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El sarcoma del estroma endometrial es una neoplasia maligna poco frecuente que se origina en el estroma endometrial. Puede tener varias formas de diferenciación, entre ellas del músculo liso y del cordón sexual. El sarcoma del estroma endometrial de bajo grado es un tipo de tumor endometrial raro, que constituye solo el 0,2% de todas las neoplasias uterinas. Su etiología es desconocida, pero algunos casos se asocian con obesidad, síndrome de ovario poliquístico, diabetes mellitus, menarquia temprana y terapia de sustitución de estrógenos o al tamoxifeno. La sintomatología es inespecífica, varía desde el dolor pélvico hasta el sangrado genital anormal progresivo y es difícil de reconocer por las imágenes radiológicas. En la mayoría de los casos, el diagnóstico se realiza mediante una evaluación anatomopatológica. La tinción inmunohistoquímica también puede ayudar a diferenciarlo de otras neoplasias. Por lo tanto, es importante tener un alto índice de sospecha para este tipo de neoplasia rara. Su tratamiento es quirúrgico y su seguimiento debe ser a largo plazo, debido al alto riesgo de recidivas tardías y metástasis. Se presenta un caso de sarcoma estromal endometrial de bajo grado.
Endometrial stromal sarcoma is a rare malignant neoplasm that originates in the endometrial stroma. It can have several forms of differentiation, including smooth muscle and sex cord. Low-grade endometrial stromal sarcoma is a rare type of endometrial tumor, constituting only 0.2% of all uterine neoplasms. Its etiology is unknown, but some cases are associated with obesity, polycystic ovary syndrome, diabetes mellitus, early menarche, and estrogen replacement therapy or tamoxifen. Symptomatology is nonspecific, ranging from pelvic pain to progressive abnormal genital bleeding, and is difficult to recognize by radiological imaging. In most cases, the diagnosis is made by pathological evaluation. Immunohistochemical staining can also help differentiate it from other neoplasms. Therefore, it is important to have a high index of suspicion for this type of rare neoplasm. Its treatment is surgical and its follow-up should be long term, due to the high risk of late recurrences and metastasis. A case of low-grade endometrial stromal sarcoma is presented.
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Introduction: Endometrial stromal sarcoma (ESS) is a rare malignant tumor of the endometrium, occurring in the age group of 40-50 years. A 42 year old female admitted in obstetrics and gynecology department withCase History: complain of abdominal pain for 5 days and history of abdominal hysterectomy before 10 year. Scar endometriosisUSG: Discussion: Uterine sarcomas are rare tumours of mesodermal origin. They constitute 2 to 6% of uterine malignancy. Of these, endometrial stromal sarcomas are rare
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Endometrial stromal tumors (ESTs) include endometrial stromal nodule (ESN), low-grade endometrial stromal sarcoma (LG-ESS), high-grade endometrial stromal sarcoma (HG-ESS), and undifferentiated uterine sarcoma (UUS). Since these are rare tumor types, there is an unmet clinical need for the systematic therapy of advanced LG-ESS or HG-ESS. Cytogenetic and molecular advances in ESTs have shown that multiple recurrent gene fusions are present in a large proportion of LG-ESSs, and HG-ESSs are identified by the tyrosine 3-monooxygenase/tryptophan 5-monooxygenase activation protein epsilon (
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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.
Subject(s)
Female , Humans , DNA Helicases , DNA-Binding Proteins , Endometrial Neoplasms , Endometrium , Lymphatic Metastasis , Sarcoma, Endometrial StromalABSTRACT
Uterine sarcomas are tumors of poor prognosis characterized by a great histopathological heterogeneity. High endometrial stromal sarcomas (ESS) occurring at a late age, with high mitotic activity and / or tumor necrosis, are very poorly prognostic tumors.If it is accepted that the standard treatment of uterine sarcomas is surgical, the place of adjuvant treatment remains controversial.We report a case of a high-grade ESS referred to initially on MRI and diagnosed on pathological examination of a hysterectomy performed in a 64-year-old postmenopausal woman with postmenopausal bleeding.
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Objective: To investigate the clinical characteristics of uterine sarcoma and clinical application of color Doppler ultrasonography in the sarcoma. Methods: We conducted a retrospective analysis on the clinical and ultrasonographic features in 128 surgically and pathologically confirmed uterine sarcoma patients from December 2011 to May 2018 in Tongji Hospital. The clinical features included age, clinical manifestation, serum tumor marker CA125 and clinical stage, and the features of ultrasonography included the size, boundary, echo type of the lesion and characteristics of blood flow signals. Results: The majority of uterine sarcoma in this study were endometrial stromal sarcoma and leiomyosarcoma. Endometrial stromal sarcoma occurred mostly in women in reproductive period, while leiomyosarcoma occurred mainly in perimenopausal and postmenopausal women. The predilection age of uterine sarcoma was 49.6 ± 13.4 years. The main clinical manifestations were abnormal uterine bleeding, including postmenopausal vaginal bleeding or irregular vaginal bleeding (47.7%), and abdominal pain (32.0%). About 20.3% of patients had no symptoms. Serum CA125 was detected before operation in all the patients, and it was in normal range (≤ 35 U/ml) in 59 patients, slightly higher than normal level (35-100 U/ml) in 51 women and significantly higher than normal range (>100 U/ml) in 18 women. Pelvic three-dimensional ultrasonography was usually characterized by large uterine tumors with solid, unclear boundary, heterogeneous echo structures with or without cystic degeneration and rich blood flow signals, which can be roughly classified as malignant tumors. Conclusion: Combined with clinical manifestations such as vaginal bleeding, abdominal pain, abdominal distension, ultrasonograms can help us identify and early predict large, ill-defined, hypoechoic or heterogeneous hypoechoic tumors with rich blood flow signals, and grasp the treatment opportunity reasonably and formulate treatment plans.
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Background Endometrial stromal sarcomas (ESSs) are the second most common uterine sarcomas. Although ESSs are often indolent, they have metastatic potential. To the best of our knowledge, there are only three reports of brain metastasis, and the present report is the first to describe a late skull metastasis of an ESS. Case Report We describe the case of a 51-year-old woman who presented abnormal vaginal bleeding 14 years ago; she was diagnosed with an uterine mass and submitted to a hysterectomy. One year ago she presented ESS lung metastasis followed by a left parietal calvarial metastasis. The optimal treatment for metastatic ESS is controversial, but the use of progesterone and aromatase inhibitors is advisable.
Introdução Sarcoma endometrial estromal (SEE) é a segunda lesão mais frequente dentre os sarcomas uterinos. Geralmente são lesões indolentes, mas com potencial de desenvolver metástase. Até o momento há apenas três relatos de metástase cerebral, sendo este o primeiro estudo a descrever uma metástase craniana tardia dessas lesões. Relato de caso Nós descrevemos o caso de uma paciente de 51 anos de idade que apresentou há 14 anos um quadro de sangramento vaginal anormal, sendo diagnosticada uma massa uterina; a paciente foi submetida a uma histerectomia. Há um ano ela evoluiu com metástase pulmonar, seguida por metástase craniana parietal esquerda. O tratamento ideal do SEE metastático ainda é controverso, mas o uso de inibidores de aromatase é aconselhável.
Subject(s)
Humans , Female , Middle Aged , Sarcoma, Endometrial Stromal , Neoplasm Metastasis , Sarcoma, Endometrial Stromal/pathologyABSTRACT
Endometrial uterine sarcoma is a very rare tumour of the uterine cavity with an incidence of 1-2 cases per 100,000 women. Low grade Endometrial stromal sarcoma (LGESS) is an occasional diagnosis in a patient presenting as leiomyoma uterus. The symptoms are nonspecific, mostly abnormal uterine bleeding in perimenopausal women. Clinically and radiologically it is difficult to diagnose this entity. Histopathological examination and immunohistochemistry confirmed the diagnosis of LGESS. In addition of surgery chemotherapy, radiotherapy and immunotherapy treatments will be quite useful in all cases of LGESS. This case report of Low grade Endometrial stromal sarcoma (LGESS) is presented here because of its rarity.
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Objective To analyze the MRI features of endometrial stromal sarcoma(ESS)and to explore its value in clinical application. Methods Thirteen patients with histologically proven ESS were collected.All cases underwent non-contrast MRI scan and DWI,10 of which underwent contrast-enhanced MRI scan.The MRI features were discussed in combination with the pathological results after operation.Results In all lesions,6 lesions were located in endometrial cavity,4 in myometrium,1 in right wall of uterus and broad ligament,1 in cervical canals,and 1 in the top segment of vagina and pelvic.Plain MRI showed the lesions with hypointensity on T 1 WI and mixed hyperintensity on T 2 WI,which exhibited polypoid or multiple nodular masses.DWI showed the lesions with different hyperintensity.In 10 patients with post-enhanced scanning,moderate or marked heterogeneous enhancement of the masses were demonstrated,in which 6 presented plateau pattern of time-signal enhanced curve and 4 were persistent pattern.Conclusion ESS had some characteristic features on MRI,and DWI and dynamic contrast-enhanced examination can help to improve the diagnosis and differential diagnosis.
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Objective To analyze the MRI features of endometrial stromal sarcoma(ESS)and to explore its value in clinical application. Methods Thirteen patients with histologically proven ESS were collected.All cases underwent non-contrast MRI scan and DWI,10 of which underwent contrast-enhanced MRI scan.The MRI features were discussed in combination with the pathological results after operation.Results In all lesions,6 lesions were located in endometrial cavity,4 in myometrium,1 in right wall of uterus and broad ligament,1 in cervical canals,and 1 in the top segment of vagina and pelvic.Plain MRI showed the lesions with hypointensity on T 1 WI and mixed hyperintensity on T 2 WI,which exhibited polypoid or multiple nodular masses.DWI showed the lesions with different hyperintensity.In 10 patients with post-enhanced scanning,moderate or marked heterogeneous enhancement of the masses were demonstrated,in which 6 presented plateau pattern of time-signal enhanced curve and 4 were persistent pattern.Conclusion ESS had some characteristic features on MRI,and DWI and dynamic contrast-enhanced examination can help to improve the diagnosis and differential diagnosis.
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OBJECTIVE: To evaluate the oncologic safety of ovarian preservation (OP) in premenopausal women diagnosed with the International Federation of Gynecology and Obstetrics (FIGO) stage I uterine sarcoma. METHODS: The National Cancer Institute's Surveillance, Epidemiology, and End Results database was accessed and a cohort of women aged ≤50 diagnosed between 1988–2013 with a sarcoma limited to the uterus was drawn. Based on site-specific surgery codes, women who underwent hysterectomy with or without oophorectomy and did not receive radiation therapy were selected for further analysis. Overall (OS) and cancer-specific (CSS) survival were determined following generation of Kaplan-Meier curves; comparisons were made with the log-rank test. A Cox-proportional hazard model was constructed to control for possible confounders. RESULTS: A total of 1,482 women were included in the analysis; 800 (54.0%) were diagnosed with leiomyosarcoma (LMS), 520 (35.1%) with low-grade endometrial stromal sarcoma (LG-ESS), and 162 (10.9%) with adenosarcoma (AS). The OP group included 418 women (28.2%). Differences in the rate of OP were noted based on histology (p=0.014), year of diagnosis (p=0.001), patient age (p<0.001) and race (p=0.012). There was no difference in OS (p=0.220) or CSS (p=0.210) between women who had OP and those who did not. Multivariate analysis confirmed that OP was not associated with a worse mortality. CONCLUSION: In this population-based cohort of women with sarcoma limited to the uterus, OP was not associated with worse oncologic outcomes. OP could be considered for women with LMS, sparing them from the morbidity associated with iatrogenic menopause. No conclusions could be made for those with LG-ESS or AS.
Subject(s)
Female , Humans , Adenosarcoma , Cohort Studies , Racial Groups , Diagnosis , Epidemiology , Gynecology , Hysterectomy , Leiomyosarcoma , Menopause , Mortality , Multivariate Analysis , Obstetrics , Ovariectomy , Proportional Hazards Models , Sarcoma , Sarcoma, Endometrial Stromal , UterusABSTRACT
Objective To investigate the MRI characteristics of low-grade endometrial stromal sarcoma (LGESS),and to improve the accuracy of the diagnosis.Methods The clinical and MRI data of 4 cases with LGESS confirmed by surgery and pathology were analyzed retrospectively.All cases underwent conventional MRI scans.Results All 4 cases had enlarged uterus in different degrees. The average diameter of lesions was 9 cm (range from 5 cm to 12.1 cm).Uterine cavity masses were found in 3 cases,all of which were solid masses and invaded into myometrium exhibiting disconnection or disappearance of the connection band of uterus,and one of which involved the bladder and right ureter,oviduct and ovary.The solid tumors in 3 cases showed isointensity on T1 WI and slight hyperin-tensity on T2 WI,1 of which was heterogenous with dilated vascular void signal in the inner and peripheral portion.In addition, myometrial mass was found in 1 case,which was cystic with septum and clear boundaries.The cystic tumor showed hypointensity on T1 WI and hyperintensity on T2 WI with hypointensive internal septum.There were 3 cases accompanied with uterine leiomyoma, and 1 case with pelvic lymph node metastasis.Conclusion Most of endometrial stromal sarcomas are located in uterine cavity,a few in myometrium.The tumors are solid,few can also show cystic,showing invasive growth into myometrium.The manifestation of low-grade endometrial stromal sarcoma has a certain characteristics on MRI,which is low or iso-signal intensity on T1 WI and high signal intensity on T2 WI,the signal intensity may be heterogeneous,so MRI can provide help for the correct diagnosis of the disease.
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Endometrial stromal sarcoma (ESS) is a rare malignancy. Development of extrauterine ESS form endometriosis is particularly rare. The majority of extrauterine ESS occurs in areas with preexisting endometriosis. The most common site is the ovary. We experienced a case of ESS of the ovary that arose from endometriosis with multiple disseminated lesions. This disease was managed by total abdominal hysterectomy, bilateral salpingo-oophorectomy, both pelvic lymph nodes dissection, omentectomy, and appendectomy followed by postoperative high-dose progesterone therapy. Here, we report this case with literature review.
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Female , Appendectomy , Endometriosis , Hysterectomy , Lymph Nodes , Ovary , Progesterone , Sarcoma, Endometrial StromalABSTRACT
Endometrial stromal sarcomas are rare uterine tumours usually seen in perimenopausal females. We report here a case of low grade endometrial stromal sarcoma in a 45 year female with a clinical diagnosis of fibroid uterus and final histological diagnosis of low grade endometrial stromal sarcoma.
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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.
Subject(s)
Female , Humans , Middle Aged , Adenomyosis , Biopsy , Diagnosis , Endometrial Stromal Tumors , Endometriosis , Estradiol , Follicle Stimulating Hormone , Hysterectomy , Lung , Ovary , Radiotherapy , Sarcoma, Endometrial Stromal , ThoraxABSTRACT
An endometrial stromal sarcoma (ESS) is an uncommon uterine neoplasm, and its primary occurrence in the intestine as an extrauterine ESS (EESS) is exceedingly rare. We hereby report a primary EESS arising in the sigmoid colon with a review of the literature. A 52-year-old woman presented with bloody stool and underwent a colon fiberscopy, which revealed a fungating mass obstructing the lumen at the distal sigmoid. A laparoscopic low anterior resection was performed, and an umbilicated polypoid mass was identified; on section, it had infiltrated the mesocolic fat and measured 3.8 cm x 2.5 cm. The tumor showed geographic sheets or nests composed of relatively monotonous stromal cells, expansion or infiltration to the proper muscle and mesocolic fat, and extensive lymphovascular invasion and metastasis to regional lymph nodes and the pelvic peritoneum. The tumor cells were strongly and diffusely immunoreactive for CD10, but negative for c-kit, CD34, and Dog1. Two months later, a hysterectomy with a bilateral salpingo-oophorectomy was performed, and no evidence of an ESS was found in the uterus.
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Female , Humans , Middle Aged , Colon , Colon, Sigmoid , Hysterectomy , Intestines , Lymph Nodes , Neoplasm Metastasis , Peritoneum , Sarcoma, Endometrial Stromal , Stromal Cells , Uterine Neoplasms , UterusABSTRACT
Endometrioid stromal sarcoma is a rare malignancy that originates from mesenchymal cells. It is classified into low-grade endometrioid stromal sarcoma (LGESS) and high-grade endometrioid stromal sarcoma. Ultrasonographic findings of LGESS resemble those of submucosal myomas, leading to the possible preoperative misdiagnosis of LGESS as uterine leiomyoma. Electronic morcellation during laparoscopic surgery in women with LGESS can result in iatrogenic intraabdominal dissemination and a poorer prognosis. Here, we report a patient with LGESS who underwent a supracervical hysterectomy and electronic morcellation for a presumed myoma in another hospital. Disseminated metastatic lesions of LGESS in the posterior cul-de-sac and rectal serosal surface were absent on primary surgery, but found during reexploration. In conclusion, when LGESS is found incidentally following previous morcellation during laparoscopic surgery for presumed benign uterine disease, we highly recommend surgical reexploration, even when there is no evidence of a metastatic lesion in imaging studies.
Subject(s)
Female , Humans , Diagnostic Errors , Hysterectomy , Laparoscopy , Leiomyoma , Myoma , Prognosis , Sarcoma , Sarcoma, Endometrial Stromal , Uterine Diseases , UterusABSTRACT
Endometrial stromal sarcoma (ESS) has a wide histopathological spectrum with CD10 as its diagnostic marker. Recently, few non-conventional ESSs have been identifi ed that lack diffuse CD10 expression. A 46-year-old, perimenopausal lady referred to us with history of vaginal bleeding. On clinical examination and radiological imaging, a polypoid endometrial tumor was identifi ed. Hysterectomy revealed a multinodular tumor in the myometrium. Microscopically, the tumor composed of rather banal oval to spindle-shaped cells in a fi bromyxoid stroma. Focal areas displayed compact cellular arrangement, unassociated with signifi cant mitoses and necrosis. Immunohistochemically, tumor cells were focally positive for CD10, estrogen receptor, progesterone receptor, p16INK4 and were diffusely positive for cyclinD1. Diagnosis of cyclinD1 and p16INK4 positive ESS was offered. This case highlights the value of additional IHC markers, especially cyclinD1 and p16INK4 in order to identify certain ESSs that lack diffuse CD10 immunoexpression; are invariably misdiagnosed as undifferentiated sarcomas, but actually form a relatively more aggressive subset of ESSs.
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El sarcoma del estroma endometrial es un tumor infrecuente, que comprende menos del 1 % de los tumores malignos ginecológicos. Presentamos el caso de una adolescente de 16 años con hemorragia uterina anormal a la que se le realizó histerectomía subtotal. Microscópicamente, la neoplasia estaba constituida por una proliferación maligna de células estromales del endometrio. El estudio inmuno-histoquímico mostró positividad para vimentina, desmina, CD10, CD117. El CD10 es un marcador inmuno-histoquímico del estroma endometrial normal y de los tumores del estroma endometrial, y es útil para el diagnóstico diferencial entre el sarcoma del estroma endometrial y el leiomioma celular o el leiomiosarcoma uterino. Las mejores opciones de tratamiento se obtienen con un enfoque multidisciplinario y en centros especializados.
Endometrial stromal sarcoma is a rare tumor comprising less than 1 % of gynecological malignancies. We report the case of a 16 year-old adolescent with abnormal uterine bleeding who underwent hysterectomy subtotal. At microscopy the neoplasm was composed of malignant proliferation of endometrial stromal cells. Immunohistochemical studies were positive for vimentin, desmin, CD10 and CD117. CD10 is an immunohistochemical marker of normal endometrial stroma and of endometrial stromal neoplasms. This marker is useful in the differential diagnosis of endometrial stromal sarcoma versus uterine cellular leiomyoma or uterine leiomyosarcoma. The best treatment options are obtained with a focus multidisciplinary, in specialized centers.
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Primary extrauterine endometrial stromal sarcoma is a rare tumor and it is infrequently associated with endometriosis. We are reporting a case of this unusual tumor in a 42-year-old female who presented with multiple nodules of tumor in the abdomen and pelvis and with metastases in para-aortic lymph nodes. The right parametrium, in addition, had a focus of endometriosis, which was contiguous with the tumor, confirming its origin.