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Table of Contents
Year : 2020  |  Volume : 3  |  Issue : 4  |  Page : 867-868

Sense and nonsense of endometrial stromal sarcoma

Department of Medical Oncology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India

Date of Submission18-Oct-2020
Date of Decision23-Oct-2020
Date of Acceptance26-Oct-2020
Date of Web Publication25-Dec-2020

Correspondence Address:
Smita Kayal
Department of Medical Oncology, Jawaharlal Institute of Postgraduate Medical Education and Research, Dhanvantari Nagar, Puducherry - 605 006
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/CRST.CRST_328_20

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How to cite this article:
Kayal S. Sense and nonsense of endometrial stromal sarcoma. Cancer Res Stat Treat 2020;3:867-8

How to cite this URL:
Kayal S. Sense and nonsense of endometrial stromal sarcoma. Cancer Res Stat Treat [serial online] 2020 [cited 2021 Jan 25];3:867-8. Available from: https://www.crstonline.com/text.asp?2020/3/4/867/304995

Endometrial stromal sarcomas (ESSs) are rare tumors of uterine mesenchymal origin, accounting for <1% of all uterine malignancies. In 2014, the World Health Organization revised the classification of these tumors based on their histological and molecular features.[1] Recent advances in the understanding of their biology may help in optimizing the currently ill-defined therapeutic strategies and allow for a more precise treatment approach for this rare tumor.[2] However, there are significant clinical barriers, especially in the resource-constrained settings, in the management of these tumors, which range in spectrum from indolent to highly aggressive.

The recently published study by Rattan et al. describes a retrospective case series of ESS with real-world challenges and outcomes from a tertiary care center in Northern India.[3] The accompanying editorial by Pasricha and Doval summarizes the pertinent literature and discusses the study results in the current therapeutic context.[4] The study delineates the common presenting symptoms that are not specific for any pathology of uterine origin and highlights the resultant difficulty and delay in the diagnosis of these rare tumors in the community practice, where the index of suspicion is low. It would also be interesting to know whether the prolonged interval from symptom onset to diagnosis or from symptom onset to definitive treatment affects the survival in these tumors, even though the data from other common malignancies such as breast, lung, and colorectal cancers are conflicting regarding the effect of delay in diagnosis and treatment on survival outcomes.[5],[6]

In Rattan et al.'s study, almost 65% of the patients had received prior treatment elsewhere, mostly surgery (total abdominal hysterectomy), before reporting for further treatment at the author's institution. A subset of patients underwent additional surgical procedures; however, the criteria for performing a more extensive surgery have not been clearly explained. In the developing countries where there is a lack of access to expert care in the suburban and rural areas, when the presenting symptoms are nonspecific, often the patients, even with common malignancies such as ovarian, uterine, biliary tract, and gastrointestinal cancers, present to tertiary centers after receiving partial and suboptimal surgical treatment elsewhere.[7],[8],[9] Common international guidelines seldom outline the approach to the management of these partially treated patients. A locally/regionally developed consensus algorithm by national experts on further evaluation with appropriate imaging, indications for additional surgical exploration or completion surgery, and adjuvant treatment can immensely help in a more uniform and accurate management of these rare uterine malignancies, which are mostly diagnosed post-hysterectomy.[10]

In this study, adjuvant therapy either with radiotherapy or chemotherapy or both at the author's institution was decided in a multidisciplinary tumor board based on the final histopathological grade, stage, and the extent of extrauterine spread of the disease. The authors have emphasized the lack of consensus guidelines and first-level evidence on the optimal adjuvant management of these tumors and thus the necessity of multidisciplinary discussions and tailored treatment approaches. Although the authors claim theirs to be the only study from India on ESS, a few recent reports on uterine sarcomas from other centers in India have included ESS in their clinicopathological description and outcome analyses.[11],[12] Moving forward, to understand the patterns of care and treatment outcomes across different centers and settings, a national registry for this rare cancer type can be developed, possibly under the aegis of the National Cancer Grid, if feasible. Data from this proposed registry could help in defining the baseline reference for disease epidemiology and outcomes, outlining the regionally prevalent hurdles, and planning prospective studies to answer locally relevant clinical queries and problems.

We commend the authors for summarizing and sharing their experience with the management of this rare tumor and for outlining the challenges faced in the real-world circumstances.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Conklin CM, Longacre TA. Endometrial stromal tumors: The new WHO classification. Adv Anat Pathol 2014;21:383-93.  Back to cited text no. 1
Ferreira J, Félix A, Lennerz JK, Oliva E. Recent advances in the histological and molecular classification of endometrial stromal neoplasms. Virchows Arch 2018;473:665-78.  Back to cited text no. 2
Rattan R, Thakur P, Gupta A, Miriyala R, Ballari N, Rai B. Endometrial stromal sarcoma: Retrospective analysis of a tertiary care experience from India. Cancer Res Stat Treat 2020;3:489-94.  Back to cited text no. 3
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Pasricha S, Doval DC. Endometrial stromal sarcoma: A rare tumor with therapeutic conundrums. Cancer Res Stat Treat 2020;3:583-5.  Back to cited text no. 4
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Porta M, Gallén M, Malats N, Planas J. Influence of “diagnostic delay” upon cancer survival: an analysis of five tumour sites. J Epidemiol Community Health 1991;45:225-30.  Back to cited text no. 5
Richards MA, Smith P, Ramirez AJ, Fentiman IS, Rubens RD. The influence on survival of delay in the presentation and treatment of symptomatic breast cancer. Br J Cancer 1999;79:858-64.  Back to cited text no. 6
Vinotha T, Anitha T, Ajit S, Rachel C, Abraham P. The role of completion surgery in ovarian cancer. J Obstet Gynaecol India 2016;66:435-40.  Back to cited text no. 7
Zaidi MY, Abou-Alfa GK, Ethun CG, Shrikhande SV, Goel M, Nervi B, et al. Evaluation and management of incidental gallbladder cancer. Chin Clin Oncol 2019;8:37.  Back to cited text no. 8
Pisano M, Zorcolo L, Merli C, Cimbanassi S, Poiasina E, Ceresoli M, et al. 2017 WSES guidelines on colon and rectal cancer emergencies: Obstruction and perforation. World J Emerg Surg 2018;13:36.  Back to cited text no. 9
Thiel FC, Halmen S. Low-grade endometrial stromal sarcoma – A review. Oncol Res Treat 2018;41:687-92.  Back to cited text no. 10
Nusrath S, Bafna S, Rajagopalan R, Thammineedi SR, Raju KV, Patnaik SC, et al. Uterine sarcomas: Experience from a tertiary cancer care center from India. Indian J Surg Oncol 2019;10:342-9.  Back to cited text no. 11
Sivakumari S, Rajaraman R, Subbiah S. Uterine sarcoma: The Indian scenario. Indian J Surg Oncol 2015;6:232-6.  Back to cited text no. 12


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