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*

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

Carcinoma ; pathology ; Endometrial Neoplasms ; pathology ; Female ; Humans ; Neoplasms, Multiple Primary ; pathology ; Ovarian Neoplasms ; pathology ; Sex Cord-Gonadal Stromal Tumors ; pathology

Actins ; metabolism ; Adult ; Diagnosis, Differential ; Endometrial Neoplasms ; metabolism ; pathology ; surgery ; Endometrial Stromal Tumors ; metabolism ; pathology ; surgery ; Female ; Humans ; Hysterectomy ; Leiomyoma, Epithelioid ; metabolism ; pathology ; Melanoma-Specific Antigens ; metabolism ; Perivascular Epithelioid Cell Neoplasms ; metabolism ; pathology ; surgery ; Receptors, Progesterone ; metabolism ; Sarcoma, Clear Cell ; metabolism ; pathology ; Uterine Neoplasms ; metabolism ; pathology ; surgery

Actins ; metabolism ; Adenocarcinoma, Clear Cell ; metabolism ; pathology ; Adult ; Carcinoma, Renal Cell ; metabolism ; pathology ; Desmin ; metabolism ; Diagnosis, Differential ; Endometrial Stromal Tumors ; metabolism ; pathology ; Female ; Follow-Up Studies ; Humans ; Hysterectomy ; Leiomyoma, Epithelioid ; metabolism ; pathology ; Melanoma ; metabolism ; pathology ; Melanoma-Specific Antigens ; metabolism ; Perivascular Epithelioid Cell Neoplasms ; metabolism ; pathology ; surgery ; Receptors, Progesterone ; metabolism ; Uterine Neoplasms ; metabolism ; pathology ; surgery ; Vimentin ; metabolism

Diagnosis, Differential ; Endometrial Stromal Tumors ; metabolism ; pathology ; surgery ; Female ; Follow-Up Studies ; Gastrointestinal Stromal Tumors ; pathology ; Humans ; Leiomyoma ; metabolism ; pathology ; Middle Aged ; Neprilysin ; metabolism ; Receptors, Estrogen ; metabolism ; Receptors, Progesterone ; metabolism ; Rectal Neoplasms ; metabolism ; pathology ; surgery ; Rectum ; surgery ; Solitary Fibrous Tumors ; metabolism ; pathology

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

Carcinoma, Renal Cell ; pathology ; Diagnosis, Differential ; Digestive System Neoplasms ; pathology ; Female ; Gastrointestinal Stromal Tumors ; pathology ; Humans ; Kidney Neoplasms ; pathology ; Leiomyoma ; pathology ; Male ; Melanoma ; pathology ; Perivascular Epithelioid Cell Neoplasms ; pathology ; Sarcoma, Clear Cell ; pathology ; Sarcoma, Endometrial Stromal ; pathology ; Skin Neoplasms ; pathology ; Soft Tissue Neoplasms ; pathology ; Uterine Neoplasms ; pathology

Adult ; Carcinoma, Endometrioid ; diagnosis ; pathology ; Cervix Uteri ; pathology ; Diagnosis, Differential ; Endometrial Neoplasms ; diagnosis ; pathology ; Endometrial Stromal Tumors ; diagnosis ; pathology ; Female ; Humans ; Middle Aged

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