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

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*

Uterine sarcomas are relatively rare tumors of mesodermal origin that constitute 2-6% of uterine malignancies. Uterine sarcomas include endometrial stromal sarcoma (ESS), leiomyosarcoma (LMS), malignant mixed m llerian tumor (MMMT) of both homologous and heterologous type, pure heterologous sarcomas, blood vessel sarcomas, and lymphoma. Endometrial stromal tumors are divided into three types: 1) endometrial stromal nodule, 2) low-grade stromal sarcoma or endolymphatic stromal myosis, and 3) endometrial stromal sarcoma. The low grade endometrial stromal sarcoma is very rare. It shows occasional local recurrence, which might arise from endometrial stroma, from adenomyosis, and rarely from endometriosis. We have experienced a case of low grade endometrial stromal sarcoma of the uterus in a 54-year-old woman, which is presented with a review of literatures.
Adenomyosis ; Blood Vessels ; Endometrial Stromal Tumors ; Endometriosis ; Female ; Humans ; Leiomyosarcoma ; Lymphoma ; Mesoderm ; Middle Aged ; Recurrence ; Sarcoma ; Sarcoma, Endometrial Stromal* ; Uterus*

Endometrial stromal sarcoma (ESS) is a mesenchymal neoplasm that usually occurs as a primary tumor of the uterine corpus, but rarely arises in other sites, such as the ovary, the pelvic cavity, mesentery, omentum, and serosal or intramural portions of the large intestine. We present a case in which multiple nodules of ESS involving the taenia coli of the ascending colon were accompanied by grossly and radiologically unrecognized small, endometrial stromal lesions (less than 0.5 cm in the greatest dimension) with only focal marginal irregularities in the subsequent hysterectomy specimen. Whether this small sized endometrial stromal tumor is an incidentally associated endometrial stromal nodule (ESN) or a small sized, low grade ESS that was preceded by metastatic lesion is debatable. However, endometrial stromal tumors with tongue-like protrusions and associated fibroblastic stromal reaction around the tumor strongly favored these nodules being the small uterine ESS mimicking ESN. We propose that meticulous search for the detection of uterine ESS is mandatory before making a diagnosis of primary extrauterine ESS even in cases having a grossly or radiologically normal uterus and that the extent of focal irregularities of ESN should be more clearly defined for the correct diagnosis of ESS and ESN.
Colon ; Colon, Ascending ; Diagnosis ; Endometrial Stromal Tumors ; Female ; Fibroblasts ; Hysterectomy ; Intestine, Large ; Mesentery ; Omentum ; Ovary ; Sarcoma, Endometrial Stromal* ; Uterus

A 50-year-old woman was admitted to our hospital due to multiple lung nodules detected incidentally on a chest X-ray. A video-assisted thoracoscopic lung biopsy revealed low-grade endometrial stromal sarcoma (LG-ESS). She had undergone a simple hysterectomy 1 year earlier owing to a diagnosis of adenomyosis. A review of her previous hysterectomy specimen showed not endometriosis but LG-ESS. According to the patient's levels of serum follicle stimulating hormone and estradiol, she was in the premenopausal state with retained and normally functioning ovaries. She then underwent ovarian ablation by radiotherapy, after which she was administered 2.5 mg of letrozole once per day. Three months later, the size of the metastatic nodules in both lungs had decreased. The patient was followed up for 24 months while continuing on letrozole, and maintained a partial remission. We report herein on a case of metastatic LG-ESS treated with letrozole after ovarian ablation by radiotherapy.
Adenomyosis ; Biopsy ; Diagnosis ; Endometrial Stromal Tumors ; Endometriosis ; Estradiol ; Female ; Follicle Stimulating Hormone ; Humans ; Hysterectomy ; Lung ; Middle Aged ; Ovary ; Radiotherapy* ; Sarcoma, Endometrial Stromal* ; Thorax

OBJECTIVETo investigate the clinicopathologic features and the prognostic factors of endometrial stromal sarcoma (ESS).

METHODS55 cases of endometrial stromal sarcoma were reviewed and categorized into 3 pathologic types based on the related literatures, i.e., low grade endometrial stromal sarcoma (LGESS), undifferentiated endometrial sarcoma with nuclear uniformity (UES-U) and undifferentiated endometrial sarcoma with nuclear pleomorphism (UES-P). Meanwhile, the pathologic features were reviewed, including fibroid, myoid, mucoid, and epithelioid differentiation and mitotic index. Clinical and follow-up data were collected.

RESULTSIn endometrial stromal sarcoma, two or three pathologic types co-existed in one case, including 12.8% (5/39) of LGESS, 5/9 of UES-U, and 5/7 of UES-P. Mitotic index varied in different regions of one tumor from rare to high. Multi-differentiation was also commonly seen in ESS. The numbers of cases in LGESS, UES-U and UES-P were 39, 9 and 7, with recurrence rate of 51.6% (16/31), 5/6 and 2/3, respectively. There was no death case in LGESS, and 2 cases were died in UES-U and UES-P, respectively. In the 2 death cases of UES-U, both had focus of UES-P. There was a significant difference in the recurrence rate between cases with different mitotic index (≥ 10/10 HPF and < 10/10 HPF, P = 0.009), especially in LGESS group. All death cases had high mitotic index (> 30/10 HPF).

CONCLUSIONSIt is a common phenomenon in ESS that two or three pathologic types may exist in one case, especially in UES-U and UES-P. And multi-differentiation is also commonly seen in ESS. So adequate pathologic sampling is important for pathologists to make a correct diagnosis of ESS in daily work. The recurrence rates are significantly higher in cases with high mitotic index, especially in LGESS. In addition, the presence of UES-P and high mitotic index may increase the risk of death in the patients.

Adult ; Aged ; Aged, 80 and over ; Cell Differentiation ; Endometrial Neoplasms ; classification ; pathology ; surgery ; Endometrial Stromal Tumors ; pathology ; surgery ; Female ; Follow-Up Studies ; Humans ; Hysterectomy ; Middle Aged ; Mitotic Index ; Neoplasm Recurrence, Local ; Sarcoma, Endometrial Stromal ; classification ; pathology ; surgery ; Survival Rate ; Young Adult

Chromosome Aberrations ; Diagnosis, Differential ; Endometrial Neoplasms ; genetics ; metabolism ; pathology ; Endometrial Stromal Tumors ; genetics ; metabolism ; pathology ; Female ; Histone Deacetylases ; metabolism ; Humans ; Immunohistochemistry ; Neprilysin ; metabolism ; Receptors, Oxytocin ; metabolism ; Repressor Proteins ; metabolism ; Sarcoma, Endometrial Stromal ; genetics ; metabolism ; pathology ; Uterine Neoplasms ; metabolism ; pathology

No abstract available.
Endometrial Stromal Tumors* ; Lung* ; Neoplasm Metastasis* ; Uterus*