High-grade Endometrial Stromal Sarcoma Research Articles

Characteristics of patients with endometrial stromal sarcoma

5145 Background: Endometrial stromal sarcoma (ESS) is by definition a low grade sarcoma, accounting for less than 1% of all tumor malignancies. The prevalence is approximately 2 per million women. By comparison, the prevalence of endometrial cancer is 700 per million women. Because of its rarity, the natural history of the disease and the optimal therapy have not been well established. Methods: After IRB approval, the charts of 78 patients diagnosed for the first time with ESS and treated at the University of Texas MDACC were reviewed, with emphasis on patient demographics, therapies, recurrence, and survival. Patients with stromal nodule, high grade ESS (undifferentiated sarcoma), or other sarcomas were excluded. Results: The median age of 78 patients was 43 (range 20–78), 85% were Caucasian, and 37% had received exogenous hormones before diagnosis. Endometrial FIGO stage was I in 52%, II in 6%, III in 30%, and IV in 12%. The primary site was uterus in 87% and extra-uterine in 13%. The median parity was 2. Among 68 patients, 78% were postmenopausal. The two-year recurrence rate was 25% and the five year recurrence rate was 51%. The five-year survival rate was 81%. Conclusions: Our study represents 36 years of collected data from MDACC. The demographic data has been recorded and tabled. The median age of onset is younger than the one of endometrial cancer, but interestingly about 75% are postmenopausal at diagnosis. The prognosis is usually good. Various treatments including hormonal therapy, chemotherapy, radiation therapy, and hysterectomy with oophorectomy have been used, and we plan to study their impact on this disease, to present at the annual meeting. No significant financial relationships to disclose.

Surveillance, Epidemiology, and End Results analysis of 2677 cases of uterine sarcoma 1989–1999

Objective. 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.

A Case of Low-Grade Endometrial Stromal Sarcoma of the Uterus

Uterine sarcomas are rare tumors of mesodermal origin. They constitute 2-6% of uterine malignancies. The three most common histologic variants of uterine sarcoma are leiomyosarcoma(LMS), malignant mixed mullerian tumors(MMMTs) of both homologous and heterologous type, and endometrial stromal sarcoma(ESS). Endometrial stromal tumors are divided into three types on the basis of mitotic activity, vascular invasion, and observed differences in prognosis: 1) endometrial stromal nodule, 2) low-grade endometrial stromal sarcoma, 3) high-grade or undifferentiated endemetrial stromal sarcoma. Lower-grade ESS is distinguished from high-grade ESS microscopically by a mitotic rate of less than 10 MF(mitotic figure)/10 HPF as well as clinically by a more protracted course. We have experienced a case of low grade endometrial stromal sarcoma of the uterus in a 39-years-old woman, which is presented with a review of literatures.

High-grade endometrial stromal sarcoma after treatment with tamoxifen in a patient treated for breast cancer

Tamoxifen has been widely used in breast cancer treatment. In recent years, the occurrence of uterine malignancies in patients receiving long-term tamoxifen therapy has attracted attention. Most of these malignancies are endometrial adenocarcinomas, but low-grade endometrial stromal sarcomas have occasionally been reported. Here we report a woman who developed a high-grade endometrial stromal sarcoma after receiving postmastectomy tamoxifen therapy. The patient underwent a left mastectomy at age 45 and subsequently received oral tamoxifen for 3 years. At age 51, she was diagnosed with endometrial stromal sarcoma, for which a radical hysterectomy was performed. High-grade endometrial stromal sarcoma was diagnosed by postoperative histologic examination. Immunostaining for the estrogen receptor was negative in sarcoma cells, but positive in the residual endometrial epithelium and the nucleus of adjacent stromal cells within the tumor. The patient has now survived disease-free for 37 months after surgery.

The proto-oncogene c-kit is expressed in leiomyosarcomas of the uterus

Objective The proto-oncogene c-kit encodes for a 145-kDa transmembrane tyrosine kinase receptor. Interaction with its ligand, stem cell factor, is essential in the development of hematopoietic stem cells, mast cells, gametocytes, melanocytes, and interstitial cells of Cajal. C-kit expression has been identified in a number of different neoplasms that includes mastocytosis/mast cell leukemia, acute myeloblastic leukemia, seminoma/dysgerminoma, and gastrointestinal stromal tumors. This study examines c-kit expression in uterine endometrial stromal sarcomas, leiomyomas, and leiomyosarcomas using immunohistochemistry. Methods Archival tissue from 38 patients with the uterine mesenchymal tumors (16 leiomyosarcomas, 8 leiomyomas, 11 low-grade endometrial stromal sarcomas, and 3 high-grade endometrial stromal sarcomas) was stained with polyclonal antibody for c-kit. Modified avidin biotin (ABC) immunoperoxidase method was employed for antibody detection. Individual tumors were considered positive if more than 10% of the cells comprising the neoplasm displayed immunoreactive staining. Staining intensity was graded 1+ to 3+ and distribution graded as focal (10–30% of the cells), intermediate (30–60% of the cells), or diffuse (>60% of the cells). Results C-kit was positive in 12 (75%) of the 16 leiomyosarcomas. The staining was 3+ and diffuse in the majority of the positive tumors. C-kit expression was not detected in any of the 8 leiomyomas. Two of the 3 high-grade endometrial stromal sarcomas displayed c-kit positivity. Staining was diffuse and 3+ in both of these tumors. Expression of c-kit was observed in only 3 of the 11 low-grade endometrial stromal sarcomas. Conclusions C-kit is expressed in uterine leiomyosarcomas and endometrial stromal sarcomas. Adjunctive diagnostic studies using c-kit may be useful in distinguishing leiomyosarcomas from benign leiomyomas in uterine tumors that offer uncharacteristic features. Furthermore, studies should investigate the prospect of treating these malignant tumors with tyrosine kinase inhibitors.

Lack of expression of c-kit protein (CD117) in mesenchymal tumors of the uterus and ovary.

c-kit is a proto-oncogene that codes for a transmembrane tyrosine kinase receptor (CD117). The gene product KIT is constitutively overexpressed in mastocytosis and gastrointestinal stromal tumors. Recently the use of the tyrosine kinase inhibitors, such as STI-571, has resulted in the successful treatment of bcr-abl-positive leukemias and gastrointestinal stromal tumors. In gastrointestinal stromal tumors, immunostaining for c-kit is diffusely positive. Because the expression of c-kit in mesenchymal tumors of the uterus and ovary has not been previously studied, we evaluated its expression in 38 of these tumors by immunohistochemistry. The number of positive labeled/total tumors were as follows: 0/8 malignant mullerian mixed tumors, 4/7 ovarian fibrosarcomas, 0/1 clear-cell ovarian sarcoma, 0/4 uterine leiomyosarcomas, 1/10 low-grade endometrial stromal sarcomas, 0/2 high-grade endometrial stromal sarcomas, and 3/6 endometrial stromal nodules. In all positive cases, no more than 5% of the cells were labeled. In conclusion, unlike gastrointestinal stromal tumors, mesenchymal tumors of the uterus and ovary rarely express c-kit. Therefore, it is unlikely that patients with these tumors will benefit from treatment with the currently available tyrosine kinase inhibitors.

CT and MR Imaging Findings of Endometrial Stromal Sarcomas

Purpose: To evaluate the imaging findings of endometrial stromal sarcoma (ESS) according to histopathologic grade. Materials and Methods: Six patients with pathologically proven ESS were included in this study. The histopathologic diagnosis was low-grade ESS for three patients and high-grade ESS for the three others. Preoperative CT or MR images were evaluated in terms of tumor size, location, growth pattern, the presence of hemorrhage or necrosis, status of the endometrial cavity, and invasion of surrounding structures. The imaging features of ESSs, which varied according to their histopathologic grade, were compared. Results: The mean maximal diameter of low-and high-grade ESSs was 6 cm and 11.2 cm, respectively. All three low-grade ESSs were located mainly in the myometrium, but two high-grade ESSs were situated in the endometrial cavity and associated with focal tumor extension into the myometrium. One high-grade ESS had completely replaced the uterus. Low-grade ESSs were relatively well-defined, but high-grade ESSs had an irregular and lobulated margin. Intratumoral hemorrhage and necrosis were, respectively, found in two and three high-grade ESSs. Widening of the endometrial cavity was noted in all three high-grade ESSs, and lymph node metastasis had occurred in one. Conclusion: The imaging findings of ESS vary from a well-defined intramural mass to a bulky infiltrating mass, and depend on their histopathologic grade.

Low-Grade Endometrial Stromal Sarcoma Recurring After 9 Years

A 61-year-old Japanese woman had recurrent low-grade endometrial stromal sarcoma (ESS) after primary treatment 9 years earlier. Initial and recurrent tumors showed the same configuration that the polycystic part showed in the solid tumor ultrasonographically. The central part of both the initial and recurrent tumors showed typical histologic findings, but bizarre cells were detected in the peripheral layer of the recurrent tumor. They resembled glandular cells, Comet cells of high-grade ESS, fibroblasts, decidual cells, and predecidual endometrial stromal cells at the middle to late secretory phases. The change and differentiation of low-grade ESS by therapy and environment seem to be the same as the differentiation in mesenchymal stem cells. We speculate that recurrence and prognosis of low-grade ESS are related by extrauterine development but not by mitotic activity or DNA content.

Low-Grade Endometrial Stromal Sarcoma Recurring After 9 Years

A 61-year-old Japanese woman had recurrent low-grade endometrial stromal sarcoma (ESS) after primary treatment 9 years earlier. Initial and recurrent tumors showed the same configuration that the polycystic part showed in the solid tumor ultrasonographically. The central part of both the initial and recurrent tumors showed typical histologic findings, but bizarre cells were detected in the peripheral layer of the recurrent tumor. They resembled glandular cells, Comet cells of high-grade ESS, fibroblasts, decidual cells, and predecidual endometrial stromal cells at the middle to late secretory phases. The change and differentiation of low-grade ESS by therapy and environment seem to be the same as the differentiation in mesenchymal stem cells. We speculate that recurrence and prognosis of low-grade ESS are related by extrauterine development but not by mitotic activity or DNA content.

The Clinicopathologic Study of Endometrial Stromal Sarcoma

Objective: The behavior of endometrial stromal sarcoma (ESS) is still not clear. We report our results about clinical and pathologic features of endometrial stromal sarcoma to understand their clinical behavior. Materials and Methods: 11 patients who were diagnosed as ESS at Asan Medical Center from Jun., 1989 to Oct., 2001 were included in this study. The FIGO classification for endometrial stromal sarcoma was used in this study. All the clinical data were obtained retrospectively from medical records. Results: The median follow-up time was 21.3 months (range; 2 to 61 months). The median duration of disease free survival was 16.7 months (ranges : 2-61 months). The mean age of the patients was 46.7 years (range: 23-63). The most common preoperative diagnosis was myoma of the uterus.(7/11 patients). The others were endometrial polyp, cervical cancer, and ESS. Only one patient was proved to be ESS by cervical punch biopsy which was taken prior to the operation. Eight patients were in stage I and two in stage III. One could not be evaluated by stage, as she had taken operation at the other hospital without clear record of it. Ten patients were low grade ESS. Six patients were treated only by surgery in a curative intent. Five patients received postoperative chemotherapy ,and no patient received radiotherapy. Eight out of eleven patients were alive without any evidence of disease and two were in the state of stable disease. One patient was dead from ESS. Three patients experienced recurrences after adjuvant chemotherapy. The two patients (2/10 low grade ESS) with low grade ESS were recurred and one in high grade ESS(1/1 high grade ESS). Conclusion: It is very difficult to diagnose ESS preoperatively. Clinical and pathological features have broad spectrum of characteristics. Surgery and adjuvant chemotherapy is the most effective treatment for patients with ESS. Less myometrial invasion, earlier tumor stage, less mitotic count are associated with longer overall survival in patients with ESS.

A Case of Low-Grade Endometrial Stromal Sarcoma Metastatic to the Lung

Endometrial stromal sarcomas (ESS) represent approximately 15-25% of all uterine sarcomas and are divided into three types: endometrial stromal nodule, low-grade ESS or endolymphatic stromal myosin, and high-grade ESS. The low-grade ESS is the most common and has a favorable prognosis with 5- and 10-year survival rates of up to 100%. Recurrence is not uncommon and tends to be limited to the pelvis. However, it shows distant metastases after long tumor-free intervals and the most common affected site is the lung, with reported incidence ranging from 7% to 28%. Here we discribe a case of low-grade ESS with pulmonary metastasis with the brief review of literature. This case, 42 year old woman, who had the past history of LAVH (Laparoscopic Assisted Vaginal Hysterectomy) with clinical diagnosis of uterine leiomyoma but proved low-grade ESS by histology was found multiple mass in lung. After pulmonary metastasectomy, she had no recurrence for 10 months after surgery.

CD10 is a sensitive and diagnostically useful immunohistochemical marker of normal endometrial stroma and of endometrial stromal neoplasms.

The CD10 antigen is expressed in acute lymphoblastic leukaemia and follicle centre cell lymphoma. A recent study investigating the expression of CD10 in a wide range of non-haematopoietic neoplasms found positive staining in a small number of endometrial stromal sarcomas as well as in normal endometrial stroma. The present study aimed to ascertain whether CD10 positivity is indeed found in normal endometrial stroma and endometrial stromal neoplasms. Staining of a range of tumours which can be confused morphologically with endometrial stromal neoplasms was also undertaken to ascertain whether antibodies against CD10 are of value in a diagnostic sense. Neoplasms included in the study were endometrial stromal nodule (n=1), low-grade endometrial stromal sarcoma (ESS) (n=13), high-grade ESS (n=6), mixed endometrial stromal-smooth muscle tumour (n=1), uterine cellular leiomyoma (n=10), uterine leiomyosarcoma (n=5), adult granulosa cell tumour (AGCT) (n=10), undifferentiated endometrial carcinoma (n=6), uterine carcinosarcoma with an endometrial stromal component (n=1) and type II uterine mesenchymal tumour with sex cord-like elements (n=1). Cases of proliferative (n=5), secretory (n=5) and atrophic (n=3) endometrium were also stained. There was positive staining of stroma but not of glands in all cases of non-tumorous endometrium. There was positive staining of the endometrial stromal nodule and of all low-grade ESS. Staining in these varied but was often diffuse and of moderate to strong intensity. There was positive staining of four of six high-grade ESS, but this was usually focal. There was also positive staining of the endometrial stromal component in the mixed endometrial stromal-smooth muscle tumour and in the uterine carcinosarcoma. Most cellular leiomyomas were completely negative although three exhibited weak positivity. There was some positivity, usually focal or weak, of three of five leiomyosarcomas. Most AGCT and undifferentiated carcinomas were completely negative although one case of each exhibited focal staining. There was focal staining of the type II uterine mesenchymal tumour with sex cord-like elements. CD10 is a reliable and sensitive immunohistochemical marker of normal endometrial stroma. Positivity, which is often strong and/or diffuse is found in endometrial stromal nodules and low-grade ESS. Positive staining with CD10, when strong and diffuse, may be useful in distinguishing these tumours from histological mimics, especially cellular leiomyoma and AGCT which are generally negative. In this situation, CD10 should be used as part of a panel which might include desmin and alpha-inhibin depending on the differential diagnosis considered. Positive staining with CD10 in a high-grade uterine sarcoma which is negative with muscle markers might indicate endometrial stromal differentiation and identify a group of neoplasms which it is correct to diagnose as high-grade ESS rather than undifferentiated uterine sarcoma.

Prognostic Parameters in Endometrial Stromal Sarcoma: A Clinicopathologic Study in 31 Patients

Objective. The aim of this study was to evaluate the behavior of endometrial stromal sarcomas (ESS) in relation to their clinical and pathologic features and to identify possible prognostic factors.Methods. Thirty-one patients with histologically proven ESS were included in the analysis. Endometrial stromal sarcoma is characterized by proliferations composed of cells with endometrial stromal cell differentiation. A breakpoint of 10 mitoses per 10 high-power fields was used in the statistical analysis to distinguish between low-grade and high-grade endometrial stromal sarcoma and to evaluate the prognostic value of mitotic count in patients with ESS.Results. The median follow-up time was 72 months (range 34–110). The median overall survival of the 31 patients was 127 months, resulting in a 5-year overall survival rate of 62%. Adjuvant therapy was administered to 25 patients; among those, 20 patients received postoperative radiotherapy and 5 patients received chemotherapy. Ten of the irradiated patients and 3 patients undergoing chemotherapy developed disease recurrence. Concerning the response rate to adjuvant chemotherapy, 1 patient showed a complete response, 1 patient a partial response, 1 patient stable disease, and 2 patients progressive disease. Altogether, 14 patients developed recurrent disease with a median disease-free survival of 11 months (range 5–60). Twelve patients died of the disease. A univariate model revealed that early tumor stage (P < 0.0007), low myometrial invasion (P < 0.008), and low mitotic count (P < 0.005) were associated with a lengthened overall survival in patients with endometrial stromal sarcoma. Age and adjuvant therapy did not influence overall survival of patients with ESS.Conclusion. Early tumor stage, low myometrial invasion, and low mitotic count are associated with a lengthened overall survival in patients with ESS.

Nonpuerperal Inversion of the Uterus Associated with Endometrial Stromal Sarcoma

Nonpuerperal uterine inversion is an extremely rare gynecologic event. The vast majority of nonpuerperal inversions occur in association with a uterine tumor, of which approximately 20% are malignant. The case of a 74-year-old patient with suspected primary cervical sarcoma is presented. At the time of surgery, nonpuerperal uterine inversion was identified. High-grade endometrial stromal sarcoma was confirmed on pathologic examination. The etiologic factors that constitute the differential diagnosis are discussed, as well as strategies for managing this unusual entity.

Pelvic malignant mixed mesodermal tumor of uncertain origin: a case report

Extra-uterine, and especially extragenital, malignant mixed mesodermal tumors (MMMT) are very rare. A large intrapelvic tumor resected from a 56-year-old woman was investigated with morphological and immunohistochemical methods. A large, soft and fragile tumor was located in the pelvic space. The tumor showed high cellularity and was biphasic; it consisted of an admixture of adenocarcinoma and various kinds of sarcomas. The latter were comprised of high-grade endometrial stromal sarcoma, pleomorphic sarcoma, and chondrosarcoma. The pleomorphic sarcoma showed a storiform pattern. The periodic acid-Schiff-positive eosinophilic hyaline droplets and globules in multinucleated giant cells revealed a typical ring-like or peripheral staining for alpha-1-antitrypsin and alpha-1 antichymotrypsin. We considered this case to be pelvic MMMT of uncertain origin, heterologous type.

Expression of metalloproteinases endometrial stromal sarcoma: immunohistochemical study using image analysis.

AIM: To investigate the expression of matrix metalloproteinases (MMP), a group of proteolytic enzymes with a central role in extracellular matrix invasion and degradation, in stromal sarcomas. METHODS: 11 endometrial...

Endometrial stromal sarcoma of the uterus: a clinicopathologic, DNA flow cytometric, p53, and mdm-2 analysis of 17 cases.

Seventeen patients with endometrial stromal sarcoma (ESS) diagnosed between 1970 and 1996 were evaluated according to DNA ploidy, S-phase fraction (SPF), p53, and mdm-2 expression, as well as traditional clinical and pathologic prognostic factors, such as tumor stage, grade, and mitotic index. DNA flow cytometric analysis and immunohistochemical staining for p53 and mdm-2 were performed on paraffin-embedded archival tissue from the uterine tumors. Flow cytometric DNA histograms were obtained from 16 patients. The patients ranged in age from 41 to 78 years (median, 57 years). Seven (41%) patients were premenopausal. Thirteen low-grade ESS were DNA diploid and had a low SPF. Of these, two overexpressed p53, while only one was mdm-2 positive. Among the four high-grade ESS we found one (25%) DNA diploid tumor and three (75%) DNA aneuploid tumors. Two (50%) had an SPF greater than 10%, three (75%) were p53-positive, and two (50%) overexpressed mdm-2. During the observation period, nine (53%) patients (five with low-grade and four with high-grade tumors) died of disease. The 5-year survival rate for patients with low-grade ESS was 74%, while all four patients with high-grade ESS died of disease within 14 months of diagnosis. Using the log-rank test, we found a significant correlation between survival and tumor grade (P = 0.007), DNA ploidy (P = 0.026), SPF (P = 0.048), and FIGO surgical stage (P = 0.026). In conclusion, we found that tumor grade was a strong predictor of clinical outcome in ESS. In addition, a worse prognosis was found for those ESS patients with advanced disease, DNA aneuploidy, and a high SPF. There was no difference between the recurrent and nonrecurrent group of early stage (surgical stage I), low-grade ESS with regard to clinicopathological features, DNA ploidy, SPF, p53, and mdm-2 expression. All patients with high-grade ESS died of disease within 14 months of diagnosis. In contrast, only three of the 11 patients with early stage, low-grade ESS died of disease.

Conservative management of endometrial stromalsarcoma in young women

Lissoni A, Cormio G, Perego P, Gabriele A, Cantu MG, Bratina G.Conservative management of endometrial stromal sarcoma in young women.Int J Gynecol Cancer 1997; 7: 364–367. Endometrial stromal sarcoma (ESS) is a rare malignancy with an associatedpoor prognosis. Total abdominal hysterectomy is considered the treatment ofchoice, and the role ofconservative, fertility-sparing surgery in young patients desiring pregnancy isnot well-defined. Six patients were managed conservatively for ESS between 1982 and 1996 atour institution. The sources of tissue for diagnosis were polyps or myomasremoved for relief ofsymptoms. Median age of the patients was 27 years (range 18–36), and allwere nulliparous. One patient had a high-grade ESS (12 mitoses per 10 HPF) withextensive necrosis andatypia. The remaining five patients had a low-grade ESS, with a median mitoticcount of 4 per 10 HPF (range 2–6). In all cases the tumor was completelyresected, with a saferesection margin of at least 2 mm. No patients received adjuvant treatment. At a median follow-up of 51 months (range 12–84) all patients arealive and well without recurrence. Two patients (33%) had a spontaneousdelivery at term, whereas one patient had a miscarriage at the 8th week ofgestation. Conservative management of endometrial stromal sarcoma may be attempted inselected patients desiring pregnancy. Adequate follow-up is mandatory in orderto detect recurrence assoon as possible. Considering the indolent growth of low-grade ESS, definitivesurgery can be postponed until after completion of the reproductivefunction.

Malignant tumors arising in endometriosis: clinical-pathological study and flow cytometry analysis

Background: Malignant transformation to endometriosis is a well documented phenomenon that occurs most commonly in the ovaries with cancer arising in extra-ovarian endometriosis being a rare event. Methods: A retrospective clinical-pathological evaluation of eleven cases with malignant tumors arising in endometriosis was performed to evaluate the prognostic impact of various factors. Nuclear DNA content (ploidy) was assessed through flow cytometric study. Results: Ovarian origin was identified in eight cases and three were associated with extra-ovarian endometriosis. Histologic type was endometrioid carcinoma in ten patients. The eleventh case had high grade endometrial stromal sarcoma. All tumors were diploid with no relation to stage, grade, or clinical outcome. The S-phase fraction (SPF) was analyzed in nine patients and no correlation could be demonstrated with any histologic parameters or clinical outcome. Conclusions: The DNA content seems to have no association with the classical prognostic parameters in these cases.

Endometrial Stromal Sarcoma: Analysis of Treatment Failures and Survival

The objective of this retrospective multicenter study was to assess the rates, times, and sites of recurrences of 26 patients with low-grade endometrial stromal sarcomas (ESS) (<10 mitoses per 10 high-power fields (HPF) and 40 patients with high-grade ESS (≥10 mitoses/10 HPF). Surgery was the initial therapy for all patients. Postoperative treatment was given without well-defined protocols. The median follow-up of survivors was 92 months (range, 4–167). Low-grade ESS: Of the 20 patients with disease confined to the uterus, 5 (25.0%) developed pelvic recurrence after a median of 36 months (range, 4–108). Of the 6 patients with disease outside the uterus, only one recurred in the pelvis after 93 months. High-grade ESS: Of the 20 patients with disease confined to the uterus, 11 (55.0%) developed recurrent disease after a median of 5 months (range, 2–76). The relapse occurred in the pelvis in 3 patients, in upper abdomen in 3, in upper abdomen and extraabdominal sites in 1, and in the pelvis and upper abdomen or extraabdominal sites in 4. Of the 12 patients with extrauterine disease confined to the pelvis, 9 (75.0%) developed recurrent disease after a median of 12 months (range, 1–49). The relapse occurred in the pelvis in 3 patients, in extraabdominal sites in 3, and in the pelvis and upper abdomen or extraabdominal sites in 3. Of the 8 patients with extrauterine disease outside the pelvis, 7 died of disease and 1 is currently alive with progressive disease after 24 months. The disease-free survival was significantly better for low-grade than that for high-grade ESS (P= 0.0001). By log-rank test the disease-free survival of high-grade ESS patients was related to stage (P= 0.0466) and mitotic count (P= 0.0014), but not to age. Cox model showed that mitotic count was the only independent prognostic variable for high-grade ESS (P= 0.006). In conclusion, low-grade and high-grade ESS have a completely different biological aggressiveness and clinical behavior.