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 polypo...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.展开更多
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 tho...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.展开更多
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 ...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.展开更多
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...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.展开更多
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...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.展开更多
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 ...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.展开更多
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 rep...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.展开更多
Endometrial stromal tumors(ESTs)include endometrial stromal nodule(ESN),low-grade endometrial stromal sarcoma(LG-ESS),high-grade endometrial stromal sarcoma(HG-ESS),and undifferentiated uterine sarcoma(UUS).Since thes...Endometrial stromal tumors(ESTs)include endometrial stromal nodule(ESN),low-grade endometrial stromal sarcoma(LG-ESS),high-grade endometrial stromal sarcoma(HG-ESS),and undifferentiated uterine sarcoma(UUS).Since these are rare tumor types,there is an unmet clinical need for the systematic therapy of advanced LG-ESS or HG-ESS.Cytogenetic and molecular advances in ESTs have shown that multiple recurrent gene fusions are present in a large proportion of LG-ESSs,and HG-ESSs are identified by the tyrosine 3-monooxygenase/tryptophan 5-monooxygenase activation protein epsilon(YWHAE)-family with sequence similarity 22(FAM22)fusion.Recently,a group of ESSs harboring both zinc finger CCCH domain-containing protein7 B(ZC3H7B)-B-cell lymphoma 6 corepressor(BCOR)fusion and internal tandem duplication(ITD)of the BCOR gene have been provisionally classified as HG-ESSs.In this review,we firstly describe current knowledge about the molecular characteristics of recurrent aberrant proteins and their roles in the tumorigenesis of LG-ESSs and HG-ESSs.Next,we summarize the possibly shared signal pathways in the tumorigenesis of LG-ESSs and HG-ESSs,and list potentially actionable targets.Finally,based on the above discussion,we propose a few promising therapeutic strategies for LG-ESSs and HG-ESSs with recurrent gene alterations.展开更多
文摘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.
基金Supported by Open Foundation of Key Laboratory of Birth Regulation and Control Technology of National Health Commission of China,No.2018KF003.
文摘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.
文摘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.
文摘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.
文摘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.
基金supported by grants no.81670123 and no.81670144 from the National Natural Science Foundation of China(NSFC).
文摘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.
文摘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.
基金supported by the WU JIEPING MEDICAL FOUNDATION(No.320.6750.18489),China。
文摘Endometrial stromal tumors(ESTs)include endometrial stromal nodule(ESN),low-grade endometrial stromal sarcoma(LG-ESS),high-grade endometrial stromal sarcoma(HG-ESS),and undifferentiated uterine sarcoma(UUS).Since these are rare tumor types,there is an unmet clinical need for the systematic therapy of advanced LG-ESS or HG-ESS.Cytogenetic and molecular advances in ESTs have shown that multiple recurrent gene fusions are present in a large proportion of LG-ESSs,and HG-ESSs are identified by the tyrosine 3-monooxygenase/tryptophan 5-monooxygenase activation protein epsilon(YWHAE)-family with sequence similarity 22(FAM22)fusion.Recently,a group of ESSs harboring both zinc finger CCCH domain-containing protein7 B(ZC3H7B)-B-cell lymphoma 6 corepressor(BCOR)fusion and internal tandem duplication(ITD)of the BCOR gene have been provisionally classified as HG-ESSs.In this review,we firstly describe current knowledge about the molecular characteristics of recurrent aberrant proteins and their roles in the tumorigenesis of LG-ESSs and HG-ESSs.Next,we summarize the possibly shared signal pathways in the tumorigenesis of LG-ESSs and HG-ESSs,and list potentially actionable targets.Finally,based on the above discussion,we propose a few promising therapeutic strategies for LG-ESSs and HG-ESSs with recurrent gene alterations.
