than
six years after the diagnosis, she remains in complete
remission. Complete blood counts, abdominal and pelvic
ultrasonography, and physical examination werenormal.
The mean age at presentation of cervical granulocytic
sarcoma is 47 years, range from 26 to 75 of age . The two
patients with isolated GS of the uterine cervix at diagnosis
described by Pathak et al. were 32 and 34 years of age, similar
to our patient.
The presenting feature in our case was vaginal bleeding
as with the majority of patients: 81–83% of presenting
Journal of Oncology3
symptoms. Abdominal pain or discomfort present in 17–
29% of patients and systemic symptoms in 6–17% were
absent in our patient. Finally a combination of other
symptoms is responsible for 17% of initial complaints
The diagnosis is not always easy when GS appears at
an extramedullary site in a nonleukemic patient. Most of
them are poorly differentiated, and only in 44% of cases
the correct diagnosis is made or suspected [9, 10]. The
most common misdiagnosis is the high grade non-Hodgkin
lymphoma. Both are composed of diffusely infiltrating,
discohesive cells that tend to spare normal structures, and
which may contain scattered lymphocytes. In GS, however,
the nuclei are typically slightly smaller with more finely
dispersed chromatin, and some cell may show recognizable
myeloid differentiation. The immunohistochemical stains
are usually diagnostic .
GS of the cervix has a poor prognosis. Treatment is
of GS in other sites including disseminated GS without
evidence of AML .
Our patient was treated with AML type chemotherapy
regimen and localized uterine radiotherapy. A complete
response was seen soon after treatment, and she remains in
complete remission. It appears appropriate to treat GS with
AML type chemotherapy protocols even in the absence of
systemic manifestations, since acute myeloid leukemia will
almost always be present . To our knowledge this is the
first case of an isolated GS of the uterine cervix without
progression into AML.
In the hope of maintaining fertility, she was treated
with an LHRH analogue during chemotherapy. She had an
ovariopexy before radiotherapy and recovered her menstrual
cycles seven months after treatment. Unfortunately she was
Our patient received a total radiation dose of 32.4Gy.
The irradiated uterus suffers somatic damage; it causes
endometrial and myometrial atrophy, scar fibrosis, and
hypovascularization which interfere with implantation .
Independently of other factors, irradiation doses of greater
than 30Gy appear to be determinant for fertility. Never-
theless some cases of pregnancy following uterine irradi-
ation of up to 30Gy have been reported in the literature
its localized form, early intensive AML type therapy appears
to be appropriate. Even though our patient has had a long
complete remission, the role of radiotherapy in addition to
chemotherapy remains unclear.
Conflict of Interest
The authors declare that there are no conflicts of interest.
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