Is high-grade endometrial stromal sarcoma the key to disease prognosis in cases of coexisting leiomyosarcoma? A case report

2020 ◽  
Author(s):  
Feng Ling ◽  
Sibei Ruan ◽  
XiaoMing Xiong ◽  
Na Li ◽  
DongMei Zhao ◽  
...  

Abstract Background:The uterine leiomyosarcoma combined high-grade endometrial stromal sarcoma (ESS) is quite rare.Case presentation: We reported such a case in which high-grade ESS with BCOR gene alterations and leiomyosarcoma coexist in a patient. And, most impressively, high-grade ESS with BCOR gene alterations caused ovarian and pelvic metastases, although its volume was less than 1% of leiomyosarcoma.Conclusions: We suggested that when both high-grade ESS BCOR gene alterations and leiomyosarcoma are present in a patient, the high-grade ESS needs to be noted in the pathological report even if it accounts for less than 1% of the tumour mass.

2020 ◽  
Author(s):  
Feng LING ◽  
Sibei Ruan ◽  
Na Li ◽  
XiaoMing Xiong ◽  
DongMei Zhao ◽  
...  

Abstract Background:The uterine leiomyosarcoma combined high-grade endometrial stromal sarcoma (HGESS) with BCOR gene alterations is exceedingly rare. The subtype of YWHAE-FAM22 HGESS is shown in the current World Health Organization classification, but not HGESS with BCOR alterations. Case presentation: We reported such a case in which HGESS with BCOR gene alterations and leiomyosarcoma coexist in a patient. And, most impressively, HGESS with BCOR gene alterations caused ovarian and pelvic metastases, although its volume was less than 1% of leiomyosarcoma.Conclusions: Given the more aggressive of HGESS with BCOR gene alterations, we suggest that it is classified as an independent subtype of HGESS, and when it coexists with other types of tumors, the HGESS with BCOR gene alterations needs to be noted in the pathological report even if it accounts for less than 1% of the tumour mass.


2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S41-S41
Author(s):  
A C Re ◽  
M Enrique ◽  
S Ren

Abstract Introduction/Objective Endometrial stromal sarcoma (ESS), a rare malignant neoplasm of endometrial stroma, accounts for less than 1% of all uterine tumors. High grade ESS (HGESS) is aggressive and commonly relapses even after surgical and neoadjuvant therapy. Abdominal and pelvic regions are common sites of metastasis, however, distant metastases to the liver, lung, vertebrae, and brain have been reported. Methods/Case Report We encountered a 49-year-old female who presented with shortness of breath, found to have a left pleural effusion and multiple pleural masses. She initially presented three years ago with heavy irregular menses and left pelvic pain for one year. D&C revealed prominent small spindle cells for which a stromal nodule and low-grade or malignant process was probable. CT scan showed an enlarged uterus. Hysterectomy with bilateral salpingo- oophorectomy, bilateral pelvic and para-aortic lymph node dissection, and partial omentectomy were performed. The uterus revealed an intramural 7 cm mass with a serpiginous growth pattern and lymphovascular invasion. Tumor cells were plump to spindled with areas of high cellularity, rounded nuclei, increased atypia and mitosis. Atypical areas were positive for cyclin D1, focally positive for CD10, and negative for ER, PR, SMA, desmin, AE1/3 and CAM5.2. FISH studies showed rearrangement of YWHAE gene (17p13.3) and no rearrangement of JAZF1 or PHF1 gene regions. Findings supported the diagnosis of HGESS. The patient received post-operative chemotherapy. Biopsy of the current pleural lesion revealed a nonspecific malignant spindle cell neoplasm positive for BCL1, CD56, CD117, CD99, TLE1 and INI1, while negative for AE1/3, CAM5.2, EMA, ER, PR, CK5/6, calretinin, SMA, desmin and S100. The CD10 stain was inconclusive. FISH studies showed rearrangement of YWHAE gene (17p13.3) and no rearrangement involving JAZF1 or PHF1 gene regions. No rearrangement of the SS18 gene region was observed and synovial sarcoma was excluded. Overall findings support the diagnosis of metastatic HGESS. Results (if a Case Study enter NA) NA Conclusion HGESS, a rare tumor with a nonspecific immunostain profile, has the ability to metastasize to rare body sites, such as the pleura in our case. Display of spindle cell morphology is a nonspecific finding that raises broad differential diagnoses. In women, with or without a history of uterine neoplasm, HGESS is a clinically worthwhile diagnosis to be mindful of.


BMC Cancer ◽  
2018 ◽  
Vol 18 (1) ◽  
Author(s):  
Arie J. Verschoor ◽  
Fabiënne A. R. M. Warmerdam ◽  
Tjalling Bosse ◽  
Judith V. M. G. Bovée ◽  
Hans Gelderblom

Author(s):  
Vincenzo Dario Mandato ◽  
Riccardo Valli ◽  
Monica Silvotti ◽  
Valentina Mastrofilippo ◽  
Giovanni Casali ◽  
...  

2010 ◽  
Vol 4 (1) ◽  
Author(s):  
Frédéric Amant ◽  
Kristel Van Calsteren ◽  
Maria Debiec-Rychter ◽  
Liesbeth Heyns ◽  
Katya Op De Beeck ◽  
...  

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Ji Sun Lee ◽  
Dayong Lee ◽  
Jisun Lee ◽  
Man-Hoon Han ◽  
Dae Gy Hong ◽  
...  

Abstract Background Primary ovarian high-grade endometrial stromal sarcoma is a very rare disease. Even though it has poor prognosis, the gold standard treatment has not been established owing to its rarity. This report aimed to present therapeutic options for primary ovarian high-grade endometrial stromal sarcoma. Case presentation A 49-year-old Asian woman presented with disseminated intravascular coagulation due to ruptured primary high-grade ovarian endometrial stromal sarcoma with multiple intraperitoneal metastases. After the initial surgery, the patient underwent adjuvant chemotherapy with three courses of Adriamycin (75 mg/m2). We performed the secondary debulking operation including total hysterectomy, metastasectomy, omentectomy, peritonectomy, appendectomy, and hyperthermic intraperitoneal chemotherapy (paclitaxel 175 mg/m2). Currently she has been alive for 28 months under a new chemotherapy regimen. Conclusion We suggest cytoreductive surgery with hyperthermic intraperitoneal chemotherapy could be a therapeutic option for primary high-grade ovarian endometrial stromal sarcoma with peritoneal dissemination.


2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Snigdha Rai

High grade endometrial stromal sarcoma (HG-ESS) is a very rare entity with poor outcome in compared to other endometrial stromal sarcoma as it is usually diagnosed in late stage. The stage of the disease, age and complete surgery are most important prognostic factors. Surgical management of a 40 years old lady with menometrorrhagia and high grade endometrial stromal sarcoma is presented.


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