No abstract available.
Sarcoma, Endometrial Stromal*

OBJECTIVE: The aim of this study was to obtain information on the prognosis and treatment of the low grade endometrial stromal sarcoma. PATIENTS AND METHODS: Three patients with low grade endometrial stromal sarcoma treated in kangnam St. Mary's hospital between 1993 and 2002 were evaluated. The clinical stage, management and outcome were analyzed. RESULTS: Those patients with low grade endometrial stromal sarcoma had a long overall survival in the presence of receiving hormonal therapy after initial surgery. Two are alive with disease-free status, one has shown a partial response to hormonal treatment. CONCLUSION: Surgery is the primary treatment for low grade endometrial stromal sarcoma. High dose hormonal replacement as adjuvant therapy will be associated with a lengthened overall survival in patients with low grade endometrial stromal sarcoma.
Humans ; Prognosis ; Sarcoma, Endometrial Stromal*

Endometrial stromal sarcomas are rare uterine tumors accounting for about 0.2% of female genital tract malignancies, which are composed of cells closely resembling normal proliferative endometrial stromal cells. The tumor is classified into a low-grade and a high-grade variety on the basis of the mitotic rate. The low- grade endometrial stromal sarcoma has many synonyms, which include endolymphatic stromal myosis, stromatosis, stromal endometriosis and endometrioid sarcoma. Because the endometrial stromal sarcoma is very rare, the preoperative diagnosis and postoperative treatment are still difficult for clinicians. We have experienced two cases of low-grade endometrial stromal sarcoma of the uterus which are presented with a review of brief literature.
Diagnosis ; Endometrial Stromal Tumors ; Endometriosis ; Female ; Humans ; Sarcoma ; Sarcoma, Endometrial Stromal* ; Stromal Cells ; Uterus

Endometrial stromal sarcomas are rare uterine tumors accounting for about 0.2% of female genital tract malignancies, which are composed of cells closely resembling normal proliferative endometrial stromal cells. The tumor is classified into a low-grade and a high-grade variety on the basis of the mitotic rate. The low- grade endometrial stromal sarcoma has many synonyms, which include endolymphatic stromal myosis, stromatosis, stromal endometriosis and endometrioid sarcoma. Because the endometrial stromal sarcoma is very rare, the preoperative diagnosis and postoperative treatment are still difficult for clinicians. We have experienced two cases of low-grade endometrial stromal sarcoma of the uterus which are presented with a review of brief literature.
Diagnosis ; Endometrial Stromal Tumors ; Endometriosis ; Female ; Humans ; Sarcoma ; Sarcoma, Endometrial Stromal* ; Stromal Cells ; Uterus

BACKGROUND: The diagnosis of endometrial stromal sarcoma (ESS) is often difficult in cases showing diverse histological differentiation or in undifferentiated endometrial sarcoma (UES). Recently, JAZF1/JJAZ1 gene fusion has been described as a defining feature of low-grade ESS (LGESS). However, its prevalence is variably reported, and the diagnostic utility has rarely been examined for cases showing various histological differentiation. METHODS: To test the diagnostic utility of JAZF1/JJAZ1 gene fusion in difficult cases, we compared the prevalence of the JAZF1/JJAZ1 fusion gene in LGESS with and without histological differentiation. RESULTS: The JAZF1/JJAZ1 fusion transcript was detected in 18 of 21 LGESS (85.7%), including 14 classical LGESS (93%), four LGESS with diverse histological differentiation (67%), and two with UES (28.6%). Positive cases included two LGESS with sex cord-like differentiation, one with osseous differentiation, and two UES. LGESS showing smooth muscle differentiation revealed the fusion transcript only in the classic area. Direct sequencing analysis of two LGESS revealed a previously reported breakpoint at t(7;17)(p15;q21). CONCLUSIONS: The JAZF1/JJAZ1 fusion gene was identified in a significant proportion of LGESS showing secondary histological differentiation except in cases with smooth muscle differentiation. Thus, this fusion gene may be useful to confirm the diagnosis in difficult cases of LGESS.
Gene Fusion ; Muscle, Smooth ; Prevalence ; Sarcoma ; Sarcoma, Endometrial Stromal

Endometrial stromal sarcoma (ESS) is a relatively rare uterine sarcoma, especially extrauterine ESS. Furthermore, retroperitoneal ESS are extremely rare. Up to now, there are only four cases of primary retroperitoneal ESS reported in the literature. We report one case of primary retroperitoneal undifferentiated endometrial stromal sarcoma after concurrent chemoradiation therapy for cervical cancer with a brief review of the literature.
Retroperitoneal Space ; Sarcoma ; Sarcoma, Endometrial Stromal ; Uterine Cervical Neoplasms

Mixed endometrial stromal and smooth-muscle tumor is one of the uncommon forms of uterine sarcoma. Only a few cases of endometrial stromal tumors showing smooth muscle differentiation have been reported in the literature. We experienced a case of low grade endometrial stromal sarcoma with smooth muscle differentiation in a 44-year-old woman, so we report this case with brief review of literatures.
Adult ; Endometrial Stromal Tumors ; Female ; Humans ; Muscle, Smooth* ; Sarcoma ; Sarcoma, Endometrial Stromal*

PURPOSE: The goal of this study was to perform a retrospective analysis of the ultrasonographic findings associated with low-grade endometrial stromal sarcoma. METHODS: Ten pathologically confirmed cases of low-grade endometrial stromal sarcoma at our institution from January 2007 to April 2014 were retrospectively reviewed. All patients underwent a preoperative transvaginal ultrasound. Two radiologists came to a consensus regarding the location, size, margin, and echogenicity of the tumor, as well as the presence of intratumoral cystic degeneration and its extent and configuration. RESULTS: Low-grade endometrial stromal sarcoma manifested as an intramural mass protruding into the endometrial cavity (n=6) or as a purely intramural mass (n=4). The maximal diameter of the lesion ranged from 4 to 9.1 cm (mean, 6.2 cm). The imaging features of low-grade endometrial stromal sarcoma were variable: six cases involved predominantly solid masses containing cystic degeneration, one was a predominantly unilocular cystic mass, two were ill-defined infiltrative solid masses, and one was a well-defined solid mass. Among the seven cases with internal cystic degeneration, five patients showed a multiseptated cystic area or a cystic area with multiple small clusters, while a unilocular cystic area within the tumor was found in two patients. CONCLUSION: Low-grade endometrial stromal sarcoma is associated with variable ultrasonographic findings with regard to the location, margin, and configuration of the lesion. Multiseptated cystic areas and multiple small areas of cystic degeneration are common.
Consensus ; Endometrial Stromal Tumors ; Humans ; Retrospective Studies ; Sarcoma ; Sarcoma, Endometrial Stromal* ; Ultrasonography ; Uterus*

The development of endometrial stromal sarcomas (ESSs) in foci of endometriosis is extremely rare, and few cases have been reported in the literature to date, particularly with regard to multiple extrauterine ESS. Here we report a case of endometrial stromal sarcoma with multiple metastasis that arose from an ovarian endometriotic lesion. The literature is also briefly reviewed.
Endometriosis ; Female ; Neoplasm Metastasis ; Ovary ; Sarcoma, Endometrial Stromal

Uterine tumor resembling ovarian sex cord tumors (UTROSCTs) are uncommon neoplasms that histologically resemble ovarian sex cord tumor. Over 50 cases have been reported up to recently. Clement and Scully reported 14 cases of UTROSCT, which they devided into two groups by clinicopathological features. Group I tumors are endometrial stromal sarcomas with partially sex-cord like elements (less than 40% of total tumor volume). On the other hand, group II tumors are mostly or entirely composed of sex-cord like elements. We have experienced a very rare case of type II UTROSCT at the uterine fundus in a 52-year-old woman and report it with a brief review of the literatures.
Female ; Hand ; Humans ; Middle Aged ; Sarcoma, Endometrial Stromal