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

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

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

OBJECTIVE: To observe the expression of HELQ and RAD51C in normal endometrial and endometrial stromal sarcoma (ESS) and analyze their correlation with the clinical features of the patients. METHODS: The expressions of HELQ and RAD51C proteins were detected by immunohistochemical staining in normal endometrial tissues (14 cases) and tumor tissues from patients with ESS (37 cases) treated in Hunan Provincial Cancer Hospital from January, 2013 to December, 2016. The correlations of the expressions of the two proteins with the patients'age, FIGO staging, tissue type, tumor size, and lymph node metastasis were analyzed. RESULTS: Immunohistochemical staining showed that the expressions of HELQ and RAD51C were both decreased in ESS patients compared with the normal group, and there was a positive correlation between HELQ and RAD51C expression ( < 0.05). HELQ expression in ESS was correlated with the tumor size and type. The expressions of HELQ and RAD51C were not correlated with the patients' age, FIGO stage and status of lymph node metastasis ( > 0.05). CONCLUSIONS Homologous recombination- directed DNA repair involving HELQ and RAD51C may participate in the occurrence and progression of ESS.
DNA Helicases ; DNA-Binding Proteins ; Endometrial Neoplasms ; Endometrium ; Female ; Humans ; Lymphatic Metastasis ; Sarcoma, Endometrial Stromal

Adult ; Endometrial Neoplasms ; surgery ; Female ; Fertility ; physiology ; Humans ; Laparoscopy ; Pregnancy ; Pregnancy Outcome ; Sarcoma, Endometrial Stromal ; surgery

Pulmonary metastases of uterine endometrial stromal sarcoma (ESS) are un com-mon. The patterns of uterine ESS metastasis to the lung are multiple pulmonary nodules, single nodule, or cystic lesions. Pulmonary intraalveolar micronodular metastases of uterine ESS are unusual and have not been reported. We experienced a case of metastatic uterine ESS presenting as pulmonary diffuse micronodules with ground glass opacities on chest computed tomography of a 37-yr-old woman who previously underwent hysterectomy due to low grade ESS of the uterus four years ago. The histologic findings of video assisted thoracotomy biopsy showed numerous intraalveolar polypoid micronodules protruding from the alveolar septums. All tumor nodules were composed of short spindle cells arranged in ill-defined whorls, and nuclear feature and sparse cytoplasm were seen in uterine ESS. Immunohistochemically, these cells showed strong nuclear staining for estrogen receptor and progesterone receptor, and diffuse cytoplasmic staining for CD10.
Adult ; Endometrial Neoplasms/*pathology/radiography ; Female ; Humans ; Lung Neoplasms/*pathology/radiography/*secondary ; Sarcoma, Endometrial Stromal/*pathology/radiography/*secondary

Uterine and extrauterine tumors composed of cells featuring endometrial stromal cells often show ovarian sex cord-like structures and smooth muscle differentiation. A few cases of endometrial stromal tumors showing rhabdoid differentiation have been reported. The present case is a 20-year-old woman with endometrial stromal sarcoma that had sex cord-like structures, smooth muscle components and rhabdoid differentiation.
Adult ; Case Report ; Cell Differentiation ; Endometrial Neoplasms/*pathology ; Female ; Human ; Muscle, Smooth/*pathology ; Rhabdoid Tumor/*pathology ; Sarcoma, Endometrial Stromal/*pathology

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

In this study, we summarize the clinical role of magnetic resonance imaging (MRI) in the diagnosis of patients with malignant uterine neoplasms, including leiomyosarcoma, endometrial stromal sarcoma, adenosarcoma, uterine carcinosarcoma, and endometrial cancer, with emphasis on the challenges and disadvantages. MRI plays an essential role in patients with uterine malignancy, for the purpose of tumor detection, primary staging, and treatment planning. MRI has advanced in scope beyond the visualization of the many aspects of anatomical structures, including diffusion-weighted imaging, dynamic contrast enhancement-MRI, and magnetic resonance spectroscopy. Emerging technologies coupled with the use of artificial intelligence in MRI are expected to lead to progressive improvement in case management of malignant uterine neoplasms.
Adenosarcoma ; Artificial Intelligence ; Carcinosarcoma ; Case Management ; Diagnosis ; Endometrial Neoplasms ; Female ; Humans ; Leiomyosarcoma ; Magnetic Resonance Imaging* ; Magnetic Resonance Spectroscopy ; Sarcoma ; Sarcoma, Endometrial Stromal ; Uterine Neoplasms*

Most of malignant tumors arising in ovarian and extraovarian endometriosis are carcinomas. Mixed mullerian tumor and endometrial stromal sarcoma arising in intestinal endometriosis are rarely described, but its clinicopathologic features have not been well characterized. Here we report a case of endometrial stromal sarcoma of the sigmoid colon arising in endometriosis with a review of six additional cases of endometrial stromal sarcoma arising in intestinal endometriosis found in English literatures. The patients ranged in age from 36 to 64 yr. Presenting symptoms were pain, bloody diarrhea, and tenesmus. Some patients had a previous history of endometriosis. Most of the tumors arose in the rectosigmoid colon. The histologic features were the same as their uterine counterpart. No death of disease had been reported. This rare tumor should not be confused with gastrointestinal stromal tumor clinically and histologically.
Endometriosis/*complications ; Female ; Humans ; Middle Aged ; Sarcoma, Endometrial Stromal/*pathology ; Sigmoid Neoplasms/*pathology