Prognostic factors, oncological treatment and outcomes of uterine sarcoma: 10 years' clinical experience from a tertiary care center in Pakistan.

BACKGROUND: Uterine sarcoma is an uncommon aggressive malignancy. Optimal management and prognostic factors have yet to be well recognized due to their rarity and various histological subtypes. This study aims to investigate these patients' prognostic factors, treatment modalities, and oncological outcomes. METHODS: A single-center retrospective cohort study was conducted on all patients diagnosed with uterine sarcoma and treated from January 2010 to December 2019 in a tertiary-care hospital in Pakistan. The data were analyzed using STATA software and stratified on the histological subtype. Survival rates were estimated using the Kaplan-Meier method. Crude and adjusted hazard ratios with 95% CI were estimated using univariate and multivariate analysis. RESULTS: Of the 40 patients, 16(40%) had uterine leiomyosarcoma (u-LMS), 10(25%) had high-grade endometrial stromal sarcoma (HGESS), 8(20%) had low-grade endometrial stromal sarcoma (LGESS) and 6(15%) had other histological subtypes. The median age of all patients was 49 (40-55.5). Thirty-seven (92.5%) patients underwent primary surgical resection, and 24 (60%) patients received adjuvant systemic chemotherapy. The survival plots showed the overall population's DFS of 64 months and the OS of 88 months (p-value = 0.001). The median DFS in all patients was 12 months, and the median OS was 14 months (p-value = 0.001). A small but significant DFS benefit was found in patients who received adjuvant systemic chemotherapy, 13.5 versus 11 months (p-value = 0.001). Multivariate Cox-regression analysis revealed that large tumor size and advanced FIGO stage were substantial factors associated with decreased survival. CONCLUSION: Uterine sarcomas are rare malignancies with poor prognosis. Multiple factors, including tumor size, mitotic count, stage of the disease, and myometrial invasion, impact survival outcomes. Adjuvant treatment may decrease the recurrence rate and improve DFS but do not affect OS.

Endometrial stromal tumors of the uterus: Epidemiology, pathological and biological features, treatment options and clinical outcomes.

Endometrial stromal tumors (EST) are uterine mesenchymal tumors, which histologically resemble endometrial stroma of the functioning endometrium. The majority of EST are malignant tumors classified as low-grade endometrial stromal sarcoma (LG-ESS), high-grade endometrial stromal sarcoma (HG-ESS), and undifferentiated uterine sarcoma (UUS). Overall, ESTs are rare malignancies, with an annual incidence of approximately 0.30 per 100'000 women, mainly affecting peri- or postmenopausal women. The most common genetic alteration identified in LG-ESS is the JAZF1-SUZ12 rearrangement, while t(10;17)(q23,p13) translocation and BCOR gene abnormalities characterize two major subtypes of HG-ESS. The absence of specific genetic abnormalities is the actual hallmark of UUS. Unlike HG-ESSs, LG-ESSs usually express estrogen and progesterone receptors. Total hysterectomy without morcellation and bilateral salpingo-oophorectomy (BSO) is the first-line treatment of early-stage LG-ESS. Ovarian preservation, fertility-sparing treatment, and adjuvant hormonal therapy ± radiotherapy may be an option in selected cases. In advanced or recurrent LG-ESS, surgical cytoreduction followed by hormonal treatment, or vice versa, are acceptable treatments. The standard treatment for apparently early-stage HG-ESS and UUS is total hysterectomy without morcellation with BSO. Ovarian preservation and adjuvant chemotherapy ± radiotherapy may be an option. In advanced or recurrent HG-ESS, surgical cytoreduction and neoadjuvant or adjuvant chemotherapy can be considered. Alternative treatments, including biological agents and immunotherapy, are under investigation. LG-ESSs are indolent tumor with a 5-year overall survival (OS) of 80-100% and present as stage I-II at diagnosis in two third of patients. HG-ESSs carry a poor prognosis, with a median OS ranging from 11 to 24 months, and 70% of patients are in stage III-IV at presentation. UUS median OS ranges from 12 to 23 months and, at diagnosis, 70% of patients are in stage III-IV. The aim of this review is to assess the clinical, pathological, and biological features and the therapeutic options for malignant ESTs.

A retrospective study of endometrial stromal sarcoma: an institutional review.

This is a retrospective study conducted at Shaukat Khanum Memorial Cancer Hospital, Lahore, from January 1995 to April 2016, to determine the clinical presentations, pathological features, cancer free survival and rate of recurrence in patients with Endometrial Stromal Sarcoma (ESS). Data was collected from May to August 2017. A total of 31 patients with a mean age of 40.0±11.72 years were treated. Among them, 12 (38.7%) had stage I, 2 (6.4%) had stage II, 6 (19.3%) had stage III and 11 (35.5%) had stage IV ESS. All patients underwent surgical management as an initial treatment modality for ESS. Out of these 31 patients, 17 were under active surveillance, 4 had expired and 10 patients were lost to follow up. Eleven (65%) patients were disease free, recurrence was noted in 4 (23.5%) patients and 2 (12%) patients had persistent disease. Recurrence of disease was managed with surgical excision and multimodality treatment. Median duration of follow-up was 38.29 months. Endometrial stromal sarcoma (ESS) is a rare uterine tumour. Our patients were young and had lower rate of recurrence. Surgical management was the mainstay of treatment in patients with resectable disease while other options used included hormonal therapy, radio therapy or chemotherapy.

Management of uterine sarcomas and prognostic indicators: real world data from a single-institution.

BACKGROUND: Uterine sarcomas consist a heterogeneous group of mesenchymal gynecological malignancies with unclear therapeutic recommendations and unspecific but poor prognosis, since they usually metastasize and tend to recur very often, even in early stages. METHODS: We retrospectively analyzed all female patients with uterine sarcomas treated in our institution over the last 17 years. Clinico-pathological data, treatments and outcomes were recorded. Kaplan-Meier curves were plotted and time-to-event analyses were estimated using Cox regression. RESULTS: Data were retrieved from 61 women with a median age of 53 (range: 27-78) years, at diagnosis. Fifty-one patients were diagnosed with leiomyosarcoma (LMS), 3 with high grade endometrial stromal sarcoma (ESS), 5 with undifferentiated uterine sarcoma (UUS), 1 with Ewing sarcoma (ES) and 1 with Rhabdomyosarcoma (RS). 24 cases had stage I, 7 stage II, 14 stage III and 16 stage IV disease. Median disease-free survival (DFS) in adjuvant approach was 18.83 months, and median overall survival (OS) 31.07 months. High mitotic count (> 15 mitoses) was significantly associated with worse OS (P < 0.001) and worse DFS (P = 0.028). CONCLUSIONS: Mitotic count appears to be independent prognostic factor while further insights are needed to improve adjuvant and palliative treatment of uterine sarcomas.

Undiagnosed Uterine Sarcomas Identified During Surgery for Presumed Leiomyoma at a National Tertiary Hospital in Thailand: A 10-Year Review.

OBJECTIVES: The aim of this study was to evaluate the rate, clinical characteristics, and survival outcomes of an undiagnosed uterine malignancy in patients who underwent surgical treatment for presumed leiomyomas. METHODS: Medical records of patients who underwent surgical treatment for presumed leiomyomas, from January 2004 to September 2013, were retrospectively reviewed, and the data were followed until September 2016. Demographic data, tumor characteristics, oncologic treatment, and response rate were analyzed by descriptive statistics. Kaplan-Meier method was used for survival analysis. This study includes follow-up data through December 31, 2016. RESULTS: A total of 11,258 medical records of presumed leiomyoma patients undergoing hysterectomy during the studied period were reviewed. Pathology of uterine sarcoma was found in 22 patients (0.2%), all of whom were included. Nineteen patients had leiomyosarcoma, and 3 had endometrial stromal sarcoma. Mean age of patients was 48.3 ± 6.9 years. All patients underwent total abdominal hysterectomy, with 20 patients undergoing concurrent bilateral salpingo-oophorectomy. Uterine sarcoma was classified as stage IB in 21 patients and stage IIIC in 1 patient. Fifteen patients were prescribed the following adjuvant treatment: chemotherapy in 13 patients and megestrol acetate in 2 patients. Thirteen patients had recurrent disease, and 3 patients died of their disease. The mean progression-free survival was 50.1 ± 41.3 months, and overall survival was 59.3 ± 43.0 months. CONCLUSIONS: One in 512 patients who underwent hysterectomy because of presumed uterine leiomyomas had inadvertent uterine sarcomas. Even with adjuvant therapy, treatment outcome was rather poor, with almost 60% recurrence rate and median progression-free survival and overall survival of less than 5 years.

Prevalence of occult pre-malignant or malignant pathology at the time of uterine morcellation for benign disease.

OBJECTIVE: To determine the prevalence of occult pre-malignant or malignant uterine pathology at the time of laparoscopic surgery with open power morcellation for benign gynecologic disease. METHODS: The present multicenter, retrospective cohort study included women who underwent open power morcellation for benign indications between January 1, 2007, and February 28, 2014, at three academic medical centers in the USA. The primary outcome was pre-malignant or malignant pathology at the time of open power morcellation, and was determined from the patients' pathology reports. RESULTS: During the study period, 1214 women underwent open power morcellation for benign indications. Similar preoperative characteristics were observed between patients with normal pathology and those with pre-malignant or malignant uterine pathology, including body mass index, parity, hypertension, diabetes, breast cancer, and smoking (all P>0.129). Among patients who underwent open power morcellation, 14 (1.2%) had occult pre-malignant or malignant pathology; 5 (0.4%) women had endometrial adenocarcinoma and 1 (0.1%) had low-grade endometrial stromal sarcoma. There were eight patients with malignant pathology who underwent additional surgical exploration and were disease free at their final clinical visit, with a median follow-up time of 42.0 months (interquartile range 5.0-62.0 months). CONCLUSION: Endometrial adenocarcinoma and low-grade endometrial stromal sarcoma were rare in the present study and there were no reports of leiomyosarcoma.

Uterine Sarcoma: Analysis of 13,089 Cases Based on Surveillance, Epidemiology, and End Results Database.

OBJECTIVE: The aim was to study the incidence and survival of patients with uterine sarcoma diagnosed in the period from 2000 to 2012 based on Surveillance, Epidemiology, and End Results (SEER) database. METHODS: All 18 registries of the SEER database were used to select cases. We included women aged 30 years or older diagnosed with uterine sarcoma. Histological subtypes were defined as leiomyosarcoma, carcinosarcoma, stromal sarcoma, adenosarcoma, and sarcoma not otherwise specified according to the 2003 World Health Organization classification. Using SEER*Stat software version 8.1.2. We calculated the age-adjusted incidence rates, extent of disease at time of diagnosis, and survival rates with different treatment modalities for white, black, and other races. Univariate and multivariate Cox proportional hazards analysis were done to examine factors affecting survival. RESULTS: We identified 13,089 patients diagnosed with uterine sarcoma in the period from 2000 to 2012. The age-adjusted incidence rate for patients aged 50 years or older was more than that of younger patients (6.4/10 vs 1.5/10, P < 0.0001). Also, the age-adjusted incidence rate for black women was twice that of white women (7.3/10 vs 3.5/10, P < 0.0001). Carcinosarcoma was the most commonly diagnosed subtype followed by leiomyosarcoma. Women aged 50 years or older had worse survival than those younger than 50 years (hazard ratio, 1.78; 95% confidence interval, 1.64-1.92; P < 0.001). The overall survival of patients who had surgery with radiation was better than those who had surgery alone (hazard ratio, 0.89; 95% confidence interval, 0.83-0.95; P < 0.001). In women with localized disease, surgery was associated with better survival than surgery with radiation (66.4% vs 74.4%, P < 0.00001). CONCLUSIONS: Uterine sarcoma is an aggressive tumor that occurs more in old age and among women of black race. Poor survival was associated with old age, black race, and advanced disease stage. Radiotherapy in patients with localized stage does not improve the survival.

Uterine Sarcomas in Patients Undergoing Surgery for Presumed Leiomyomas: 10 Years' Experience.

STUDY OBJECTIVE: To find the incidence of sarcomas in patients undergoing surgery for presumed leiomyomas. DESIGN: Retrospective study (Canadian Task Force classification II-3). SETTING: Paul's Hospital, Centre for Advanced Laparoscopy and Infertility, Kochi, India. PATIENTS: All women who underwent total laparoscopic hysterectomy (TLH), laparoscopic myomectomy (LM), and hysteroscopic myomectomy (HM) at Paul's Hospital for presumed leiomyomas from January 1, 2004 to December 31, 2014 that were then diagnosed as sarcomas on histopathologic examination. INTERVENTIONS: TLH, LM, and HM. MEASUREMENTS AND MAIN RESULTS: A total of 2678 patients underwent TLH, LM, and HM at Paul's Hospital for presumed leiomyomas from January 1, 2004 to December 31, 2014. Five patients were diagnosed as leiomyosarcoma and 3 patients as endometrial stromal sarcoma on histopathologic examination. Women's ages ranged from 12 to 53 years. Histopathologic diagnosis of leiomyosarcoma was made in 3 patients from the TLH group and 2 patients from the myomectomy group. Two patients from the TLH group and 1 patient from the myomectomy group were diagnosed as endometrial stromal sarcoma on histopathologic examination. The incidence of uterine sarcomas (leiomyosarcoma and endometrial stromal sarcoma) in patients undergoing surgery for presumed leiomyomas was found to be .29% (1 in 335 patients) in our study. CONCLUSION: Over a period of 10 years (2004-2014), the incidence of uterine sarcomas in patients undergoing surgery for presumed leiomyomas was found to be .29% in our study.

Clinical characteristics and management experience of unexpected uterine sarcoma after myomectomy.

OBJECTIVE: To identify the prevalence of unexpected uterine sarcoma after myomectomy for presumed leiomyoma and compare clinical outcomes after primary myomectomy with and without power morcellation. METHODS: In a retrospective study, a review was undertaken of the medical records of patients who had unexpected uterine sarcoma after myomectomy with and without power morcellation at Peking Union Medical College Hospital, Beijing, China, between January 2009 and December 2013. RESULTS: Among 4248 patients who underwent myomectomy for presumed leiomyoma, 9 (0.2%) had unexpected uterine sarcoma (1 [<0.1%] had leiomyosarcoma; 8 [0.2%] endometrial stromal sarcoma). The malignancy was identified in 5 (0.2%) of 3068 women who were treated by laparoscopy with power morcellation and 4 (0.3%) of 1180 who underwent laparotomy (P=0.274). All nine patients were alive after a mean follow-up of 31.2 months in the laparoscopy group and 40.5 months in the laparotomy group. CONCLUSION: The overall incidence of unexpected uterine sarcoma after myomectomy was low. Incidental power morcellation of unexpected uterine sarcoma seemed to cause no increase in sarcoma dissemination.

Retrospective analysis of 80 cases with uterine carcinosarcoma, leiomyosarcoma and endometrial stromal sarcoma in China, 1988-2007.

OBJECTIVE: Uterine sarcomas are rare gynecological malignancies with poor prognosis and high mortality. We provides clinical information of uterine sarcoma patients at Changhai Hospital of Secondary Military Medical University in Shanghai, China, over a 20-year period. DESIGN AND METHODS: Satisfied the criteria for the study, a total of 80 female patients with uterine sarcomas were retrospectively evaluated. Overall survival was analyzed by Kaplan-Meier method. MAIN OUTCOME MEASURES: The following information was extracted from our medical records: age, presentations, blood types, stages, ultrasonographic results, therapies and follow-up. RESULTS: Of the 80 patients, the mean age of onset was 57.3±2.03 years, and the highest frequency occurred in 51-60 age group. Endometrial stromal sarcoma was the most common histological type (47.5%). Even population of these patients presented was with early stage (I&II) and advanced stages (III&IV). Among 79 patients underwent primary surgery, 74 cases was hysterectomy and bilateral salping-ooophorectomy. Equal to disease-specific survival, overall survival rates at 1-, 3- and 5-year were 81.3%, 62.5% and 40% respectively. Age, menopausal status, blood type, stage, and pathologic types were all proved to be correlated with the survival. CONCLUSION: Our retrospective data in part reflect clinical characteristics of uterine sarcoma in China, and form the basis for further concerning researches.

Incidence and survival trends of uncommon corpus uteri malignancies in the Netherlands, 1989-2008.

INTRODUCTION: Corpus uteri cancer is the most common malignancy of the female reproductive tract in industrialized countries, and its incidence is increasing. Although most of these tumors are of the common endometrial type, there are also many uncommon tumors of the corpus uteri. We examined the incidence and survival of patients with uncommon epithelial tumors, carcinosarcomas, and sarcomas of the corpus uteri diagnosed since 1989. METHODS: All common and uncommon malignancies of the corpus uteri registered in the nationwide population-based Netherlands Cancer Registry (NCR) during 1989-2008 were included (n = 30,960). The histological subtypes were described according to the Blaustein classification system. Age-standardized incidence for 1989-2008 was calculated per 1,000,000 person-years (p-y), and relative survival was calculated according to the type of uncommon tumor. RESULTS: The incidence of corpus uteri malignancies increased from 159 to 177 per 1,000,000 p-y, mainly owing to the rise in endometrioid adenocarcinomas from 106 to 144 per 1,000,000 p-y. In contrast, the incidence of uncommon epithelial endometrial carcinomas (UEECs) decreased from 30 to 13 per 1,000,000 p-y, although carcinosarcomas increased slightly from 5.1 to 6.9 per 1,000,000 p-y. Furthermore, a remarkable shift in incidence of endometrial stromal cell sarcomas (ESS) was observed from high-grade ESSs to low-grade ESSs after 2003. Five-year relative survival for patients with UEEC decreased from 72% to 54% and for patients with serous adenocarcinoma from 73% to 51%. Coinciding with an increase in the incidence of common adenocarcinoma of the corpus uteri, there was a decline in uncommon adenocarcinomas and more or less a stable incidence of sarcomas and carcinosarcomas. CONCLUSION: The decrease in UEEC tumors consisted largely of fewer serous carcinomas, possibly and likely reflecting a more precise histopathological classification of villoglandular tumors. Unfortunately, relative survival for patients with UEEC, sarcomas, and carcinosarcomas did not improve over the study period, indicating a need for more research on treatment strategies for this group of patients.

Incidence of uterine leiomyosarcoma and endometrial stromal sarcoma in Nordic countries: results from NORDCAN and NOCCA databases.

OBJECTIVES AND STUDY DESIGN: Uterine sarcomas are an uncommon and heterogeneous group of malignancies. Their etiology is mainly unknown. Here, we analyzed trends in incidence and occupational variation in risk of uterine leiomyosarcomas (LMS) and endometrial stromal sarcomas (ESS) in the Nordic countries aided by NORDCAN and NOCCA (Nordic Occupational Cancer) databases. MAIN OUTCOME MEASURES: Incidence rates per 100,000 and Standardized incidences rates (SIR) obtained from NORDCAN and NOCCA databases. RESULTS: The incidence rates were about 0.3 per 100,000 for ESS and about 0.4 per 100,000 for LMS in Denmark, Finland, Iceland, and Norway. During the study-period (1978-2007), the incidence rates in each country were quite similar and constant. The age-specific incidence of LMS showed a peak around menopause. Significantly increased risk for LMS occurred in shoe and leather workers, farmers and teachers, whereas significantly low risk was detected with packers in the NOCCA data from Finland, Norway, and Sweden. For ESS no occupations showed either increased or decreased incidences. CONCLUSIONS: The incidence trends of LMS and ESS in our study were constant in four Nordic countries over time. The elevated risk for LMS with women exposed to leather work and animal dust indicates further exploration.

Does the FIGO 2009 endometrial cancer staging system more accurately correlate with clinical outcome in different histologies? Revised staging, endometrial cancer, histology.

OBJECTIVE: In 2009, the International Federation of Gynecology and Obstetrics (FIGO) staging system was revised for endometrial cancers. Different histologies were examined in a large population database. The FIGO 1988 and 2009 staging systems were compared for stage at presentation, differences in patient populations, and disease-specific survival (DSS). METHODS/MATERIALS: A total of 10,839 cases from 1998 to 2006 were analyzed from the Surveillance, Epidemiology, and End Results (SEER) Program. Examined histologies included 1377 cases of clear cell carcinoma (CC), 2304 cases of uterine papillary serous carcinoma (PS), 755 cases of carcinosarcoma (CS), and 6403 cases of grade 3 endometrial adenocarcinoma (G3A). The median follow-up was 26 months. For each stage and histology, DSS for patient characteristics was examined. RESULTS: Of the 10,839 women with CC, PS, CS, and G3A had a median age of 67 years. White, black, and other ethnicities composed 87.5%, 12%, and 7% of this group, respectively.A higher percentage of non-G3A histology (CS, PS, and CC) was found in 58% of black women versus 39% of white women. The best to worst 5-year DSS was G3A (76.2%), CC (68.8%), PS (59%), and CS (53.4%). Patients with FIGO IIIC2 disease had inferior survival outcomes in CC (P = 0.0079) and G3A (P = 0.047) compared with FIGO IIIC1 disease, whereas DSS was not significantly different for CS and PS between stages IIIC1 and IIIC2. CONCLUSIONS: These findings describe differences in the DSS of various aggressive histologies of EC, with poorer DSS in PS, CC, or CS histologies. Analysis demonstrated the usefulness of the new FIGO staging for DSS prediction between stages IIIC1 and IIIC2 for CC and G3A, and 2 divisions for stage I rather than three.

Clinical practice guidelines for the management of patients with endometrial cancer in France: recommendations of the Institut National du Cancer and the Société Française d'Oncologie Gynécologique.

INTRODUCTION: Endometrial cancer is the most common gynecological malignancy in France, with more than 6500 new cases in 2010. The French National Cancer Institute has been leading a clinical practice guidelines (CPG) project since 2008. This project involves the development and updating of evidence-based CPG in oncology. OBJECTIVE: To develop CPG for diagnosis, treatment, and follow-up for patients with endometrial cancer. METHODS: The guideline development process is based on systematic literature review and critical appraisal by experts, with feedback from specialists in cancer care delivery. The recommendations are thus based on the best available evidence and expert agreement. RESULTS: Main recommendations include a routine pelvic magnetic resonance imaging in association with magnetic resonance imaging exploration of the para-aortic lymph nodes for locoregional staging, surgical treatment based on total hysterectomy with bilateral salpingo-oophorectomy with or without lymphadenectomy, and clinical examination for the follow-up. The initial laparoscopic surgical approach is recommended for stage I tumors. Lymphadenectomy and postoperative external radiotherapy are recommended for patients with high risk of recurrence but are restricted for patients with low or intermediate risk. If brachytherapy is indicated, it should be given at a high-dose rate rather than a low-dose rate. Routine imaging, biologic tests, and vaginal smears are not indicated for follow-up.

Trends in endometrial cancer incidence rates in the United States, 1999-2006.

BACKGROUND: Risk factors for endometrial cancer, such as hormone replacement therapy (HRT) and obesity, have changed significantly in the last decade. We investigated trends in endometrial cancer histologic subtypes on a national level during 1999-2006. METHODS: Data covering 88% of the U.S. population were from central cancer registries in the National Program of Cancer Registries (NPCR) and Surveillance, Epidemiology, and End Results (SEER) programs that met high-quality United States Cancer Statistics (USCS) criteria. Our analyses included females with microscopically confirmed invasive uterine cancer (n=257,039). Age-adjusted incidence rates and trends for all invasive uterine cancers and by endometrial cancer histologic subtypes (type I and II) were assessed. RESULTS: There were 145,922 cases of type I endometrial cancers and 15,591 cases of type II for 1999-2006. We found that type I endometrial cancers have been increasing, whereas type II endometrial cancers and all invasive uterine cancers have been relatively stable throughout the 1999-2006 period. CONCLUSIONS: During the past decade, the overall burden of uterine cancer has been stable, although there have been changes in underlying histologies (e.g., endometrial). Changes in trends for underlying histologies may be masked when reviewing trends irrespective of histologic subtypes. Our findings suggest the need to examine trends of uterine cancer by histologic subtype in order to better understand the burden of endometrial cancer in relation to these subtypes to help women at increased risk for developing more aggressive types of endometrial cancer (e.g., type II).

Lymphadenectomy in uterine low-grade endometrial stromal sarcoma: an analysis of 19 cases and a literature review.

BACKGROUND: The role of lymphadenectomy in low-grade endometrial stromal sarcoma (ESS) is controversial. The risk of nodal metastases ranges from 0% to 44%, but data are inconclusive. The objective of this study and of the literature review was to investigate the incidence of nodal involvement in macroscopically early-stage tumors. METHODS: All consecutive patients with histologically proven uterine low-grade ESS who underwent surgery in our institution were considered eligible for the analysis. Until July 2006, pelvic systematic lymphadenectomy was performed based on the physician's choice, whereas after July 2006, all women with apparent early-stage tumor underwent systematic pelvic nodes dissection. RESULTS: From January 1985 to March 2009, 64 women were surgically treated and 19 underwent bilateral systematic pelvic lymphadenectomy. The median count of nodes removed was 20 (range, 11-30). Nodal involvement was depicted in 3 of 19 women but only in 1 in 16 (5%) women with apparent early-stage disease. Specifically, we did not find nodal metastases in 9 women with macroscopically early-stage tumors treated after July 2006. According to the literature review, the risk of nodal metastases is 5% (range, 0%-16%) in early-stage disease. CONCLUSIONS: There is no indication to offer a systematic lymphadenectomy in apparent early-stage low-grade ESS according to our analysis and to the literature review. Retroperitoneal surgery should be limited in case of bulky nodal disease or in case of nodal recurrences.

Clinical characteristics of endometrial stromal sarcoma from an academic medical hospital in China.

OBJECTIVES: The purpose of this study was to evaluate the clinical features, treatments, and outcomes of endometrial stromal sarcoma (ESS) in China. METHODS: Seventy consecutive ESS patients were treated at Peking Union Medical College Hospital from 1983 to 2005, and 51 of them completed the treatment and follow-up. The demographic, clinicopathologic, treatment, and survival information was retrospectively reviewed. Data were analyzed using Kaplan-Meier methods and Cox proportional hazards regression. RESULTS: In all, the mean age of the patients was 43.5 years. Irregular vaginal bleeding and uterine enlargement were presented in 71.0% and 65.7% of the cases, respectively. Uterine cavity lesions were found in 17 patients (24.3%). Twenty-six cases (37.2%) were diagnosed preoperatively through diagnostic curettage. Among 51 patients who completed the treatment and follow-up in Peking Union Medical College Hospital, 37 were diagnosed as having low-grade ESS (LGESS) and 14 high-grade ESS, which is now classified as undifferentiated endometrial sarcoma (UES). The median overall survival time was 334 months, and the 5-year survival rate was 87.8%. Twenty-six of 51 patients, including 14 with LGESS and 12 with UES, developed disease recurrence. The tumor's classification, initial surgery, and adjuvant therapy were the factors related to the disease-free survival, whereas only the tumor's classification was associated with the overall survival. CONCLUSIONS: Endometrial stromal sarcoma is a rare kind of uterine malignancy; the possibilities of preoperative diagnosis may be improved by diagnostic curettage. Low-grade ESS and UES represent 2 distinct clinical entities and should be treated as such. The tumor's classification may be the most important prognostic factor.

Cervical sarcomas: an analysis of incidence and outcome.

OBJECTIVE: Cervical sarcomas are exceedingly rare neoplasms associated with a poor prognosis. The objective of this study was to examine the treatment and outcome of women with cervical sarcomas. METHODS: A hospital-based tumor registry was searched to identify all patients with cervical sarcomas treated between 1986 and 2003. The medical records of all patients were reviewed. All pathologic specimens were reviewed by a single pathologist. RESULTS: Among 1583 with cervical malignancies, 8 cervical sarcomas were identified. All patients presented with vaginal bleeding. The lesions were clinically staged as IB1 (2), IB2 (4), IIIA (1), and IIIB (1). Five of the tumors were carcinosarcomas. Other histologies included sarcoma NOS (12.5%), leiomyosarcoma (12.5%), and endometrial stromal sarcoma (12.5%). Initial treatment included surgery in 5 patients, radiation in 2, and chemoradiation in 1. Six patients were treated with curative intent, 5 received adjuvant therapy. While both patients treated palliatively died from progressive disease, the other 6 patients remain alive after a mean follow-up of 2.5 years. Two patients have recurred. One patient underwent a thoracotomy for an isolated pulmonary metastasis and is alive with no evidence of disease. The second patient developed pulmonary metastases and is alive 8 months after recurrence. CONCLUSIONS: Cervical sarcomas are rare neoplasms. Most patients present with vaginal bleeding and a palpable cervical mass. While the optimal management of these tumors is uncertain, aggressive primary therapy can result in prolonged survival and cure.

Surveillance, epidemiology, and end results analysis of 2677 cases of uterine sarcoma 1989-1999.

OBJECTIVES: To determine the association of race with incidence, histology, treatment, and survival in women with uterine sarcoma during the period 1989-1999. METHODS: Uterine sarcomas were defined as leiomyosarcoma, carcinosarcoma, high-grade endometrial stromal sarcoma (HGESS), adenosarcoma, and sarcoma not otherwise specified (NOS). We used cases from Surveillance, Epidemiology, and End Results (SEER) program to compare uterine sarcoma among women >35 years of age. Using data from 1989 to 1999, we compared race-specific age-adjusted incidences, histological distributions, extent of disease at diagnosis, and race-specific survival. RESULTS: During the period of 1989-1999, 2677 women were diagnosed with uterine sarcoma, 2098 (78%) of whom were white and 420 (16%) of whom were black, and 159 (6%) of whom were of other races. The overall age-adjusted incidence for blacks was twice that of whites and more than twice that of women of other races (7/10(5) vs. 3.6/10(5) vs. 2.7/10(5), P < 0.0001). Racial differences in the incidence of uterine sarcoma existed for leiomyosarcoma (1.51/10(5) for blacks vs. 0.91/10(5) for whites, and 0.89 for women of other races, P < 0.01) and carcinosarcoma (4.3/10(5) for blacks, vs. 1.7/10(5) for whites, and 0.99 for women of other races, P < 0.001), but not for other histological types. Blacks with stage II disease were less likely to receive radiation in addition to surgery compared to whites (33% vs. 54%, P < 0.05). Five-year relative survival of patients with disease beyond the uterus was significantly longer for those that received radiation and surgery compared to those that received surgery alone. There were no racial differences in survival for women that received similar therapy. CONCLUSIONS: Adjuvant therapy improved survival for women with stage II-IV disease. Survival of black and white patients who received comparable treatment was similar.