CT and MRI features of endometrial stromal sarcomas / 中国介入影像与治疗学

Objective To: observe CT and MRI features of endometrial stromal sarcoma (ESS). Methods: Plain and enhanced imaging data, including CT (n=5) and MRI (n=6) of 11 patients with ESS confirmed by postoperative pathology were analyzed retrospectively. Results: There were 9 cases of singe low-grade ESS and 2 cases of single undifferentiated sarcoma. The lesions located in the uterine cavity in 7 cases, in the myometrium in 4 cases, manifested as circular masses in 8 cases and as irregular masses in 3 cases. The mean maximum diameter of lesions was (9.18±1.36)cm. The boundaries of lesions were clear in 4 cases and unclear in 7 cases. Based on CT and MRI findings, there were 4 patients with solid masses, 6 patients with solid-cystic masses and 1 case with cystic mass. Solid component of lesions manifested as iso- or hypo-attenuation compared with myometrium on plain CT images in 5 patients, including 4 cases with nonuniform density lesions and 1 case with uniform density lesion. MRI showed signal reducing on ADC images in 6 patients. Among them, lesions in 5 cases manifested as iso- or hypo-signal on T1WI, hyper-signal or slightly high signal on T2WI, and slightly high signal on DWI, lesion in another case (cystic mass) manifested as hyper-signal on T1WI, high signal on T2WI and high signal in central area of lesion on DWI. Incremental and continuous enhancement were found in 10 cases, while no enhancement was found in the rest one case (cystic mass). Cystic and necrosis changes were found in 8 cases, and invasions of deep layer myometrium were found in 6 cases. Some of the patients had complications, including adenomyosis in 2 cases, uterine fibroid in 5 cases, pelvic effusion in 5 cases, intrauterine hemorrhage in 1 case and salpingian dropsy in 2 cases. Conclusion: ESS has characteristic CT and MRI findings, which can provide useful references for diagnosis.

Clinicopathological study of BCOR rearrangement in high grade endometrial stromal sarcoma / 中华病理学杂志

Objective@#To investigate clinicopathological, cytogenetic features and differential diagnoses of high grade endometrial stromal sarcoma (HGESS) with BCOR gene rearrangement.@*Methods@#Five cases of HGESS with BCOR rearrangement were collected from consultant files (2016-2018) at Fudan University Shanghai Cancer Center. Interphase FISH was performed using a dual color break-apart probe. The clinical data, histologic features and immunohistochemical findings were reviewed.@*Results@#All 5 cases occurred in adult women with a median age of 48 (range, 45-55) years. Abdominal pain and abnormal vaginal bleeding were the most common symptoms. Microscopically, the tumors showed mainly tongue-like and/or intersecting myometrial invasion. Stromal myxoid matrix and/or collagen plaques were prominent in all the cases. Most tumors consisted of uniform, haphazard fascicles of short spindle cells with mild to moderate nuclear atypia. Mitotic figures and necrosis were easily identified. Significant nuclear pleomorphism was not seen. Most tumors were rich in thick-walled small vessels. Prominent perivascular tumor cell whorling seen in conventional low-grade endometrial stromal sarcoma was not seen. All tumors expressed CD10 with only focal or absent desmin, SMA and/or h-caldesmon staining. ER or PR expression was seen in 4 tumors and 1 tumor showed both marker expression. Diffuse cyclin D1 was present in 2 tumors. BCOR immunoreactivity was present with strong staining in 3 cases and moderate staining in 1 case respectively. Ki-67 index ranged from 10% to 30%. Fluorescence in situ hybridization confirmed chromosomal aberration of BCOR gene in all tumors, that were previously diagnosed as myxoid leiomyosarcoma (2 cases), spindle cell uterine sarcoma (2 cases) and low-grade endometrial stromal sarcoma (1 case). Limited follow-up information revealed that 3/5 patients developed tumor recurrence, metastasis or death within one year.@*Conclusion@#BCOR rearranged HGESS has distinct morphological features and aggressive clinical behavior. In the presence of significant overlapping morphologic features between BCOR rearranged HGESS and other myxoid uterine mesenchymal tumors, especially myxoid leiomyosarcoma, molecular analysis is essential for accurate diagnoses.

What MRI features suspect malignant pure mesenchymal uterine tumors rather than uterine leiomyoma with cystic degeneration? / 부인종양

OBJECTIVE: To retrospectively assess conventional magnetic resonance imaging (MRI) features that differentiate malignant pure mesenchymal uterine tumors (MPMUT); endometrial stromal sarcoma (ESS) and leiomyosarcoma (LMS) from uterine leiomyoma with cystic degeneration (ULCD). METHODS: We retrospectively reviewed magnetic resonance (MR) images of 30 patients with ULCD, 18 with ESS, and 15 with LMS, to assess tumor location, margin, T2 signal intensity (SI), speckled appearance, and peripheral band using univariate and multivariate analyses. RESULTS: ULCD more frequently showed subserosal location (53.3%), well-defined margin (96.7%), and speckled appearance (90.0%) compared with ESS (0%, 33.3%, and 33.3%, respectively) or LMS (20.0%, 33.3%, and 60.0%, respectively). In quantitative T2 SI comparisons, the T2 SI ratio of the main solid tumor portion to gluteus maximus muscle differed significantly among the three groups, with ULCD showing a lower SI ratio (0.62) compared with ESS (2.44) and LMS (1.13). On multivariate analysis, an ill-defined margin (odds ratio [OR]=44.885; p=0.003) and high T2 SI (OR=4.396; p=0.046) were the significant MR differentiators. CONCLUSION: An ill-defined tumor margin and high T2 SI ratio of the main solid tumor-to-gluteus maximus muscle were useful MRI features in the differentiation of MPMUT from ULCD.

Values of JAZF1 gene rearrangement detected by fluorescence in-situ hybridization in diagnosis of endometrial stromal tumours / 中华病理学杂志

Objective@#To investigate the role of JAZF1 gene rearrangement in the diagnosis and differential diagnosis of endometrial stromal sarcomas by fluorescence in situ hybridization (FISH).@*Methods@#JAZF1 gene rearrangement was analyzed by FISH in 129 cases of ESS diagnosed from January 2008 to December 2016 including 105 cases of low-grade endometrial stromal sarcoma (LG-ESS), 21 cases of high-grade endometrial stromal sarcoma (HG-ESS) and 3 cases of undifferentiated uterine sarcoma (UUS). Sixteen cases of the related tumours in uterus were also collected as control group. The results were compared with our previous studies of JAZF1/JJAZ1 fusion gene in ESS by RT-PCR.@*Results@#Detection of JAZF1 gene rearrangement by FISH was successfully analyzed in 144 cases. JAZF1 gene alteration was detected in 63 cases, all of which were LG-ESS, with an overall positivity of 60.6% (63/104), while no JAZF1 gene rearrangement was found in all other cases. JAZF1 gene rearrangement was present in LG-ESS with classic histology (69.3%, 52/75), smooth muscle differentiation (2/10), sex cord-like differentiation (4/5), fibromyxoid change (1/5), clear cell change (0/1), skeletal muscle differentiation (0/1), and schwannoma-like palisading pattern (0/1). The different components in all the cases of LG-ESS with variant histology had the clonal origin, with or without JAZF1 gene alteration. Compared to the results of JAZF1/JJAZ1 fusion gene by RT-PCR, the positive rate of JAZF1 gene rearrangement in LG-ESS by FISH (61.9%, 26/42) was significantly higher than that of RT-PCR (30.0%, 12/40; P<0.01).@*Conclusions@#JAZF1 gene rearrangement is present only in LG-ESS, but not in HG-ESS, UUS or other related tumours in uterus. The frequency of JAZF1 gene rearrangement varies between classic LG-ESS and different morphologic variants. It is frequently, but not consistently, present in classic LG-ESS and less often positive in variant cases. The results of JAZF1 gene alterations in LG-ESS with different morphologic variants support the contention that the endometrial stromal and their variant morphologic components have the same clonal origin. Detection of JAZF1 gene rearrangement by FISH is very useful for the diagnosis and differential diagnosis of ESS.

Long-term survival of patients with recurrent endometrial stromal sarcoma: a multicenter, observational study / 부인종양

OBJECTIVE: The aim of this study was to evaluate the clinical behavior and management outcome of recurrent endometrial stromal sarcoma (ESS). METHODS: A retrospective review of charts of 10 patients with recurrent ESS was performed and relapse-free interval, relapse site, treatment, response to treatment, duration of follow-up and clinical outcome extracted. Survival outcome measures used were post-relapse survival which was defined as the time from first evidence of relapse to death from any cause. Living patients were censored at the date of last follow-up. RESULTS: The median age and median relapse-free interval at the time of initial relapse were 51.5 years and 66.5 months, respectively. The number of relapses ranged from one to five. Sixteen surgical procedures for recurrent disease included nine (56.0%) complete resections. There was no statistically significant difference between initial recurrent tumors and second/subsequent recurrent tumors in the rate of complete surgery (44.4% vs. 71.4%, respectively, p=0.36). Of the eleven evaluable occasions when hormonal therapy was used for recurrent disease, disease control was achieved in eight (72.7%). There was no difference between initial recurrent tumors and second/subsequent recurrent tumors in disease control rate by hormonal therapy (85.7% vs. 50.0%, respectively, p=0.49). The 10-year post-relapse survival rate was 90.0% and the overall median post-relapse survival 119 months (range, 7 to 216 months). CONCLUSION: Post-relapse survival of patients with ESS can be expected to be >10 years when treated by repeated surgical resection and hormonal therapy or both.

Endometrial Stromal Sarcoma Presented as an Incidental Lung Mass with Multiple Pulmonary Nodules / 결핵및호흡기질환

Low-grade endometrial stromal sarcoma (ESS) is an uncommon gynecologic malignancy of mesodermal origin. Pulmonary metastasis of low-grade ESS can occur years and decades after the treatment of the primary disease. Low-grade ESS is frequently mistaken as benign uterine neoplasm like uterine leiomyoma, which can potentially lead to a misdiagnosis. We present a case of a 42-year-old woman with low-grade ESS, that initially presented as an incidental lung mass with multiple pulmonary nodules, seven years after an uterine myomectomy. A 6.9x5.8 cm-sized intrapelvic mass suspected of uterine origin was discovered while searching for potential extrathoracic primary origin. A pelviscopy and simultaneous thoracoscopic lung biopsy were conducted for pathologic diagnosis. Finally, the diagnosis was confirmed as low-grade ESS with lung metastasis based on the histopathologic examination with immunohistochemical stain, which was showed positive for CD10 and hormone receptor markers (estrogen and progesterone receptors) in both pelvic and lung specimens.

Novos conceitos e revisão atualizada sobre sarcomas uterinos / New concepts and update review of uterine sarcomas

Realizou-se uma revisão sistemática das publicações dos últimos dez anos sobre os sarcomas uterinos. Este artigo traz novos conceitos, como a mudança do carcinossarcoma para carcinoma metaplásico. Evidencia-se a necessidade de individualizar o tratamento dos sarcomas, pois existem muitas diferenças de comportamento clínico, padrão metastático e prognóstico entre os diferentes tipos histológicos. Também foram enfocadas as controvérsias existentes quanto a extensão cirúrgica, a necessidade de ooforectomia e de esvaziamento ganglionar e o papel da adjuvância

It was performed a systematic review of publications for the past 10 years about uterine sarcomas. This article introduces new concepts, such as the change of carcinosarcoma to metaplastic carcinoma. This study highlights the need to individualize the treatment of uterine sarcomas, as there are many differences in clinical behavior, prognosis and metastatic pattern between the different histological types. Controversies have also been focused on the surgical extension, the need for lymph node dissection and oophorectomy and the role of adjuvant therapy

Endometrial Stromal Sarcoma Presenting as Prevesical Mass Mimicking Urachal Tumor

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, pelvic cavity, mesentery, omentum and intestine. Herein, we present a rare case of low-grade ESS presented as prevesical mass. A 60-yr-old woman who had undergone total hysterectomy for endometriosis eleven years ago was presented with incidentally detected prevesical pelvic mass. Since malignant transformation of urachal remnants was possible, the mass was suspected to be a urachal tumor. Extraction of the mass was performed, and the histopathologic diagnosis was low-grade ESS. In summary, prevesical tumor is rare but in patients with endometriosis, we suggest endometriosis and its possible malignant changes should be taken into account in the differential diagnosis of prevesical mass.

Clinical review of 97 patients with endometrial stromal sarcoma / 中华妇产科杂志

Objective To probe into the advantages and disadvantages of intravenous chemotherapy and intraperitoneal chemotherapy for advanced epithelial ovarian cancer.Methods All of the 226 patients with advanced epithelial ovarian cancer were treated by maximum cytoreductive surgery or non-effective cytoreductive surgery and received 6-8 courses of postoperative regular chemotherapy(chemotherapy regimens,TP:taxol and cis-platinum or carboplatinum;PC:cis-platinum and cyclophosphamide;PAC:cis platinum and adriamycin and cyclophosphamide)during Jan 1998-Jan 2006.We systematically compared the characteristics of patients in intraperitoneal chemotherapy(IPC)group and intravenous chemotherapy(IVC)group.We measured the incidence rate of the response,side-effects,the recurrence time of intraperitoneal tumor and survival time of the two groups respectively.ResultsFor the first phase after operation(three courses of treatment),the response rate of two groups were 75.8％and 52.8％respectively.For the response rate of IPC was higher than that of IVC(P＜0.01).The second phase after operation(all courses finished),the response rate of two groups were 93.9％and 87.7％,respectively(P＞0.05).After maximum cytoreductive surgery,the recurrence rate of IPC and IVC were 47.0％and 59.4％,respectively(P＞0.05).After non-effective cytoreductive surgery of IPC and IVC groups,the recurrence rates were 84.8％and 86.2％,respectively(P＞0.05).The recurrence time of intraperitoneal tumor of IPC and IVC groups were 24 and 18 months,respectively(P=0.001).The overall survival time ofgroups IPC and IVC were 32 and 30 months(P=0.188).There were some differences in the side-effect between IPC and IVC.The rates of chemotherapeutic phlebitis of IPC and IVC were 34.0％and 10.8％ respectively(P＜0.01).The rates of serious gastrointestinal reaction of IPC and IVC were 33.8％and 25.8％,respectively(P=0.236).There was no significant difference in bone marrow depression,intestinal adhesion and intestinal obstruction.ConclusionsIPC can extend the disease progression free survival than IVC,without increasing overall survival period.IPC can also reduce the side-effect of chemotherapeutic phlebitis.However,IPC is used limitedly,and can not substitute for IVC.Combination of IPC with IVC may enhance their effectiveness and reduce the side-effects.

Pulmonary Metastases of Uterine Endometrial Stromal Sarcoma: Diffuse Micronodular and Ground Glass Opacities: A Case Report

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.

Uterine Low Grade Endometrial Stromal Sarcoma Presented as Extrauterine Masses: A Case Report

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.