Prognosis of uterine and extrauterine low-grade endometrial stromal sarcoma: an observational cohort study.

OBJECTIVE: Little is known about the survival differences between uterine and extrauterine low-grade endometrial stromal sarcoma (LGESS). Survival outcomes, consisting of disease-free survivals and overall survivals (OS), were compared in these two entities. METHODS: From February 2012 to June 2019, all primary LGESS cases and LGESS cases with first recurrence in the study center were reviewed. The clinicopathological characteristics and survival outcomes of extrauterine and uterine LGESS patients were compared for both primary and recurrent diseases. RESULTS: During the study period, 143 patients with primary LGESS and 56 patients with recurrent LGESS were included and followed up to 1 June 2020, among whom 8 (5.6%) and 10 (17.8%) patients were identified as having extrauterine LGESS. Patients with primary and recurrent extrauterine LGESS had similar clinicopathological characteristics to those of patients with uterine LGESS. In primary or in recurrent LGESS cases, in univariate analysis, patients with uterine and extrauterine LGESS had similar disease-free intervals after the last treatment, and they also had similar OSs after the diagnosis. Ovarian preservation led to significantly increased recurrence for primary LGESS [hazard ratio (HR) 4.9, 95% CI: 2.3-10.1, P <0.001) and repeated recurrence for recurrent LGESS (HR 3.1, 95% CI: 1.3-7.3, P =0.009). Surgical treatment for recurrent LGESS decreased repeated recurrence after the first recurrence (HR 0.2, 95% CI: 0.1-0.7, P =0.006). No factors were found to be associated with the OS of primary or recurrent LGESS. CONCLUSION: The clinical characteristics and survival outcomes of extrauterine LGESS are similar to those of uterine LGESS. Surgery is the treatment of choice for recurrent LGESS. Ovarian preservation is detrimental to disease-free survival but not to OS in both uterine and extrauterine LGESS.

Survival outcomes and prognostic factors of endometrial stromal sarcoma and undifferentiated uterine sarcoma.

PURPOSE: To review the diagnostic and therapeutic procedures of patients diagnosed with Endometrial Stromal Sarcoma (ESS) and Undifferentiated Uterine Sarcoma (USS) at our institution and investigate their clinical outcomes and factors affecting prognosis. METHODS: We retrospectively collected demographic data, preoperative diagnostic methods and therapeutic management of patients treated for ESS and UUS between January 1995 and December 2019 at Vall d'Hebron Barcelona Hospital Campus, Spain. Overall survival and disease-free survival were calculated. Cox proportional-hazards regression models were calculated. RESULTS: Sixty-three patients were included in the study, of which 51(81%) had a diagnosis of ESS and 12(19%) of UUS. Twenty patients (31.7%) were diagnosed after a previous non-oncologic surgery, and 12 of them (60%) suffered from tumor disruption. Cytoreductive procedures were needed in 29 patients (46%), and optimal cytoreduction was achieved in 80.9% of the patients. The median follow-up was 7.6 years (IQR = 0.99-14.31). Five-year overall survival was 57.6% (44.2-68.8) and was significantly better for low-grade ESS (LG-ESS) patients (p < 0.01). Five-year disease-free survival was 57.1% (42.8-69.1) and was also significantly higher in LG-ESS cohort (p = 0.03). After multivariate analysis histological type, age, FIGO stage, optimal surgery and mitotic index were found significantly correlated with survival. For high-grade EES (HG-ESS) and USS patients adjuvant radiotherapy also correlated with improved survival. CONCLUSION: Overall survival and disease-free survival are significantly better in patients with LG-ESS cohort. HG-ESS and UUS show similar survival outcomes. Age, FIGO stage, optimal surgery and histological type were significantly correlated with survival in the global cohort, whilst adjuvant radiotherapy correlated with improved survival in HG-ESS and UUS patients.

Effect of bilateral salpingo-oophorectomy on the overall survival of premenopausal patients with stage I low-grade endometrial stromal sarcoma; a National Cancer Database analysis.

OBJECTIVES: Investigate the prevalence of bilateral salpingo-oophorectomy (BSO) for women ≤50 years with early stage low-grade endometrial stromal sarcoma (LGESS) and its impact on overall survival (OS). METHODS: Women ≤50 years, diagnosed with stage I LGESS and managed with hysterectomy between 2004 and 2015 were identified from the National Cancer Database. Patient demographics were recorded and compared with the chi-square test. OS for patients diagnosed between 2004 and 2014 with at least one month of follow-up was assessed using Kaplan-Meier curves, and compared with the log-rank test. RESULTS: A total 743 patients with a median age of 44 years met the inclusion criteria. Use of radiatiotherapy (9%), chemotherapy (0.8%) and hormonal therapy (11%) was infrequent. BSO was performed in 541 (72.8%) patients. Patients who had ovarian preservation (OP) were younger (median age 43 vs 45 years, p < 0.001), less likely to have comorbidities (6.9% vs 12.4%, p = 0.034), or undergo LND (30.7% vs 44.4%, p = 0.001). There were no differences between the two groups in terms of substage or patient race. Five year OS rates for patients who did (n = 490) and did not (n = 191) undergo BSO were 96.2% and 97.1% and there was no difference in OS, p = 0.50. Even after controlling for presence of comorbidities performance of BSO was not associated with better survival (HR: 1.28, 95% CI: 0.51, 3.19). CONCLUSIONS: Ovarian function was preserved in approximately one third of women ≤50 years with stage I LGESS with no clear detriment to overall survival. As BSO is associated with long term health effects in this patient population OP could be considered in selected women with stage I LGESS.

High-grade transformation of low-grade endometrial stromal sarcomas lacking YWHAE and BCOR genetic abnormalities.

High-grade histologic transformation of low-grade endometrial stromal sarcoma (LGESS) is rare. Here, we describe the clinicopathologic features and gene fusion status of 12 cases (11 primary uterine corpus and 1 primary vaginal), 11 diagnosed prospectively from 2016, and 1 retrospectively collected. Targeted RNA sequencing and/or fluorescence in situ hybridization was employed in all cases. High-grade transformation was seen at the time of initial diagnosis in eight patients and at the time of recurrence in four patients, 4-11 years after initial diagnosis of LGESS. High-grade morphology consisted of generally uniform population of round to epithelioid cells with enlarged nuclei one to two times larger than a lymphocyte, visible nucleoli, and increased mitotic index (range, 6-30; median, 16 per 10 high-power fields); there was often an associated sclerotic and/or myxoid stroma. Estrogen receptor, progesterone receptor, and CD10 expression was absent or significantly decreased (compared with the low-grade component) in the high-grade foci of five tumors. One tumor demonstrated positive (diffuse and strong) cyclin D1 and BCOR staining. p53 staining was wild type in both components of all eight tumors tested. JAZF1-SUZ12 (n = 6), JAZF1-PHF1 (n = 3), EPC1-PHF1, (n = 1), or BRD8-PHF1 (n = 1) fusions were detected in 11 tumors; no fusions were found in one by targeted RNA sequencing. Patients presented with FIGO stages I (n = 4), II (n = 4), III (n = 1), and IV disease (n = 2). Median overall survival calculated from the time of histologic transformation was 22 months (range, 8 months to 8 years) with five patients who died of disease 8-18 months after transformation. High-grade transformation may occur in LGESS with JAZF1 and PHF1 rearrangements at the time of or years after initial diagnosis. Such high-grade transformation is characterized by nuclear enlargement, prominent nucleoli, and increased mitotic index compared with typical LGESS. Histologic high-grade transformation may herald aggressive behavior.

[Clinicopathological study of BCOR rearrangement in high grade endometrial stromal sarcoma].

Objective: To investigate clinicopathological, cytogenetic features and differential diagnoses of high grade endometrial stromal sarcoma (HGESS) with BCOR gene rearrangement. Methods: Five cases of HGESS with BCOR rearrangement were collected from consultant files (2016-2018) at Fudan University Shanghai Cancer Center. Interphase FISH was performed using a dual color break-apart probe. The clinical data, histologic features and immunohistochemical findings were reviewed. Results: All 5 cases occurred in adult women with a median age of 48 (range, 45-55) years. Abdominal pain and abnormal vaginal bleeding were the most common symptoms. Microscopically, the tumors showed mainly tongue-like and/or intersecting myometrial invasion. Stromal myxoid matrix and/or collagen plaques were prominent in all the cases. Most tumors consisted of uniform, haphazard fascicles of short spindle cells with mild to moderate nuclear atypia. Mitotic figures and necrosis were easily identified. Significant nuclear pleomorphism was not seen. Most tumors were rich in thick-walled small vessels. Prominent perivascular tumor cell whorling seen in conventional low-grade endometrial stromal sarcoma was not seen. All tumors expressed CD10 with only focal or absent desmin, SMA and/or h-caldesmon staining. ER or PR expression was seen in 4 tumors and 1 tumor showed both marker expression. Diffuse cyclin D1 was present in 2 tumors. BCOR immunoreactivity was present with strong staining in 3 cases and moderate staining in 1 case respectively. Ki-67 index ranged from 10% to 30%. Fluorescence in situ hybridization confirmed chromosomal aberration of BCOR gene in all tumors, that were previously diagnosed as myxoid leiomyosarcoma (2 cases), spindle cell uterine sarcoma (2 cases) and low-grade endometrial stromal sarcoma (1 case). Limited follow-up information revealed that 3/5 patients developed tumor recurrence, metastasis or death within one year. Conclusion: BCOR rearranged HGESS has distinct morphological features and aggressive clinical behavior. In the presence of significant overlapping morphologic features between BCOR rearranged HGESS and other myxoid uterine mesenchymal tumors, especially myxoid leiomyosarcoma, molecular analysis is essential for accurate diagnoses.

Hormone therapy following surgery in low-grade endometrial stromal sarcoma: Is it related to a decrease in recurrence rate?

BACKGROUND: Low-grade endometrial stromal sarcoma (LGESS) is, in most cases, a slow-growing malignancy; however, it is related with high recurrence rates. The aim of this study is to determine which factors may be associated with the recurrence rate of LGESS. METHODS: The clinicopathological features and treatment options in 37 patients with LGESS were evaluated. RESULTS: All patients underwent the hysterectomy and bilateral salpingo-oophorectomy. Additionally, lymphadenectomy was performed in 56.8% (n = 21) of the patients. Among the patients who underwent lymphadenectomy, 14.3% (n = 3) had lymph node metastasis. The disease was limited to the uterus in 75.7% of patients. Treatment following surgery was radiotherapy in three patients, chemotherapy in seven patients, hormone therapy in 12 patients, and chemotherapy plus hormone therapy in one patient. Megestrol acetate was used in all patients who received hormone therapy. Median follow-up time was 96 months. The 5-year disease-free survival and disease-specific survival were 72% and 97%, respectively. The recurrence rate was 27%. Only hormone therapy following surgery was significantly associated with a lower recurrence rate, even in patients with stage 1 disease. None of the patients treated with hormone therapy following surgery had recurrence, whereas recurrence occurred in 38.5% of the patients who underwent surgery only (p = 0.039). CONCLUSION: Hormone therapy after surgery should be considered a viable option for decreasing the LGESS recurrence rate, regardless of the disease stage.

Endometrial stromal sarcoma-A retropsective analysis of factors affecting recurrence.

OBJECTIVE: To assess the factors associated with disease free survival and overall survival in endometrial stromal sarcoma (ESS). METHODS: This was a single institution retrospective analysis done at Amrita Institute of Medical Sciences. Records from January 2005 to October 2016 were analysed and 42 patients with ESS were identified. Clinicopathological, surgical management, adjuvant treatment and outcome data were collected. Disease free and overall survivals were analysed using Kaplan-Meier method and compared using log rank test. RESULTS: Out of 38 patients included in analysis 28 (73.7%) had low grade ESS (LGESS) and 10 (26.3%) had high grade ESS (HGESS). The median follow up period was 28 months (range 1-110 months). The 5year disease free survival (DFS) and overall survival (OS) for the entire cohort were 62.9 and 89.1% respectively. High grade ESS was significantly associated with recurrence on univariate analysis (p=0.045). Complete staging surgery in both HGESS and LGESS and adjuvant treatment in HGESS and advanced stage LGESS (cohort 1) were associated with improved 5-year DFS of 90.9% (p<0.001). Completion staging surgery in patients with initial incomplete surgery (cohort 3) was associated with improved 5-year DFS of 100% (p<0.001). Complete Staging surgery but no adjuvant treatment in HGESS and advanced stage LGESS (cohort 2) was associated with significantly poor 2-year DFS of 20% (5-year DFS rate 0%; as all patients recurred within 27 months) (P<0.001). Significantly reduced 5-year DFS rate of 25% (p<0.001) was also seen in cases where initial incomplete surgery was not followed by complete staging and adjuvant treatment (cohort 4). Adjuvant treatment in HGESS was associated with improved 5-year DFS of 66.7% (p=0.043). Resection of recurrent lesions were associated with improved mean survival of 41.2 months. CONCLUSION: Incomplete surgery and no adjuvant treatment in ESS are associated with poor DFS. Complete staging surgery is associated with improved DFS. Resection of recurrent disease is associated with survival advantage.

Low-grade and high-grade endometrial stromal sarcoma: A National Cancer Database study.

OBJECTIVE: To provide refined prognostic information from large cohorts of women with low-grade or high-grade endometrial stromal sarcoma (ESS). METHODS: We performed an observational retrospective cohort analysis of women diagnosed with low-grade or high-grade ESS from the 1998-2013 National Cancer Database. Kaplan-Meier and multivariable accelerated failure time survival analyses were performed to identify prognostic factors after multiple imputation of missing data. Recursive partitioning methods were used to rank prognostic factors in high-grade ESS. Matched cohort analyses were performed to hypothesis-test effects of adjuvant treatments. RESULTS: We identified 2414 and 1383 women with low-grade or high-grade ESS, respectively. Women with high-grade ESS had markedly decreased survival compared to women with low-grade ESS (five-year survival (95% CI): 32.6 (30.1-35.3%) versus 90.5% (89.3-91.8%), P<0.001). Among women with high-grade ESS, median survival (95% CI) was only 19.9 (17.1-22.1) months. Increased age and tumor size were associated with decreased survival in low-grade ESS. In high-grade ESS, additional negative prognostic factors were distant or nodal metastasis, omission of lymphadenectomy, and pathologically-positive surgical margins (all P<0.001). Use of adjuvant chemotherapy (time ratio (TR) (95% CI): 1.36 (1.17-1.58), P<0.001) and radiotherapy (TR (95% CI): 1.57 (1.32-1.87), P<0.001) were associated with increased survival for high-grade ESS. CONCLUSION: The contrasting excellent versus poor prognosis of low-grade versus high-grade ESS, respectively, was confirmed. The best treatment of high-grade ESS is early and complete surgical resection including lymphadenectomy. Adjuvant chemotherapy and radiotherapy may increase survival of women with high-grade ESS.

Neuron navigator-2 and cyclin D2 are new candidate prognostic markers in uterine sarcoma.

The objective of this study was to validate the diagnostic and clinical role of four protein products of genes previously found to be differentially expressed in uterine low-grade endometrial stromal sarcoma (LG-ESS) compared to uterine leiomyosarcoma (LMS). Protein expression by immunohistochemistry of transgelin (TGLN), neuron navigator-2 (NAV2), fatty acid binding protein-3 (FABP3), and cyclin D2 (CCND2) was analyzed in 305 uterine sarcomas (231 LMS, 74 LG-ESS). Expression was analyzed for association with clinicopathologic parameters and survival. TGLN (p < 0.001), NAV2 (p < 0.001), and FABP3 (p = 0.005) were overexpressed in LMS compared to LG-ESS, whereas nuclear CCND2 (p < 0.001) was overexpressed in LG-ESS. NAV2 expression was associated with shorter overall survival in patients with LMS (p = 0.037), whereas nuclear CCND2 expression in LG-ESS was significantly related to longer survival (p = 0.012) in univariate analysis. Nuclear CCND2 expression was an independent prognosticator in Cox multivariate analysis (p = 0.023). In conclusion, TGLN, FABP3, NAV2, and nuclear CCND2 aid in differentiating LG-ESS from LMS. NAV2 and CCND2 are novel candidate prognostic markers in LMS and LG-ESS, respectively.

Outcome and prognosis in uterine sarcoma and malignant mixed Mullerian tumor.

PURPOSE: There is low evidence regarding the optimal treatment in patients with uterine sarcomas and malignant mixed Mullerian tumors (MMMTs). This study provides an overview of experience at our center with patients diagnosed with uterine sarcoma and MMMT, in relation to the clinical management and outcome. METHODS: The medical records for 143 patients with low-grade endometrial stromal sarcoma (ESS), leiomyosarcoma (LMS), and high-grade (undifferentiated) endometrial sarcoma (UES) and MMMT were reviewed. All available clinical and pathological data were collected and analyzed. Putative prognostic factors were entered into a multivariate analysis using a Cox proportional hazards ratio model, and survival data were calculated. RESULTS: The 5-year overall survival rates were significantly different between patients with ESS, LMS, and UES and MMMT (86 vs. 40 vs. 57 vs. 45 %; P < 0.001). The multivariate analysis showed that the patients' age, higher FIGO stage (III-IV), a history of smoking, prior pelvic radiation, diabetes, and residual tumor after surgery were associated with a poorer overall survival. Histological subtypes of LMS (HR 4.68; 95 % CI 1.35-16.17), UES (HR 1.21; 95 % CI 0.26-5.77) and MMMT (HR 1.63; 95 % CI 0.42-6.43) were also associated with a poorer overall survival than ESS (P = 0.008). Adjuvant therapies showed no associations with overall survival. CONCLUSIONS: Adjuvant therapy has so far not shown any overall survival benefit, and the focus is therefore on primary surgery. In future studies, the entities should be investigated separately in relation to prognostic factors and effective therapeutic management.

Influence of different treatment modalities on survival of patients with low-grade endometrial stromal sarcoma: A retrospective cohort study.

BACKGROUND: To assess the efficacy of different treatment modalities on the outcome of patients with low-grade endometrial stromal sarcoma (LG-ESS). METHODS: Patients with LG-ESS who received hysterectomy from March 1991 to December 2013 were retrospectively analyzed. The associations between clinicopathologic variables and disease free survival (DFS) were evaluated. RESULTS: One hundred and fourteen patients met the eligibility requirements. All patients received hysterectomy as the main treatment, 17.5% (20/114) of patients received ovarian preservation, and 62.3% (71/114) of patients received lymphadenectomy. Fifty-six patients received chemotherapy, 36 patients received radiotherapy, and 11 patients received endocrine therapy. The median follow-up duration was 40 months. The 5-year and 10-year DFS rates were 91.8% and 77.4%, respectively. The 5-year and 10-year overall survival rates were 96.7% and 96.7%, respectively. Univariate analyses showed that there were no risk factors associated with DFS. Lymphadenectomy, lymph node status, ovarian preservation, chemotherapy, radiotherapy, and endocrine therapy had no significant effect on DFS. CONCLUSIONS: Hysterectomy has been the mainstay of treatment for LG-ESS. The optimal treatment strategy in LG-ESS remains to be determined.

Long-term survival of patients with recurrent endometrial stromal sarcoma: a multicenter, observational study.

OBJECTIVE: The aim of this study was to evaluate the clinical behavior and management outcome of recurrent endometrial stromal sarcoma (ESS). METHODS: A retrospective review of charts of 10 patients with recurrent ESS was performed and relapse-free interval, relapse site, treatment, response to treatment, duration of follow-up and clinical outcome extracted. Survival outcome measures used were post-relapse survival which was defined as the time from first evidence of relapse to death from any cause. Living patients were censored at the date of last follow-up. RESULTS: The median age and median relapse-free interval at the time of initial relapse were 51.5 years and 66.5 months, respectively. The number of relapses ranged from one to five. Sixteen surgical procedures for recurrent disease included nine (56.0%) complete resections. There was no statistically significant difference between initial recurrent tumors and second/subsequent recurrent tumors in the rate of complete surgery (44.4% vs. 71.4%, respectively, p=0.36). Of the eleven evaluable occasions when hormonal therapy was used for recurrent disease, disease control was achieved in eight (72.7%). There was no difference between initial recurrent tumors and second/subsequent recurrent tumors in disease control rate by hormonal therapy (85.7% vs. 50.0%, respectively, p=0.49). The 10-year post-relapse survival rate was 90.0% and the overall median post-relapse survival 119 months (range, 7 to 216 months). CONCLUSION: Post-relapse survival of patients with ESS can be expected to be >10 years when treated by repeated surgical resection and hormonal therapy or both.

Prognostic factors, treatment and outcome in a Turkish population with endometrial stromal sarcoma.

PURPOSE: To analyze treatment modalities and prognostic factors in patients with Stage I-II endometrial stromal sarcoma (ESS). MATERIALS AND METHODS: Twenty four patients (nineteen with low-grade ESS [LGESS] and five with high-grade ESS [HGESS]) were assessed retrospectively in terms of general characteristics, prognostic factors, treatment methods and survival. RESULTS: Twenty patients were at Stage I and three were at Stage II. The stage of one patient could not be determined. With respect to age and comorbidity, no statistically significant difference was found among disease-free survival (DFS) (p=0.990; p=0.995). However, DFS was significantly shorter in Stage II than Stage I patients (p=0.002). It was also significantly shorter in HGESS patients than in LGESS patients (p=0.000). There was no statistically significant differences among the overall survival (OVS) times of patients with respect to age at diagnosis and comorbid disease (p=0.905; p=0.979) but OVS was significantly shorter in patients with HGESS (p=0.00) and Stage II disease (p=0.001). No statistically significant difference was found with respect to OVS between patients who received radiotherapy (RT) and those who did not receive RT (p=0.055). It was not statistically possible to include other treatment modalities in the analysis because of the small sample size. CONCLUSIONS: Grade and stage of a tumour were found to be the most important prognostic factors. It was not possible to determine the optimal surgical method and the effect of adjuvant treatment since the number of cases was insufficient.

Entropy-based adaptive nuclear texture features are independent prognostic markers in a total population of uterine sarcomas.

Nuclear texture analysis measures the spatial arrangement of the pixel gray levels in a digitized microscopic nuclear image and is a promising quantitative tool for prognosis of cancer. The aim of this study was to evaluate the prognostic value of entropy-based adaptive nuclear texture features in a total population of 354 uterine sarcomas. Isolated nuclei (monolayers) were prepared from 50 µm tissue sections and stained with Feulgen-Schiff. Local gray level entropy was measured within small windows of each nuclear image and stored in gray level entropy matrices, and two superior adaptive texture features were calculated from each matrix. The 5-year crude survival was significantly higher (P < 0.001) for patients with high texture feature values (72%) than for patients with low feature values (36%). When combining DNA ploidy classification (diploid/nondiploid) and texture (high/low feature value), the patients could be stratified into three risk groups with 5-year crude survival of 77, 57, and 34% (Hazard Ratios (HR) of 1, 2.3, and 4.1, P < 0.001). Entropy-based adaptive nuclear texture was an independent prognostic marker for crude survival in multivariate analysis including relevant clinicopathological features (HR = 2.1, P = 0.001), and should therefore be considered as a potential prognostic marker in uterine sarcomas.

Undifferentiated uterine sarcoma: a rare, not well known and aggressive disease: report of 13 cases.

PURPOSE: Undifferentiated uterine sarcomas (UUS) are rare and aggressive tumor with scarce data on the outcome and best treatment. We aimed to describe the behavior among patients with UUS at our institution. MATERIALS AND METHODS: Thirteen patients with UUS treated in our centre from 1979 to 2010 were analyzed. STATISTICS: descriptive analysis for frequencies and Kaplan-Meier actuarial method for overall survival (OS). RESULTS: Patients mean age was 66 years. Three had FIGO 2009 stage IA, five IB, two IIB, and three IVB. Ten patients underwent surgery and eight received postoperative radiotherapy. Three patients received adjuvant chemotherapy. The median follow-up was 16 months (2-276 months). Stage I patients developed two local relapses and three distant metastases (DM). DM was also observed in stage II patients and in 61.5 % of the entire series. Fifty percent of patients receiving radiotherapy remain alive without relapse. The median OS was 16 months, being 17 months for stage I and 9 for the remainder. CONCLUSIONS: Poor outcome of UUS was associated with a high incidence of DM. Stage I had the best outcome. Radiotherapy seems to have benefited patients, with 100 % of local control and 50 % of long-term survivors. The high incidence of metastasis suggests the need for more accurate initial assessment.

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.

Endometrial stromal sarcomas: immunoprofile with emphasis on HMB45 reactivity.

OBJECTIVES: We describe the morphologic and immunohistochemical features of 17 endometrial stromal neoplasms, 16 sarcomas, and one stromal nodule. METHODS: We reviewed 35 cases interpreted as endometrial stromal neoplasms, but 17 high-grade endometrial stromal sarcomas (ESS) and one case of mixed endometrial sarcoma and leiomyosarcoma were excluded from the study. Data from the Surveillance Epidemiology and End Results program on low- and high-grade ESS for 1973 through 2003 were obtained. RESULTS: One uterine primary ESS had collections of clear cells (20%), while a metastatic ESS contained predominantly clear cells (90%). CD10 (88.2%) and smooth muscle actin (70.5%) were the most common positive immunohistochemical markers. The latter marker was located in the cytoplasm in 47% of the ESS and in the nucleus in 23.5%, a previously unreported feature. HMB45 was detected in 23.5% of the ESS, which contrasts with the 2% reported by other authors. CONCLUSIONS: The presence of clear cells and HMB45 reactivity does not justify the term perivascular epithelioid cell tumors for these neoplasms. Two of 17 patients with ESS died of metastatic disease. However, among 274 cases of ESS (all stages included) collected by the Surveillance Epidemiology and End Results Program of the National Cancer Institute during a 30-year period, the 10-year survival rate was 94%.

Primary endometrioid stromal sarcoma of the ovary: a clinicopathologic study of 27 cases with morphologic and behavioral features similar to those of uterine low-grade endometrial stromal sarcoma.

Twenty-seven endometrioid stromal sarcomas of the ovary from patients 38 to 76 (mean 56) years of age are reported. The tumors were unilateral in 20 cases and bilateral in 7. They were solid (9), solid and cystic (9), or predominantly cystic (6) when this information was known and ranged from 1 to 20 (mean 9.5) cm. The solid areas typically had a tan-yellow cut surface, with areas of hemorrhage and/or necrosis noted in 6; however, in addition, blood was often present in the cyst lumens. On microscopic examination, the predominant and frequently exclusive pattern was a diffuse growth of small cells with interspersed arterioles, the latter appearing round to elongated. A fibromatous pattern was present in 14 of the tumors but was extensive in only 3. A vague nodular growth was observed in 10 tumors but was never striking; a storiform growth was seen in 2 tumors, being conspicuous in 1. Hyaline plaques were present in 10 tumors but were striking in only 2. Sex cord-like or smooth muscle differentiation was seen in 7 and 6 tumors, respectively, being striking in 2 and 3 of them. Foam cells were present in 6 tumors. The tumors showed minimal cytologic atypia. The mitotic index ranged from <1 to 17/10 high-power fields (HPF), being <1/10 HPF in 12, 1 to 5/10 HPF in 9, 6 to 10/10 HPF in 2, and >10/10 HPF in 4 tumors. Infarct-type necrosis was noted in 12 tumors. Hemorrhage, typically recent, was seen in 20 cases, being conspicuous in 5. Ovarian endometriosis was intimately associated with the tumor in 16 cases. Seven patients had stage I tumors, 5 stage II, 13 stage III, and 2 stage IV. Follow-up information was available for 21 patients; 10 were alive and free of disease from 4 to 21 years postoperatively (follow-up being ≥ 11 y in 5); 6 were alive with disease from 1 to 22 years postoperatively; 5 patients are known to have died of disease, with the interval being unknown in 1, and 2, 4, 13, and 17 years in the others. Follow-up information was unavailable in the remaining 6 patients. These findings indicate that these tumors, as in the uterus, often have an indolent course with a better prognosis than other ovarian sarcomas, indicating the importance of correct diagnosis. The differential diagnosis of these neoplasms is in the first instance with a metastasis from the uterus; knowledge of the status of the uterus is paramount in this distinction. Associated ovarian endometriosis suggests a primary tumor. When a primary ovarian origin is determined, the differential diagnosis is most often with a sex cord-stromal tumor, particularly a granulosa cell tumor because of a diffuse growth of cells with scant cytoplasm.