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Clinicopathological characteristics and treatment of patients with high-grade endometrial stromal sarcoma: A retrospective study of 40 cases

Reprinted from Medicine (Baltimore) 2022 Jan. 14;101(2):e28490. doi: 10.1097/MD.0000000000028490


To investigate the clinicopathological characteristics of patients with high-grade endometrial stromal sarcoma (HG-ESS).The clinicopathological characteristics, treatments, and prognostic information of consecutive HG-ESS patients were collected from medical records and then evaluated.A total of 40 women were included in the analysis. The immunohistochemical profiles indicated that HG-ESS tumors tend to be locally or weakly positive for vimentin (100%) and CD10 (72.0%) but mostly negative for desmin (7.7%) and AE1/AE3 (9.1%). The progression-free survival intervals and the clinical benefit rates of patients receiving radiotherapy and/or chemotherapy were slightly longer and higher than those receiving simple observation (progression-free survival: 6 and 5 months vs 2 months; clinical benefit rate: 83.3% and 75.0% vs 28.6%). The 1-year disease-specific survival (DSS) rate was 62.7%. Tumor size, myometrial invasion, lymphovascular space invasion, cervical involvement, Federation International of Gynecology and Obstetrics (FIGO) stage, and residual disease all significantly affected the DSS rate (P < .001, =.002, <.001, =.004, <.001, and <.001, respectively). For patients with stage I disease, the 1-year DSS rate was as high as 91.7%, in contrast to 66.7%, 26.7%, and 0% for those with stage II, III, and IV disease, respectively.HG-ESS is associated with an adverse prognosis. FIGO stage could effectively predict the prognosis of patients with this lethal disease. Immunohistochemical markers, vimentin+/CD10+ (local or very weak), in combination with desmin-/AE1/AE3-, may be helpful for improving the diagnostic accuracy of this lethal condition. The therapeutic roles of adjuvant chemotherapy and radiotherapy warrant further investigation.

Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.

Coming home to roost: The self-seeding hypothesis of tumor growth

Reprinted from NCI Cancer Bulletin for February 8, 2011

The spread of cancer cells from their original location to other sites in the body, known as metastasis, has long been thought of as a one-way journey. But some researchers also believe that metastatic cancer cells can fuel primary tumor growth, with potentially important implications for the timing and nature of cancer treatment.

The concept of tumor self-metastasis, or tumor “self-seeding,” originated at Memorial Sloan-Kettering Cancer Center, based on a series of studies led by Drs. Joan Massagué, head of the Metastasis Research Center, and Larry Norton, deputy physician-in-chief of the center’s breast cancer programs.

In studies of mice, Dr. Massagué observed that breast tumors expressing genes associated with metastasis were growing faster than tumors that didn’t express these genes, even though the genes had no apparent role in increased cell division or decreased cell death. “Moreover, the fraction of dividing cells was not higher in fast-growing tumors versus tumors that are slower growing,” explained Dr. Norton.

Endometrial stromal sarcoma arising from endometriosis of the terminal ileum: The role of immunohistochemistry in the differential diagnosis

J Obstet Gynaecol Res. 2012 Mar 13. doi: 10.1111/j.1447-0756.2011.01783.x. [Epub ahead of print]

Low-grade endometrial stromal sarcoma (LESS) is an uncommon uterine malignancy. Occasionally, it may develop in extrauterine endometriotic lesions and present morphological characteristics mimicking various neoplasms, making its diagnosis very challenging. We report a rare case of a 56-year-old woman presenting with a pelvic mass, initially presumed to be of ovarian origin. After surgical excision the diagnosis of a LESS arising from foci of endometriosis of the terminal ileum was established. Pelvic lymph nodes and omentum were also infiltrated. The patient received adjuvant chemotherapy and medroxyprogesterone; she is alive with no evidence of disease after a follow-up of 38 months. Immunohistochemical characteristics of the tumor are very important for the differential diagnosis of this rare neoplasm and include diffuse strong positivity for CD 10, estrogen receptor expression and CD 34 negativity.

Endometrial Stromal Sarcoma Diagnosed after Uterine Morcellation in Laparoscopic Supracervical Hysterectomy

Endometrial stromal sarcoma is a rare uterine cancer with no reliable method for preoperative diagnosis. A 30–year–old parous woman underwent laparoscopic supracervical hysterectomy because of a leiomyoma. The uterus was removed from the abdominal cavity with an electric morcellator with a spinning blade. The pathology report revealed low–grade endometrial stromal sarcoma. Two months after the initial surgery, a second laparoscopic procedure was performed. The final pathology report confirmed low–grade endometrial stromal sarcoma involving the ovary, fallopian tube, and ovarian artery. It was concluded that morcellation of leiomyomas at laparoscopic supracervical hysterectomy may potentially increase metastasis if the tumor is a sarcoma.

Link to abstract:

Endometrial Stromal Sarcoma of the Uterus with Arterial Tumor Embolus

Reprinted from the Internet Journal of Obstetrics & Gynecology
Link to article

 Abstract: Endometrial stromal sarcoma (ESS) of the uterus is extremely rare. We report a case of ESS of the uterus with tumor embolus in an artery in a 36 year-old woman. The patient is a Caucasian woman who presented with vaginal bleeding. Surgical biopsy revealed low grade ESS (LGESS). Subsequently, total abdominal hysterectomy and right salpingo-oophorectomy was performed. A 3.8 cm polypoid mass was identified in the endometrial cavity, invading into the outer half of the anterior uterine wall. About 5-10% of the tumor exhibited undifferentiated sarcoma. Based on the aggressive nature of the tumor, the patient underwent left salpingo-oophorectomy and was subsequently treated with chemotherapy. She was followed up with periodic CT scans and was found to have recurrent ESS in the retroperitoneum 20 months after total hysterectomy and bilateral salpingo-oophorectomy. Rebulking surgery was not performed as the tumor showed diffuse abdominal and retroperitoneal involvement. Although LGESS may behave as an indolent tumor, it can be associated with an aggressive process and a high recurrence rate. A poor prognosis may occur when LGESS is admixed with undifferentiated components especially with evidence of arterial tumor embolus, which is herein reported.

ESS: A population-based analysis

Chan JK, Kawar NM, Shin JY, Osann K, Chen LM, Powell CB, Kapp DS.

1Division of Gynecologic Oncology, Department of Obstetrics, Gynecology, and Reproductive Sciences, San Francisco School of Medicine, UCSF Helen Diller Family Comprehensive Cancer Center, University of California, 1600 Divisadero Street, Box 1702, San Francisco, CA 94143, USA.

To determine independent prognostic factors for the survival of patients with endometrial stromal sarcoma (ESS), data were abstracted from the Surveillance, Epidemiology, and End Results (SEER) database of the National Cancer Institute from 1988 to 2003. Kaplan-Meier and Cox proportional hazards models were used for analyses. Of 831 women diagnosed with ESS, the median age was 52 years (range: 17-96 years). In total, 59.9% had stage I, 5.1% stage II, 14.9% stage III, and 20.1% had stage IV disease. Overall, 13.0, 36.1, and 34.7% presented with grades 1, 2, and 3, respectively. Patients with stage I-II vs III-IV disease had 5 years DSS of 89.3% vs 50.3% (P<0.001) and those with grades 1, 2, and 3 cancers had survivals of 91.4, 95.4, and 42.1% (P<0.001). In multivariate analysis, older patients, black race, advanced stage, higher grade, lack of primary surgery, and nodal metastasis were independent prognostic factors for poorer survival. In younger women (

Pub-Med Abstract

Gynecologic oncologists welcome sarcoma advocacy

From the Sarcoma Alliance News, Summer 2008, Volume 7, No. 1

Suzie Siegel writes an interesting article about uterine sarcomas in the Summer 2008 issue of the Sarcoma Alliance newsletter. Suzie discusses many of the challenges that we face, as well as current research on the effects of ovary removal, radiation, and chemotherapy.

Please note that the article discusses all uterine sarcomas, not just ESS.

Download the newsletter here

Visit the Sarcoma Alliance website 

Immunohistochemical studies on uterine carcinosarcoma, leiomyosarcoma, and endometrial stromal sarcoma: expression and prognostic importance of ten different markers

Uterine sarcomas are rare and aggressive gynecologic malignancies. In this immunohistochemical (IHC) study, expression of Ki-67, p53, CD10, CD44, desmin, smooth muscle actin, estrogen receptor α (ERα), androgen receptor (AR), progesterone receptor A (PRA), and c-kit and their influence on survival in cases of uterine carcinosarcoma (CS), leiomyosarcoma (LMS), and endometrial stromal sarcoma (ESS) were evaluated. Medical records were reviewed and data collected concerning all uterine sarcomas treated during a 12-year period at Helsinki University Central Hospital. There was sufficient histological material for IHC analysis and slide review in 67 cases. Survival analysis was performed using the Kaplan-Meier method, and median survival times with 95% confidence intervals are given. Survival in cases of LMS was statistically significantly affected by the expression of p53, ERα, and PRA. Striking differences in expression of IHC markers when comparing results with those in earlier studies were the absence of AR immunoreactivity in all uterine sarcomas and low incidence of c-kit (15%; in endometrial stromal sarcoma). None of the markers was statistically significantly associated with survival of ESS and CS patients. The expression of p53, ERα, and PRA in uterine LMS may give prognostic information concerning the behavior of the disease. Hormonal therapy could be recommended as a treatment option in cases of hormone receptor-positive LMS.

Department of Obstetrics and Gynecology, University of Helsinki, P.O. Box 140, 00029, Helsinki, HUS, Finland. Abstract

Immunohistochemical study and fluorescent in situ hybridization analysis of JAZF1 in 67 cases of endometrial stromal tumors collected in a tissue microarray

Background:Endometrial stromal tumours are rare uterine lesions comprising undifferentiated endometrial sarcoma (UES), endometrial stromal sarcoma low grade (ESS) and stromal nodule (SN). Complex caryotypes and frequent 7p deletion are found in the malignant types, and a translocation t(7;17) involving two finger zinc gene JAZF1and JJAZ1/SUZ12 has also been recently described. Methods:We performed an immunohistochemical study to evaluate the expression of oestrogen and progesterone receptors, Ki67, P16 and P53 and a in situ fluorescent hybridization (FISH) studying JAZF1(7p15) locus using formalin fixed paraffin embedded tissues of 12 SN, 41 ESS and 14 UES collected in a tissue microarray. Results:Immunohistochemical analysis of SN and ESS showed a high nuclear expression of both oestrogen receptor (OR) and progesterone receptor (PR), associated with a low nuclear expression of Ki67, P16 and P53. In opposite, UES exhibit a low expression of OR and PR and a high nuclear expression of Ki67, P16 and P53. The difference for each of the immunostainings was highly statistically significant between ESS and UES (p<0,0005), but not between SN and ESS. Among the 44 cases with available FISH status, the dissociation of JAZF1(7p15) meaning the specific translocation was found in 25% of SN (n=2/8), 35% of ESS (n=8/23), and was absent in UES (n=0/13). Interestingly, we observed also a deletion of the second allele of the JAZF1 gene in 80% of the 10 cases with dissociation of JAZF1. Conclusions:Immunohistochemical analysis of OR, PR, P16, P53 and Ki67 could be very useful for the differential diagnosis of endometrial stromal tumours especially between ESS and UES. The absence of JAFZ1 dissociation in UES suggest different oncogenic pathways between UES and NS/ESS, that support the current World Health Organization classification of tumours. Moreover, the translocation was associated in 80% with deletion of second JAZF1 allele suggesting a loss of function of the gene. However, further study are necessary to find out the regulatory consequences of JAZF1 function's loss and translocation.

Immunological Therapies Can Relieve Aromatase Inhibitor-Related Joint Symptoms in Breast Cancer Survivors

Reprinted from American Journal of Clinical Oncology, December 2010 - Volume 33 - Issue 6 - pp 557-560, doi: 10.1097/COC.0b013e3181cae782

Objectives: Aromatase inhibitors can cause joint symptoms. The purpose of this pilot study was to evaluate the feasibility of immunologic therapies for this kind of joint symptoms.

Methods: A total of 16 postmenopausal women with stage I–III breast cancer with joint symptoms related to Aromatase inhibitors were enrolled. They received immunologic therapies of thymosin α1 1.6 mg, twice a week for 4 weeks. Outcome measures included the Brief Pain Inventory-Short Form, Western Ontario and McMaster Universities Osteoarthritis index, and the Functional Assessment of Cancer Therapy-General quality of life measure. Interferon-gamma and interleukin-4 were determined to evaluate immunomodulatory activity. Paired Samples Test and linear regression analysis were used to statistics the outcome measures.

Lymphadenectomy in Uterine Low-Grade Endometrial Stromal Sarcoma: An Analysis of 19 Cases and a Literature Review

Background: The role of lymphadenectomy in low-grade endometrial stromal sarcoma (ESS) is controversial. The risk of nodal metastases ranges from 0% to 44%, but data are inconclusive. The objective of this study and of the literature review was to investigate the incidence of nodal involvement in macroscopically early-stage tumors.

Methods: All consecutive patients with histologically proven uterine low-grade ESS who underwent surgery in our institution were considered eligible for the analysis. Until July 2006, pelvic systematic lymphadenectomy was performed based on the physician's choice, whereas after July 2006, all women with apparent early-stage tumor underwent systematic pelvic nodes dissection.

Prognostic factors and the impact of different classification systems in the gynecologic sarcomas

From the American Society of Clinical Oncology 

Background: Prognostic factors for GS used in available studies are the presence of metastasis, performance status and factors included in FIGO staging. The objective of this retrospective study was to analyze clinical and pathological prognostic factors, trying to improve the usual (FIGO) classification. Methods: We analyzed 75 patients with ovarian (11) or uterine sarcoma (64) diagnosed from 6/1983 to 12/2007. Pathology: 26 leiomyosarcoma, 35 carcinosarcoma, 10 endometrial stromal, 4 high-grade undifferentiated sarcoma. Results: Medium age was 57 (26-82) years. With a median follow-up of 30.4 months (6-305), 5-year and 10-year survival was 71% and 69%. Survival significant prognostic factors in the univariate analysis: histologic subtype (stromal 100%, leiomyo 69%, carcinosarcoma 64%, undifferentiated 50%, p=0.01); tumor grade (I=100%, II=80%, III=58%, p=0.0003); AJCC sarcoma staging (I=100%, II=71%, III=68%, IV=55%, p=0.005); FIGO staging (I=89%, II=67%, III=58%, IV=50%, p=0.004). Disease relapse occurred in 36 (48%) patients: local only 54%, distant 31%, both 15%. Median time to relapse 13 months, 5-year and 10-year survival was 38% and 32%. Median survival in local vs distant recurrence was 21 and 13 months (non-significant). 5-year survival in radical intention treatment of relapse was 65% vs 13% in palliative setting patients (p<0.001). Conclusions: Histologic type and tumor grade should be considered prognostic factors in GS. FIGO and AJCC staging methods have a good correlation with prognosis. Radical treatment (surgery ± radiotherapy) of relapsed disease should be always considered.

Link: here

J Clin Oncol 26: 2008 (May 20 suppl; abstr 21505)
Author(s): X. Gonzàlez Farré, A. López Pousa, M. Quintana, J. Fernández Plana, D. Páez López-Bravo, À. Roselló, O. Gallego, S. Bagué, A. Tibau, J. Pérez, A. Barnadas Molins

Radiation Therapy in Addition to Gross Total Resection of Retroperitoneal Sarcoma

From the Journal of Oncology
Volume 2008 (2008), Article ID 824036, 11 pages



Purpose. Typical treatment of retroperitoneal sarcomas (RPSs) is surgery with or without radiation therapy for localized disease.  With surgery alone, local failure rates are as high as 90%; this led to radiation therapy playing an important role in the treatment of RPSs. Methods. Thirty-one patients with retroperitoneal sarcoma treated with gross total resection and radiation therapy make up this retrospective analysis. Nineteen were treated preoperatively and 12 postoperatively (median dose, 59.4 Gy)—sixteen also received intraoperative radiation therapy (IORT) (median dose, 11 Gy).  Patients were followed with stringent regimens, including frequent CT scans of the chest, abdomen, and pelvis. Results. With a median follow-up of 19 months (range 1–66 months), the 2-year overall survival (OS) rate is 70% (median, 52 months).  The 2-year locoregional control (LRC) rate is 77% (median, 61.6 months).  The 2-year distant disease free survival (DDFS) rate is 70% (median not reached).  There were no differences in radiation-related acute and late toxicities among patients treated pre- versus postoperatively, whether with or without IORT. Conclusions. Compared to surgery alone, neoadjuvant or adjuvant radiation therapy offers patients with RPS an excellent chance for long-term LRC, DDS, and OS. The integration of modern treatment planning for external beam radiation therapy and IORT allows for higher doses to be delivered with acceptable toxicities.

Survival rates for Uterine Sarcoma, by stage

Survival rates are often used by doctors as a standard way of discussing a person's prognosis (outlook). Some patients with cancer may want to know the survival statistics for people in similar situations, while others may not find the numbers helpful, or may even not want to know them.

The 5-year survival rate refers to the percentage of patients who live at least 5 years after their cancer is diagnosed. Of course, many people live much longer than 5 years (and many are cured).

Transition from low-grade endometrial stromal sarcoma to high-grade endometrial stromal sarcoma

We report on a case of a primary low-grade endometrial stromal sarcoma (ESS) that progressed to a secondary high-grade ESS. In the secondary tumor, the immunohistochemical profile and focal tumor cell proliferation pattern suggested that this tumor was not truly undifferentiated, but possessed features of endometrial stroma. Low-grade ESS of our patient's primary tumor showed p53 protein overexpression, which is unusual in low-grade ESS, and her secondary high-grade ESS showed more prominent p53 immunoreactivity. This indicates that low-grade ESS that shows p53 immunoreactivity might progress to high-grade ESS, and it is considered that such cases of low-grade ESS should pay attention to the prognosis. Immunoreactivity for epidermal growth factor receptor was observed in both tumors, suggesting a relationship between the primary and secondary tumors in our case. Further study requires more immunohistochemical data for cases in which low-grade ESS transitions to high-grade ESS; in particular, data on epidermal growth factor receptor expression are necessary to define new therapeutic strategies for ESS.


Int J Gynecol Pathol. 2010 Jul;29(4):374-7.

Link to abstract:

Unexpected findings on Gallium-67 Scintigraphy

Detection of Undifferentiated Endometrial Stromal Sarcoma

Wu YC, Hsieh TC, Sun SS, Lo WC, Lin TY, Yang CF, Yen KY, Kao CH.

From the *Department of Nuclear Medicine and PET Center, China Medical University Hospital, and †China Medical University, Taichung, Taiwan; ‡Department of Biomedical Imaging and Radiological Science, China Medical University, Taichung, Taiwan; §Division of Hematology and Oncology, Department of Internal Medicine, China Medical University Hospital, Taichung, Taiwan; Department of Pathology, China Medical University Hospital, Taichung, Taiwan; ∥School of Medicine, China Medical University, Taichung, Taiwan.


We reported an extremely rare case of undifferentiated endometrial stromal sarcoma imaged with gallium-67 scintigraphy. This previously healthy 32-year-old woman presented with cough and dyspnea for days. Unexpectedly, the pathology of the opacity in the right pulmonary hilar region demonstrated metastatic high-grade epithelioid sarcoma. Gallium scintigraphy performed to detect possible origin showed abnormal uptake in the right supraclavicular region, chest region and pelvic region. Computed tomography-guided biopsy of the pelvic mass revealed undifferentiated endometrial stromal sarcoma. This case demonstrated the usefulness of gallium-67 scintigraphy in the detection of the primary disease and the evaluation of the metastatic disease.

Link to abstract