granulocytic sarcoma ( gs ) is a rare solid tumor consisting of immature myeloid cells first described as a chloroma by burns in 1823 because its green color due to myeloperoxidase staining [ 1 , 2 ] . 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 ( cml ) . in rare cases , they appear as an isolated mass without prior evidence of leukemia , but in this situation , patients usually develop aml within 8 days to 28 months [ 46 ] . 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 . 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 estimated at 36.4% followed by the cervix and uterus . the 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 . described in their review of 25 patients with gs of the cervix only two cases of confirmed isolated initial cervical involvement . in both cases 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 . 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 . 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    5  cm . the mass was homogeneous , well defined , infiltrating , but spared the rectum and the bladder wall . 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 110/70  mm  hg . gynecologic examination revealed an enlarged cervix , which was firm , elastic and bruised easily without parametrial or vaginal cul - de - sac involvement . laboratory studies revealed the following values : hemoglobin 13.3  g / dl , platelet 218  10/ml , and white - cell count 7300 per cubic millimeter , with 62.5% neutrophils , 30.2% lymphocytes , and 5.3% monocytes . cytological examination of cervical smears was composed of 50% of hematopoietic cells , small to medium sized blasts , with high nuclears - cytoplasmic ratio , fine chromatin , and fine granulations in the cytoplasm of myeloid origin . a biopsy was performed ( two fragments of 1.3  0.5  0.2 and 1.1  0.7  0.2  cm ) which showed extensive infiltration of the squamous epithelium wall by small - sized blast cells . ( 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 ) . bone marrow biopsy ( bmb ) and aspirate were normal as were cytogenetics .      as most patients with a localized gs evolve into an aml , our patient received aml type induction chemotherapy with cytosine arabinoside ( 200  mg / m/day for seven days ) and idarubicin ( 8  mg / m/day for five days ) . a postchemotherapy examination of the cervix was normal , and a biopsy performed one month after chemotherapy confirmed the absence of residual blast cells . the patient desired subsequent pregnancies in the future and so the following treatment strategy was adopted : one cure of consolidation chemotherapy with high - dose cytosine arabinoside ( 3  g / m  2/day for 4 days ) and idarubicin ( 12  mg / m/day for 2 days ) followed by laparoscopic protective ovariopexy and external beam radiotherapy of 30  gy limited to uterus . this was followed by a second cycle of consolidation chemotherapy with high dose cytosine arabinoside ( 3  g / m  2/day for 4 days ) .     at the last follow - up in december 2008 , more than six years after the diagnosis , she remains in complete remission . 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 : 8183% of presenting symptoms . abdominal pain or discomfort present in 1729% of patients and systemic symptoms in 617% were absent in our patient . finally a combination of other symptoms is responsible for 17% of initial complaints [ 1 , 2 ] . 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 ] . 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 . few reports of complete remission have been described after aggressive multimodality treatment of gs in other sites including disseminated gs without evidence of aml . 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 had an ovariopexy before radiotherapy and recovered her menstrual cycles seven months after treatment . unfortunately she was unable to conceive and even failed several attempts at in vitro fertilization . 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 30  gy appear to be determinant for fertility . nevertheless some cases of pregnancy following uterine irradiation of up to 30  gy have been reported in the literature . granulocytic sarcoma of the cervix is a rare entity and even in 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 .
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 .