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was an increase in serum cancer antigen 125.She underwent bilateral adnexectomy and tumor resection.The right ovary had been replaced by a multinodular mass that was 8.5 cm×4.5 cm×3.5 cm in size.In addition,there was a 24 cm×15 cm×13 cm mesenteric mass,which was also multinodular,with local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
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 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 pelv...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.展开更多
OBJECTIVE To investigate the clinical and pathologicalcharacteristics, diagnosis and treatment of stromal sarcoma ofthe breast (SSB). Methods: The clinical and pathological dataof 6 patients with SSB treated between 1...OBJECTIVE To investigate the clinical and pathologicalcharacteristics, diagnosis and treatment of stromal sarcoma ofthe breast (SSB). Methods: The clinical and pathological dataof 6 patients with SSB treated between 1954 and 2007 wereretrospectively analyzed.METHODS The clinical and pathological data of 6 patients withSSB 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 rapidlyaugmented in a short time period in 3 patients. One patient haddiscontinuous pain and 3 patients had masses located in the upperouter quadrant of the breast. The median tumor radius was 6.0cm (range, 3-15 cm). According to the AJCC breast cancer stagingstandard (6th edition), 1 case was of stage ⅡA, 2 cases were ofstage ⅡB, 2 cases were of stage ⅢB and one case couldn't bestaged. Four patients were initially treated by excising the tumorand then undergoing mastectomy or modified radical mastectomyafter recurrence. Radical mastectomy was suitable for those withpectoralis major muscle involvement. Two patients receivedsimple mastectomy, 2 patients underwent radical mastectomy andanother 2 patients received modified radical mastectomy. Aftersurgery, all patients were identified as SSB through pathology,with focal ossification in one case and mucinous degeneration inanother one case. Four patients who underwent axillary lymphnode dissection did not have lymph node metastases. Threepatients received chemotherapy after surgery. After a medianfollow-up time of 36.5 months (8-204 months), 4 patients hadrecurrence after local excision and 3 patients had recurrence morethan 2 times with a median time to recurrence of 2.5 months (1to 4 months) after surgery. One patient had lung metastases at 7months after the initial surgery and the other 5 patients were alivewithout disease at the end of the follow-up period.CONCLUSION SSB is difficult to diagnose preoperativelyand is characterized by its tendency to recur locally. To obtainnegative margins, wide local excision or mastectomy must beperformed. Axillary lymph node dissection is not mandatory. Theroles of adjuvant chemotherapy and radiotherapy have still beencontroversial.展开更多
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 devel...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.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
Diffuse intestinal Kaposi's sarcoma shares macroscopicand histopathologic features with gastrointestinal stromatumors. Correct diagnosis may pose a clinical challengeWe describe the case of a young HIV-1-infected ...Diffuse intestinal Kaposi's sarcoma shares macroscopicand histopathologic features with gastrointestinal stromatumors. Correct diagnosis may pose a clinical challengeWe describe the case of a young HIV-1-infected Africanlady without advanced immunodeficiency, who presentedwith a diffuse spindle cell tumor of the gut. Initiadiagnosis was of a gastrointestinal stromal tumor, basedon endoscopy and histopathology. Further evaluationrevealed evidence for human herpesvirus 8 (HHV8) andthe diagnosis had to be changed to diffuse intestinaKaposi's sarcoma. Antiretroviral triple therapy togethewith chemotherapy was commenced, and has led to therapid remission of intestinal lesions. With a backgroundof HIV infection, the presence of HHV8 as the causativeagent of Kaposi's sarcoma should be determined, asdistinct treatment is indicated.展开更多
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. Th...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.展开更多
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 ...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.展开更多
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 metap...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.展开更多
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.
文摘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.
文摘OBJECTIVE To investigate the clinical and pathologicalcharacteristics, diagnosis and treatment of stromal sarcoma ofthe breast (SSB). Methods: The clinical and pathological dataof 6 patients with SSB treated between 1954 and 2007 wereretrospectively analyzed.METHODS The clinical and pathological data of 6 patients withSSB 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 rapidlyaugmented in a short time period in 3 patients. One patient haddiscontinuous pain and 3 patients had masses located in the upperouter quadrant of the breast. The median tumor radius was 6.0cm (range, 3-15 cm). According to the AJCC breast cancer stagingstandard (6th edition), 1 case was of stage ⅡA, 2 cases were ofstage ⅡB, 2 cases were of stage ⅢB and one case couldn't bestaged. Four patients were initially treated by excising the tumorand then undergoing mastectomy or modified radical mastectomyafter recurrence. Radical mastectomy was suitable for those withpectoralis major muscle involvement. Two patients receivedsimple mastectomy, 2 patients underwent radical mastectomy andanother 2 patients received modified radical mastectomy. Aftersurgery, all patients were identified as SSB through pathology,with focal ossification in one case and mucinous degeneration inanother one case. Four patients who underwent axillary lymphnode dissection did not have lymph node metastases. Threepatients received chemotherapy after surgery. After a medianfollow-up time of 36.5 months (8-204 months), 4 patients hadrecurrence after local excision and 3 patients had recurrence morethan 2 times with a median time to recurrence of 2.5 months (1to 4 months) after surgery. One patient had lung metastases at 7months after the initial surgery and the other 5 patients were alivewithout disease at the end of the follow-up period.CONCLUSION SSB is difficult to diagnose preoperativelyand is characterized by its tendency to recur locally. To obtainnegative margins, wide local excision or mastectomy must beperformed. Axillary lymph node dissection is not mandatory. Theroles of adjuvant chemotherapy and radiotherapy have still beencontroversial.
文摘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.
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.
文摘Diffuse intestinal Kaposi's sarcoma shares macroscopicand histopathologic features with gastrointestinal stromatumors. Correct diagnosis may pose a clinical challengeWe describe the case of a young HIV-1-infected Africanlady without advanced immunodeficiency, who presentedwith a diffuse spindle cell tumor of the gut. Initiadiagnosis was of a gastrointestinal stromal tumor, basedon endoscopy and histopathology. Further evaluationrevealed evidence for human herpesvirus 8 (HHV8) andthe diagnosis had to be changed to diffuse intestinaKaposi's sarcoma. Antiretroviral triple therapy togethewith chemotherapy was commenced, and has led to therapid remission of intestinal lesions. With a backgroundof HIV infection, the presence of HHV8 as the causativeagent of Kaposi's sarcoma should be determined, asdistinct treatment is indicated.
文摘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.
基金supported by CAMS Innovation Fund for Medical Sciences (CIFMS) (Grant No.2016-12M-2-004)Ministry of Science and Technology (Grant No.2014FY121100)
文摘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.
文摘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.
