Metastatic low-grade endometrial stromal sarcoma of the sigmoid colon three years after hysterectomy

A 49-year-old woman, who had undergone hysterectomy for low-grade endometrial stromal sarcoma (ESS) 3 years ago, presented with a 2-wk history of lower abdominal pain. Barium enema and sigmoidoscopy disclosed a polypoid submucosal tumor. Histopathologic features of biopsy specimens from the lesion were similar to those of the resected uterine ESS. Under the diagnosis of metastatic ESS of the sigmoid colon, sigmoidectomy was performed. Microscopic examination demonstrated dense proliferation of spindle cells with little nuclear atypia, which were sometimes arranged in whorled pattern around abundant arterioles. Mitotic count is below 1 in 10 highpower fields. Immunohi-stochemically, the neoplastic cells were strongly positive for vimentin, estrogen receptor and progesterone receptor but negative for α-smooth muscle actin, S-100 protein and CD34. Thus, a final diagnosis of low-grade ESS metastasis to the sigmoid colon was made.Her postoperative course was uneventful and hormonal therapy with progestational agents is entertained.

Fertility-sparing surgeries without adjuvant therapy through term pregnancies in a patient with low-grade endometrial stromal sarcoma:A case report

BACKGROUND Low-grade endometrial stromal sarcoma(LGESS)is a rare indolent tumor with a favorable prognosis.With the importance of improving quality of life recognized,fertility-sparing surgery may be an option for those young women.However,most of the reports suggested that stage IA patients might be candidates for fertility-sparing surgery,and adjuvant hormonal treatment was considered a feasible adjuvant therapy for reducing the recurrence risk of patients with LGESS and hysterectomy was recommended after the completion of pregnancy and delivery.CASE SUMMARY A 28-year-old pregnant woman diagnosed with stage IB LGESS was treated by fertility-sparing surgery when term cesarean section delivery was performed.Without any adjuvant treatment,she had the other successful term pregnancy and cesarean section 45 mo after first fertility-sparing surgery.Moreover,only hysteroscopic resection was performed to retain fertility again even when the tumor recurred after 6 years.So far the patient’s fertility and disease-free status have remained for more than 8 years without any adjuvant therapy despite local resection of the sarcoma.And the two babies were in good health.CONCLUSION For young patients with stage I LGESS,it seems that repeated fertility-sparing surgeries could be performed even after two term deliveries and the tumor recurrence,and it might be attempted without adjuvant therapy but the counseling should be considered as mandatory.

Metastatic low-grade endometrial stromal sarcoma with variable morphologies in the ovaries and mesentery: A case report

BACKGROUND Low-grade endometrial stromal sarcoma(LGESS)classically exhibits a proliferative morphology.However,morphological variation of extrauterine tumors presents a diagnostic challenge.CASE SUMMARY We report the case of a 76-year-old female patient with extensive extrauterine and abdominal neoplastic lesions.Computed tomography showed massive pleural and ascitic fluid,and there was an increase in serum cancer antigen 125.She underwent bilateral adnexectomy and tumor resection.The right ovary had been replaced by a multinodular mass that was 8.5 cm×4.5 cm×3.5 cm in size.In addition,there was a 24 cm×15 cm×13 cm mesenteric mass,which was also multinodular,with local invasion of the intestinal serosa and underlying muscle.Under the microscope,the tumors in different places exhibited two different patterns,thus presenting great challenges to diagnosis and treatment.Thorough pathological assessment eliminated all differential diagnoses in favor of metastatic LGESS derived from a 20-year-old primary tumor initially misdiagnosed as leiomyosarcoma.CONCLUSION LGESS morphology varies according to tumor location.Accurate diagnosis is critical for appropriate treatment and improved prognosis and patient care.

Endometrial stromal sarcoma extending to the pulmonary artery:A rare case report

BACKGROUND Endometrial stromal sarcoma(ESS)is a rare malignant mesenchymal tumor.Early in the disease,the findings on magnetic resonance imaging are similar to those of leiomyoma.When the lesion involves both vascular and cardiac tissue,it might be misdiagnosed as intravenous leiomyomatosis,which is not common in the clinic.CASE SUMMARY We present the case of a 34-year-old female patient with tumor embolus,which extended from the right iliac vein and ovarian vein to the inferior vena cava(IVC),and then to the right atrium and right ventricle,and finally protruded into the pulmonary artery.The patient had undergone a hystero-myomectomy 7 years previously.Based on the findings of the imaging examinations,the diagnosis of intravenous leiomyomatosis was considered preoperatively.The patient then underwent complete resection of the endovascular and intracardiac tumor embolus.The postoperative pathology results confirmed metastatic ESS with endovascular and intracardiac involvement.The patient was discharged from hospital in good condition,and there was no sign of recurrence 5 mo after the operation.CONCLUSION Extending from the iliac vein and ovarian vein to the IVC,this metastatic ESS invaded both vascular and cardiac tissues.For patients with ESS involving vascular and cardiac tissues,pathological examinations are essential for the differential diagnosis,such as intravenous leiomyomatosis.In addition,due to the high recurrence rate of ESS,long-term and close follow-up evaluation is necessary.

Endometrial stromal sarcoma:a case diagnosed during infertility treatment

The patient with irregular menstruation went to our Department(Gynecological Department,the Third Affiliated Hospital,Sun Yat-sen University,Guangzhou,China) with the complaint of no pregnancy at the age of 26 in 2007,and oral contraceptive pills were given when simple type of endometrial hyperplasia was pathologically diagnosed,and then ovulation induction was performed.But after nearly two years from the beginning of infertility treatment,endometrial stromal sarcoma was diagnosed and radical treatment was performed for the young patient.And the relative relationship between endometrial stromal sarcoma and infertility is worth discussing.

Metastatic low-grade endometrial stromal sarcoma with sex cord and smooth muscle differentiation: A case report

BACKGROUND Metastatic low-grade endometrial stromal sarcoma(LG-ESS) with sex cord-like and smooth muscle-like differentiation is rare. This article reports such a case with multiple recurrences and with extensive pelvic and abdominal metastasis.CASE SUMMARY A 47-year-old female patient was diagnosed with multiple cystic masses in the pelvic cavity by magnetic resonance imaging examination. Based on the postoperative pathological and immunohistochemical analyses of the surgical specimen, she was diagnosed with a metastatic low-grade endometrial stromal sarcoma with sex cord and smooth muscle differentiation.CONCLUSION LG-ESS is a low-grade malignant tumor with a high recurrence rate and metastasis probability. It is easily misdiagnosed initially. It is essential to distinguish LG-ESS with sex cord-like differentiation from uterine tumour resembling ovarian sex cord tumour.

Liver metastasis of endometrial stromal sarcoma

Resection of liver metastases from gynaecological tumours is uncommon.Endometrial stromal sarcomas(ESS) are low incidence gynecological tumours which can originate in previous sites of endometriosis or following metaplasia of the pelvic peritoneal wall,and which are exceptionally associated with liver metastasis.We present a 68-year-old woman with a ESS and metachronic liver metastasis treated by liver resection.There is very little literature on clinical management about liver metastasis from ESS,but extrapolating the data obtained with liver metastasis from sarcomas and uterine tumours,we should recommend resection as this is considered a resectable extrauterine metastasis.In cases with more sites of extrauterine disease,liver resection should also be performed if the other sites are resectable.

Experience of endometrial stromal sarcoma with fertility conservation treatment

The paper introduced the experience of a case of recurrence of endometrial stromal sarcoma with fertility conservation treatment after full-term delivery. A 24-year-old unmarried patient who, with her increasing amount of menstruation in the late six months, had a mass in her lower-abdominal which was confirmed by B-ultrasonic diagnosis as myometrium mixed mass, was given an enucleation of uterus tumor. The pathological examination of tumor sample revealed it as lowgrade endometrial stromal sarcoma (LESS). And the patient underwent 6 cycles of chemotherapy over 8 months before she got pregnant and had a full-term delivery. But in 34 months after delivery, the disease recurred with metastatic of pelvic and peritoneal cavity and the patient had to receive comprehensive therapy. Conclusion: The unmarried young patient with stage I low-grade endometrial stromal sarcoma has the chance to preserve uterus for her pregnancy and delivery. But the using of cesarean needs do staging operation and prognosis evaluation.

Endometrial stromal sarcoma presenting as multiple primary tumor

Endometrial Stromal Sarcoma (ESS) is a rare uterine malignancy. The annual incidence of ESS has been reported as 1 - 2 per million women. In the general population approximately 10 percent of cancer patients may develop a second primary cancer. Synchronous multiple primary tumors constitute only 20% of all multiple primary tumors, and by definition, occur within a period of two month. This case report is presented to document the occurrence of ESS as a “Synchronous Multiple Primary Tumor”, which is an extremely rare event. Previously only one case report has documented such an occurrence. The literature is also briefly reviewed. This case underlines the requirement of a thorough initial evaluation for a patient with a gynecological malignancy and a regular follow-up. Follow-up includes patient education regarding symptoms of any recurrence, physical examination repeated at 6 - 12 monthly interval, along with imaging studies when indicated.

Correlation between magnetic resonance diffusion-weighted imaging ADC value of endometrial stromal sarcoma and the malignant biological features

Objective:To study the correlation between magnetic resonance diffusion-weighted imaging ADC value of endometrial stromal sarcoma and the malignant biological features.Methods:A total of 34 patients with endometrial stromal sarcoma who received surgical resection in Hubei Provincial Hospital of Integrated Chinese & Western Medicine between May 2014 and August 2016 were selected as the malignant group of the research, and 58 patients with uterine fibroids who received surgical resection between August 2015 and October 2016 were selected as the control group of the research. Magnetic resonance diffusion-weighted imaging was done before operation to measure apparent diffusion coefficient (ADC value). The lesions were collected after operation to determine the expression of proliferation genes as well as estrogen and progestogen receptors.Results:Endometrial stromal sarcoma ADC value of malignant group was significantly lower than uterine fibroid ADC value of control group;CyclinD1, Rb, Sp1, Survivin, ER , ERβ, PRA and PRB protein expression in endometrial stromal sarcoma lesions of malignant group were significantly higher than those of control group while SULT1E1 protein expression was significantly lower than that of control group;CyclinD1, Rb, Sp1, Survivin, ER , ERβ, PRA and PRB protein expression in endometrial stromal sarcoma lesions of subgroup with low ADC value were significantly higher than those of subgroup with high ADC value while SULT1E1 protein expression was significantly lower than that of subgroup with high ADC value.Conclusion: Magnetic resonance diffusion weighted imaging ADC values can be used to evaluate the malignant biological behavior of endometrial stromal sarcoma.

A nomogramfor predicting overall survival in patients with low-grade endometrial stromal sarcoma: A population-based analysis

Background:Low-grade endometrial stromal sarcoma(LG-ESS)is a rare tumor that lacks a prognostic prediction model.Our study aimed to develop a nomogram to predict overall survival of LG-ESS patients.Methods:A total of 1172 patients confirmed to have LG-ESS between 1988 and 2015 were selected from the Surveillance,Epidemiology and End Results(SEER)database.They were further divided into a training cohort and a validation cohort.The Akaike information criterion was used to select variables for the nomogram.The discrimination and calibration of the nomogram were evaluated using concordance index(C-index),area under time-dependent receiver operating characteristic curve(time-dependent AUC),and calibration plots.The net benefits of the nomogram at different threshold probabilities were quantified and compared with those of the International Federation of Gynecology and Obstetrics(FIGO)criteria-based tumor staging using decision curve analysis(DCA).Net reclassification index(NRI)and integrated discrimination improvement(IDI)were also used to compare the nomogram’s clinical utilitywith that of the FIGO criteria-based tumor staging.The risk stratifications of the nomogram and the FIGO criteria-based tumor staging were compared.Results:Seven variables were selected to establish the nomogram for LG-ESS.The C-index(0.814 for the training cohort and 0.837 for the validation cohort)and the time-dependent AUC(>0.7)indicated satisfactory discriminative ability of the nomogram.The calibration plots showed favorable consistency between the prediction of the nomogram and actual observations in both the training and validation cohorts.The NRI values(training cohort:0.271 for 5-year and 0.433 for 10-year OS prediction;validation cohort:0.310 for 5-year and 0.383 for 10-year OS prediction)and IDI(training cohort:0.146 for 5-year and 0.185 for 10-year OS prediction;validation cohort:0.177 for 5-year and 0.191 for 10-year OS prediction)indicated that the established nomogram performed significantly better than the FIGO criteria-based tumor staging alone(P<0.05).Furthermore,DCA showed that the nomogram was clinically useful and had better discriminative ability to recognize patients at high risk than the FIGO criteria-based tumor staging.Conclusions:A prognostic nomogram was developed and validated to assist clinicians in evaluating prognosis of LG-ESS patients.

Successful term pregnancy after laparoscopic surgery of low grade endometrial stromal sarcoma

Low grade endometrial stromal sarcoma （LGESS） is a ,very rare malignant tumor. The standard treatment of LGESS is total hysterectomy with bilateral adnexectomy. Fertility preserving treatment for LGESS is rarely reported and the prognosis needs to be studied. Here we report a case of term birth pregnancy after fertility preserving treatment for LGESS.

Multifactor Regulation on Expressions of MMP-9 and TIMP-1 in Endometrial Stromal Cells

Objective To investigate the regulatory effect of multifactor on the matrix metalloproteinases-9 （MMP-9） and the tissue inhibitor of metalloproteinase-1 （TIMP-1） in endometrial stromal cells. Methods The endometrial stromal cells separated from the proliferative endometrial tissues were incubated with medium alone, 17-β estradiol （E2,10^-8 mol/L）, medroxyprogesterone acetate （MPA, 10^-6 mol/L）, E2（10^-8 mol/L）＋MPA （10^-6 mol/L）, E2 （10^-8 mol/L）＋MPA （10^-6 mol/L）＋RU486 （10^-5 mol/L） or HB-EGF （10 ng/ml） for 48 h respectively. The expressions of MMP-9 and TIMP-1 were detected by in situ hybridization, immunocytochemistry, reverse transcriptase-polymerase chain reaction （RT-PCR） and Western blotting. Results Compared with control group [mRNA, 0. 729 ± 0. 090 （MMP-9） and 1.056± 0.154 （TIMP-1）; protein, 0.545 ±0.086 （MMP-9） and 0.745 ±0.154 （TIMP-1）], expressions of MMP-9 and TIMP-1 in E2 alone, progestin alone or E2 combined with progestin group were respectively：mRNA, 0.413 ± 0.069, 0.402 ± 0.073 and 0.407 ± 0.039; 0.487 ± 0.093, 0.503 ± 0.093 and 0.468 ± 0.075：protein, 0.294 ± 0.076, 0.331 ±0.064 and 0.265 ±0.049; 0.425 ±0.085, 0.397 ±0.065 and 0.435 ± 0.099. RU486 weakened the expression level of down-regulation, while HB-EGF elevated the level of MMP-9 and TIMP-1 after 48 h treatment （mRNA, 0.955 ± 0.068 and 1.396 ± 0.238; protein, 0. 780 ± 0.109 and 0.985 ± 0.165）. Conclusions 1） Both E2 and progestin can down-regulate the expressions of MMP-9 and TIMP-1 in endometrial stromal cells, but RU486 can inhibit the effect. 2） HB-EGF can elevate the level of MMP-9 and TIMP-1. 3） E2, progestin and HB-EGF have effect on the ratio of MM-P/TIMP-1.

Intestinal Kaposi's sarcoma may mimic gastrointestinal stromal tumor in HIV infection

Diffuse intestinal Kaposi＇s sarcoma shares macroscopic and histopathologic features with gastrointestinal stromal tumors. Correct diagnosis may pose a clinical challenge. We describe the case of a young HIV-1-infected African lady without advanced immunodeficiency, who presented with a diffuse spindle cell tumor of the gut. Initial diagnosis was of a gastrointestinal stromal tumor, based on endoscopy and histopathology. Further evaluation revealed evidence for human herpesvirus 8 （HHV8） and the diagnosis had to be changed to diffuse intestinal Kaposi＇s sarcoma. Antiretroviral triple therapy together with chemotherapy was commenced, and has led to the rapid remission of intestinal lesions. With a background of HIV infection, the presence of HHV8 as the causative agent of Kaposi＇s sarcoma should be determined, as distinct treatment is indicated.

Gradient decrease in telomerase and its RNA in progressive passage of human endometrial stromal stem cells

The purpose of this study was to explore the change of telomerase in passage from human endometrial stromal stem cells isolated from human endometrium.Telomerase activity of cultured endometrial stromal cells was assessed at mRNA and protein levels using RT-PCR and immunohistochemistry technique.Telomerase mRNA and protein levels were higher at early passages,and then had a gradually decreased trend of immunoreactive intensity and gradually weakened positive cells with progressive passage in endometrial stromal stem cells.These results suggest that telomerase is contributed to the insenecence of endometrial stem cell.

Analysis of 6 Cases of Stromal Sarcoma of the Breast

OBJECTIVE To investigate the clinical and pathological characteristics, diagnosis and treatment of stromal sarcoma of the breast （SSB）. Methods： The clinical and pathological data of 6 patients with SSB treated between 1954 and 2007 were retrospectively analyzed. METHODS The clinical and pathological data of 6 patients with SSB treated between 1954 and 2007 were retrospectively analyzed. RESULTS All patients were female and one was menopausal. The median age of the patients was 39 years old （range, 20-55）. All cases had a history of a palpable mass. The tumor rapidly augmented in a short time period in 3 patients. One patient had discontinuous pain and 3 patients had masses located in the upper outer quadrant of the breast. The median tumor radius was 6.0 cm （range, 3-15 cm）. According to the AJCC breast cancer staging standard （6th edition）, 1 case was of stage ⅡA, 2 cases were of stage ⅡB, 2 cases were of stage ⅢB and one case couldn＇t be staged. Four patients were initially treated by excising the tumor and then undergoing mastectomy or modified radical mastectomy after recurrence. Radical mastectomy was suitable for those with pectoralis major muscle involvement. Two patients received simple mastectom）~ 2 patients underwent radical mastectomy and another 2 patients received modified radical mastectomy. After surgery, all patients were identified as SSB through pathology, with focal ossification in one case and mucinous degeneration in another one case. Four patients who underwent axillary lymph node dissection did not have lymph node metastases. Three patients received chemotherapy after surgery. After a median follow-up time of 36.5 months （8-204 months）, 4 patients had recurrence after local excision and 3 patients had recurrence more than 2 times with a median time to recurrence of 2.5 months （1 to 4 months） after surgery. One patient had lung metastases at 7 months after the initial surgery and the other 5 patients were alive without disease at the end of the follow-up period. CONCLUSION SSB is difficult to diagnose preoperatively and is characterized by its tendency to .recur locally. To obtain negative margins, wide local excision or mastectomy must be performed. Axillary lymph node dissection is not mandatory. The roles of adjuvant chemotherapy and radiotherapy have still been controversial.

Current status of the adjuvant therapy in uterine sarcoma: A literature review

Uterine sarcomas (US) are rare mesenchymal tumours accounting approximately for 3%–7% of all uterine cancers. Histologically, US are classified into mesenchymal tumours or mixed epithelial and mesenchymal tumours. The group of mesenchymal tumours includes uterine leiomyosarcoma (uLMS, 65% of cases), endometrial stromal sarcoma (ESS, 21%)– traditionally divided into low grade (LG-ESS) and high grade–undifferentiated uterine sarcoma (5%) and other rare subtypes such as alveolar or embryonal rhabdomyosarcoma. Despite the fact that several drugs demonstrated clinical activity in advanced or metastatic settings, the role of postoperative therapy in US remains controversial. In this review, we have summarised the current state of the art, including the chief trials on adjuvant treatment modalities in US, especially focusing on uLMS, LG-ESS and other rare histotypes.

TNF<i>α</i>and IL1<i>β</i>Stimulate Differential Gene Expression in Endometrial Stromal Cells

The purpose of this study was to test the hypothesis that specific macrophage-secreted cytokines cause gene expression changes in endometrial stromal cells that reproduce the effects of macro-phages in the development of endometriosis. Telomerase-immortalized human endometrial stromal cells (T-HESC) were treated with tumor necrosis factor α (TNFα, 5 ng/ml) and interleukin 1β (IL1β, 1 ng/ml). Differential expression of 249 genes was identified by DNA microarray. Ontologies such as peptidases, cell adhesion, cell death/cell cycle, growth factors, cytoskeletal organization, defense/immune system, signal transduction, and transcriptional regulation which are related to the development of endometriosis were represented by these genes. The up-regulation of interleukin 8 (IL8), interleukin 6 (IL6), IL1β and matrix metallopro-teinase 3 (MMP3) in response to TNFα ± ILIβ in T-HESC cells was confirmed by real time RT-PCR. TNFα ± ILIβ did not affect the migration or invasion of T-HESC cells. This study reinforces our previous investigations on communication between cells of the immune system and endometrial stromal cells and their potential role in the development of endometriosis.

Effect of deforolimus and VEGF on angiogenesis in endometrial stromal cells following three-dimensional culture

The presence of endometrial tissue outside of the uterine cavity is named endometriosis and is the most common gynecologic disorder in women. Determining the inhibitory effect of a Deforolimus on angiogenesis in a three-dimensional (3-D) culture of human endometrial stromal cells (hEnCs) in vitro. The important mechanism in the pathogenesis of endometriosis is angiogenesis, and deforolimus has been shown to have anti-angiogenic activity. This was an in vitro study of human endometrial stromal cells in 3-D culture of fibrin matrix. Endometrial stromal cells isolated and placed in a 3-D fibrin matrix culture system for angiogenesis with VEGF and inhibit angiogenesis by deforolimus. Finally these cells analyzed by CD31 antibodies. After 3 weeks, in cells treated with VEGF, endothelial cell branching was observed and rudimentary capillary-like structures formed. In the presence of 5μM of deforolimus, angiogenesis was reduced. The deforolimus were shown to be effective in inhibiting the mechanisms of angiogenesis.

Incidence, distribution of histological subtypes and primary sites of soft tissue sarcoma in China

Objective: Soft tissue sarcomas(STSs) are rare malignancies deriving from mesenchyme.In this study, we reported the epidemiology of STS in China using population-based cancer registry data.Methods: In 2017, qualified data from 339 cancer registries were included in the national database.All STS cases were retrieved based on the morphological and topographical codes of International Classification of Diseases for Oncology, and were categorized into different histological subtypes and primary sites accordingly.Nationwide new STS cases were estimated using incidence rate of STS and the national population, and were reported for gastrointestinal stromal tumor(GIST) and STSs other than GIST separately by sex and region.Distribution of histological subtypes and primary sites of STS were calculated, as well as primary sites of GIST.Results: Approximately 39,900 new STS cases occurred nationwide in China in 2014, accounting for 1.05% of overall cancer incidence.The crude incidence rate was 2.91/100,000 and generally increased with age.An overall female predilection was found.GIST was the most common histological subtype, followed by nerve sheath tumor and malignant peripheral nerve sheath tumor,leiomyosarcoma, liposarcoma, and fibrosarcoma.About 67.5% of GIST occurred in stomach while 1.4% were recorded outside the gastrointestinal tract.Connective, subcutaneous and other soft tissues were the most common primary site, of which extremities were the major subsite.Conclusions: The burden of STS is not serious in China relatively.However, due to their histological and topographical complexity, STSs should not be unnoticed, and more basic and clinical studies should focus on STSs.