A Case Report of Recurrent Endometrial Stromal Sarcoma to the Breast, Lung and Pelvis after 15 years of Subtotal Hysterectomy

Methods: This is a rare case report of A 45-year-old woman with (LG-ESS), patients’ history was collected by the authors ourselves through a structured interview, data was obtained from the patient’s file including radiological pictures, pathological reports and drug doses. using PubMed data base for discussion this case. This woman with a history of hysteracomy and bilateral salpingooopharectomy 15 year earlier was admitted to Al Basheer hospital in 2015, presented with left breast mass, biopsy revealed (LGESS). CT scan have shown multiple breast and lung nodules. Doctor prescribed tamoxifen tab 20 mg once daily. After three months of follow up, the size of the metastatic nodules was increased, new umbilical mass and left hemipelvic mass were discovered. Then, megestrol acetate oral suspension had been prescribed 160mg daily. After 6 months of follow up the patient shown good response to treatment; the size of metastatic lungs and breast nodules were decreased, Umbilical and left hemipelvic lesions were decrease, but new small focal liver lesion was seen.

in the age group of 40-50 years [1]. Histologically; ESS divided into low-grade and high-grade tumors. Low-grade endometrial stromal sarcoma (EES) is a rare tumor with a high recurrence rate but a favorable prognosis [2]. Relating to the treatment of ESS; Based on the literature review, it shows that low-grade ESS arising in the vagina can be treated by surgical resection effectively with close observation for recurrence or metastatic [3]. In addition, responses to adjuvant hormonal treatment of these recurrences and metastasis have been published in many case reports and series. We reporting a rare case of an endometrial stromal sarcoma metastasizing to the breast, liver, pelvis and lung after a 15-yearperiod in a 45 years old women.

Case Study
A 45 years old female as a case of metastatic breast sarcoma at 2015, have two girls and one boy, she aborted one time. Since about 17 years old, patient suffered from heavy vaginal bleeding for sex months duration and seeking health care provider to treat it. Patient was diagnosed by physician as a case of endometrial hyperplasia and fiboid, after that she did hysrtecomy and bilateral salpingo-oorphectomy then specimen was taken as a biopsy to confirm the diagnosis, but unfortunately she loose the pathological report. From 2000 until 2015 the patient didn't suffer from any sign and symptoms related to her illness. In October 2015 the patient felt accidentally hard irregular lump in her left breast associated with red color skin change. After seeking health care provider, the patient did complete breast mass excision, a pathological report showed metastatic low grade sarcoma of endometrial origin. This result was confirmed by pathological features and IHC staining in which CD10 and vimentin were positive, but desmin, S100, SMA and CD 34 were negative. PR and ER were positive and Her 2 neu was negative. CT scan examination had shown multiple lung nodules, largest one measuring (2.9*2.9) cm in the right lower lobe.
In this case, the signs and symptoms of recurrence was happened after 15 years from initial problem that was treated by subtotal hysrtecomy and bilateral oorphectomy which was the main surgical procedures to treat LGSS and consider the first line treatment of LGSS [13]. Comparing to other case reports and series of LGESS, the disease may recur in a wide spectrum time ranged more than 17 years to less than 10 years. In a case report that Mean age at diagnosis was 55 years, and 59% were postmenopausal. The majority of patients with uterine sarcomas were postmenopausal. And the oncologist should be intervene with 41% of patients with uterine sarcoma had a high preoperative index of suspicion [11]. IHC used to confirm the diagnosis of LGSS , which is specific and sensitive to distinguish between uterine cancers especially between LGSS and leiomyosarcoma , in LGSS; ER,PR receptors and CD 10 are positive as what was found in Park and his colleagues case series which contains 10 cases of Endometrial stromal sarcoma that have positive CD10 and estrogen receptor (ER) in lung nodules biopsy that confirm that LG ESS was metastatic to the lug due to the same of IHC of it [12]. which was compatible with IHC in our case that revealed a positive test for CD10 +, vimentin, PR and ER while Her 2 neu, desmin, S100, SMA and CD 34. Concerning to recurrence treatment, Physician prescribed tamoxifen tab. 20 mg per day, after 3 months patients in our case did not show response with disease progression seen as increase in size of breast and lung nodules and appearance of another mass lesion which was seen in left hemipelvic and measured about 4.8*5.3 cm.
In Reich and Regauer case series was conducted in 2006, that contains of 10 patients with endometrial stromal sarcomas (ESS) were on estrogen replacement therapy (ERT) or on tamoxifen.
Physician was decided to discontinue of tamoxifen due to 8/10 of patients were developed metastatic disease [14] disease progression with tamoxifen uses also seen in a case of a 32-year-old woman with Which has an agonist effect on endometrial stromal cells that stimulate tumor growth and increase in the incidence of uterine sarcomas in women taking tamoxifen.
Moreover, tamoxifen has been associated with earlier recurrence and disease progression in ESS that available in literature review [16]. Megace syrup 160 mg / day was used as second choice after tamoxifen in our case which showed good response as tumor size regression. In three cases reports were reported in 2010 were mimic to our case ,those LG-ESS with lung and pelvic metastasis and have a history of hysterectomy, bilateral salpingo-oophorectomy ,all of three cases were treated with megestrol acetate for long periods and the disease under control from recurrence or local and distal metastatic [17] so surgery in addition using hormonal therapy for a long period achieve stabilization of the disease. Another case of LGESS was reported by Zaza KJ et al in 2015 with past history of uterine fibroids and treated by subtotal hysterectomy and left salpingo-oophorectomy ten years previously then treated by megestrol acetate, the disease was stable without any recurrence or metastatic for 2 years follow up [18].
Megestrol acetate was considered as effective hormonal therapy for multiple pulmonary metastases from LGSS which was prescribed in 2010 to the 30 years old female had LGSS with multiple pulmonary metastases and underwent Hysterectomy, right salpingo-oophorectomy, paraaortic and pelvic lymph node sampling, and omentectomy. Patient was treated with high dose megestrol acetate (800 mg/day) for 4 months followed by 200 mg/day for 20 months.
The nodules began to regress and completely disappeared 17 months after surgery. Patient still alive with asymptomatic pulmo-  [19]. In 2009, A case series contain 11patients with residual or recurrent LGESS treated by hormonal therapy just, were retrieved from patients' file with high response rate (median 48+ months). (4 complete response; 5 partial response), one showed stable disease (26+ months) and one progressive disease. This series confirm that hormonal therapy is a first line of treatment when LGSS recurrence and non resectable [20].

Conclusion
There is a possibility of recurrence of LGESS after long time of the first diagnosis (15) years in our case. Administration of adjuvant megestrol acetate daily was effective treatment to achieve favorable prognosis rather than administration of tamoxefin.