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"Journal of Clinical and Diagnostic Research is at present a well-known Indian originated scientific journal which started with a humble beginning. I have been associated with this journal since many years. I appreciate the Editor, Dr. Hemant Jain, for his constant effort in bringing up this journal to the present status right from the scratch. The journal is multidisciplinary. It encourages in publishing the scientific articles from postgraduates and also the beginners who start their career. At the same time the journal also caters for the high quality articles from specialty and super-specialty researchers. Hence it provides a platform for the scientist and researchers to publish. The other aspect of it is, the readers get the information regarding the most recent developments in science which can be used for teaching, research, treating patients and to some extent take preventive measures against certain diseases. The journal is contributing immensely to the society at national and international level."



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MBBS, MD (Pathology),
Sanjay Gandhi institute of trauma and orthopedics,
Bengaluru.
On Aug 2018




Dr. Mamta Gupta,
"It gives me great pleasure to be associated with JCDR, since last 2-3 years. Since then I have authored, co-authored and reviewed about 25 articles in JCDR. I thank JCDR for giving me an opportunity to improve my own skills as an author and a reviewer.
It 's a multispecialty journal, publishing high quality articles. It gives a platform to the authors to publish their research work which can be available for everyone across the globe to read. The best thing about JCDR is that the full articles of all medical specialties are available as pdf/html for reading free of cost or without institutional subscription, which is not there for other journals. For those who have problem in writing manuscript or do statistical work, JCDR comes for their rescue.
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Reviewing articles is no less a pain staking process and requires in depth perception, knowledge about the topic for review. It requires time and concentration, yet I enjoy doing it. The JCDR website especially for the reviewers is quite user friendly. My suggestions for improving the journal is, more strict review process, so that only high quality articles are published. I find a a good number of articles in Obst. Gynae, hence, a new journal for this specialty titled JCDR-OG can be started. May be a bimonthly or quarterly publication to begin with. Only selected articles should find a place in it.
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Dr. Mamta Gupta
Consultant
(Ex HOD Obs &Gynae, Hindu Rao Hospital and associated NDMC Medical College, Delhi)
Aug 2018




Dr. Rajendra Kumar Ghritlaharey

"I wish to thank Dr. Hemant Jain, Editor-in-Chief Journal of Clinical and Diagnostic Research (JCDR), for asking me to write up few words.
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Every one of us: authors, reviewers, editors, and publisher are responsible for enhancing the stature of the journal. I wish for a great success for JCDR."



Thanking you
With sincere regards
Dr. Rajendra Kumar Ghritlaharey, M.S., M. Ch., FAIS
Associate Professor,
Department of Paediatric Surgery, Gandhi Medical College & Associated
Kamla Nehru & Hamidia Hospitals Bhopal, Madhya Pradesh 462 001 (India)
E-mail: drrajendrak1@rediffmail.com
On May 11,2011




Dr. Shankar P.R.

"On looking back through my Gmail archives after being requested by the journal to write a short editorial about my experiences of publishing with the Journal of Clinical and Diagnostic Research (JCDR), I came across an e-mail from Dr. Hemant Jain, Editor, in March 2007, which introduced the new electronic journal. The main features of the journal which were outlined in the e-mail were extensive author support, cash rewards, the peer review process, and other salient features of the journal.
Over a span of over four years, we (I and my colleagues) have published around 25 articles in the journal. In this editorial, I plan to briefly discuss my experiences of publishing with JCDR and the strengths of the journal and to finally address the areas for improvement.
My experiences of publishing with JCDR: Overall, my experiences of publishing withJCDR have been positive. The best point about the journal is that it responds to queries from the author. This may seem to be simple and not too much to ask for, but unfortunately, many journals in the subcontinent and from many developing countries do not respond or they respond with a long delay to the queries from the authors 1. The reasons could be many, including lack of optimal secretarial and other support. Another problem with many journals is the slowness of the review process. Editorial processing and peer review can take anywhere between a year to two years with some journals. Also, some journals do not keep the contributors informed about the progress of the review process. Due to the long review process, the articles can lose their relevance and topicality. A major benefit with JCDR is the timeliness and promptness of its response. In Dr Jain's e-mail which was sent to me in 2007, before the introduction of the Pre-publishing system, he had stated that he had received my submission and that he would get back to me within seven days and he did!
Most of the manuscripts are published within 3 to 4 months of their submission if they are found to be suitable after the review process. JCDR is published bimonthly and the accepted articles were usually published in the next issue. Recently, due to the increased volume of the submissions, the review process has become slower and it ?? Section can take from 4 to 6 months for the articles to be reviewed. The journal has an extensive author support system and it has recently introduced a paid expedited review process. The journal also mentions the average time for processing the manuscript under different submission systems - regular submission and expedited review.
Strengths of the journal: The journal has an online first facility in which the accepted manuscripts may be published on the website before being included in a regular issue of the journal. This cuts down the time between their acceptance and the publication. The journal is indexed in many databases, though not in PubMed. The editorial board should now take steps to index the journal in PubMed. The journal has a system of notifying readers through e-mail when a new issue is released. Also, the articles are available in both the HTML and the PDF formats. I especially like the new and colorful page format of the journal. Also, the access statistics of the articles are available. The prepublication and the manuscript tracking system are also helpful for the authors.
Areas for improvement: In certain cases, I felt that the peer review process of the manuscripts was not up to international standards and that it should be strengthened. Also, the number of manuscripts in an issue is high and it may be difficult for readers to go through all of them. The journal can consider tightening of the peer review process and increasing the quality standards for the acceptance of the manuscripts. I faced occasional problems with the online manuscript submission (Pre-publishing) system, which have to be addressed.
Overall, the publishing process with JCDR has been smooth, quick and relatively hassle free and I can recommend other authors to consider the journal as an outlet for their work."



Dr. P. Ravi Shankar
KIST Medical College, P.O. Box 14142, Kathmandu, Nepal.
E-mail: ravi.dr.shankar@gmail.com
On April 2011
Anuradha

Dear team JCDR, I would like to thank you for the very professional and polite service provided by everyone at JCDR. While i have been in the field of writing and editing for sometime, this has been my first attempt in publishing a scientific paper.Thank you for hand-holding me through the process.


Dr. Anuradha
E-mail: anuradha2nittur@gmail.com
On Jan 2020

Important Notice

Case report
Year : 2023 | Month : September | Volume : 17 | Issue : 9 | Page : QD01 - QD04 Full Version

Endometrial Stromal Sarcoma in a Young, Nulliparous Woman: A Case Report


Published: September 1, 2023 | DOI: https://doi.org/10.7860/JCDR/2023/63730.18388
S Subhasri, K Nivedita, N Noorul Nasheeha, K Rajkumar, V Sriram

1. Junior Resident, Department of Obstetrics and Gynaecology, Sri Manakular Vinayagar Medical College and Hospital, Puducherry, India. 2. Professor, Department of Obstetrics and Gynaecology, Sri Manakular Vinayagar Medical College and Hospital, Puducherry, India. 3. Assistant Professor, Department of Obstetrics and Gynaecology, Sri Manakular Vinayagar Medical College and Hospital, Puducherry, India. 4. Assistant Professor, Department of Oncosurgery, Sri Manakular Vinayagar Medical College and Hospital, Puducherry, India. 5. Associate Professor, Department of Pathology, Sri Manakular Vinayagar Medical College and Hospital, Puducherry, India.

Correspondence Address :
S Subhasri,
Junior Resident, Department of Obstetrics and Gynaecology, Sri Manakular Vinayagar Medical College and Hospital, Puducherry, India.
E-mail: Subhasri1410@gmail.com

Abstract

Endometrial stromal tumours are tumours of endometrial stromal origin and are classified into Endometrial Stromal Nodule (ESN), Low-Grade Endometrial Stromal Sarcoma (LG-ESS), and High-Grade Endometrial Stromal Sarcoma (HG-ESS). LG-ESS and HG-ESS are rare tumours, accounting for 1% of uterine malignancies and 10% of uterine sarcomas. These tumours commonly occur in perimenopausal women between the ages of 45 and 50 years. Their incidence is rare in younger women. Endometrial stromal tumours are usually confused with leiomyoma, uterine Leiomyosarcoma (LMS), or other sarcomas. The authors here present a case report of a 28-year-old nulligravid patient who presented with a history of heavy menstrual bleeding and dysmenorrhea for a duration of six months. Ultrasonography of the abdomen and pelvis suggested fibroid with degenerative changes, and Magnetic Resonance Imaging (MRI) indicated leiomyoma variants such as: i) Stromal Tumours of Uncertain Malignant Potential (STUMP)/atypical/cellular leiomyoma; ii) myxoid degeneration of leiomyoma. To arrive at a definitive diagnosis, myomectomy was performed considering the woman’s young age and nulliparity. Histopathology allowed for a differential diagnosis of LG-ESS, LMS, and cellular leiomyoma. Consequently, the patient underwent total abdominal hysterectomy with left salphingo opherectomy, right salpingectomy, and preservation of the right ovary. The definitive diagnosis is made by histopathological examination coupled with immunohistochemistry of the hysterectomy specimen. Hysterectomy is the definitive treatment of LG-ESS considering their ability to infiltrate and become malignant.

Keywords

Leiomyosarcoma, Low grade, Uterine malignancy

Case Report

A 28-year-old nulligravida presented with a history of heavy menstrual bleeding and dysmenorrhea for a duration of six months. She attained menarche at the age of 14, with regular and normal menstrual cycles. She has no history of contraceptive use or any significant medical or family history. Upon presentation at the hospital, she did not appear pale, and no palpable abdominal mass was found. As she was not sexually active, a bimanual examination was deferred.

Ultrasonography of the abdomen and pelvis showed a heterogeneous mixed solid to cystic lesion in the uterus, suggestive of fibroid with degenerative changes. Magnetic Resonance Imaging (MRI) of the abdomen and pelvis revealed a bulky uterus measuring 10.4×8.7×9.9 cm, with a well-encapsulated heterogeneous lesion predominantly hyperintense on both T1 and T2, with a peripheral T2 hypointense rim arising from the posterior and left lateral wall of the uterus. There was no suppression on fat saturation images, no blooming on gradient images, no pelvic/para-aortic lymphadenopathy, and no ascites. The differential diagnosis included: i) leiomyoma variants like STUMP/atypical/cellular leiomyoma; ii) myxoid degeneration of leiomyoma (Table/Fig 1),(Table/Fig 2),(Table/Fig 3). Given these findings, she underwent examination under anaesthesia and hysteroscopic-guided endometrial biopsy.

During bimanual examination, a uterus of 14 weeks in size and a posterior wall fibroid measuring 5×5 cm felt through the pouch of Douglas were noted. Hysteroscopic-guided endometrial biopsy revealed a thickened polypoidal endometrium, and the histopathological report indicated endometrial hyperplasia without atypia. Considering the woman’s young age and nulliparity, myomectomy was performed to arrive at a definitive diagnosis. Intraoperative findings revealed a posterior uterine wall tumour measuring 7×6 cm with variable consistency, which was enucleated in toto (Table/Fig 4). Grossly, the specimen had a yellowish tan color (Table/Fig 5). Microscopic examination revealed necrotic tissue with tumour cells arranged as nodules with interlacing fascicles of smooth muscle cells. The individual tumour cells were round to spindle-shaped with bland nuclear features and occasional mild atypia. One focus showed vascular and muscular invasion of tumour cells. The histopathological diagnosis included a differential diagnosis of LG-ESS, LMS, and cellular leiomyoma, and the patient was posted for definitive surgery.

Total abdominal hysterectomy with left salphingo opherectomy, right salpingectomy, and right ovarian preservation were performed. Microscopy showed tumour cells arranged in nodules and sheets infiltrating the myometrial wall for more than 50% of the myometrium. The tumour cells had round to oval hyperchromatic nuclei, with 8 to 12 mitoses per 10 high-power fields (Table/Fig 6),(Table/Fig 7). The tumour was involved in the blood vessels (Table/Fig 8). Additional immunohistochemistry studies revealed positive staining for CD10 (Table/Fig 9). HPE and immunohistochemistry with CD10 confirmed the diagnosis of LG-ESS. According to International Federation of Gynecology and Obstetrics (FIGO) staging, it was classified as Stage-Ib disease. The postoperative clinical course was uneventful. At the three-month follow-up visit, pelvic ultrasound, MRI pelvis, and chest X-ray were performed, and all were normal. The plan is to continue follow-up with clinical examination and the same investigations after six months, followed by annual check-ups.

Discussion

Endometrial stromal sarcoma is a rare malignant tumour. It was first reported by Norris HJ and Taylor HB in 1966, who classified ESS into low-grade and high-grade types based on the mitotic index (1). According to the 2014 WHO classification, ESS is divided into four categories: ESN, LG-ESS, HG-ESS, and Undifferentiated Uterine Sarcoma (UUS) (2). LG-ESS and HG-ESS are rare malignancies, accounting for 1% of uterine malignancies and 10% of uterine sarcomas (3).

ESS most often affects perimenopausal women around 45 to 55 years. The presenting complaints are similar to leiomyoma of the uterus: abnormal uterine bleeding, abdominal pain, or pelvic mass. An enlarging pelvic mass can cause pressure, and some patients may be asymptomatic (4). Martinez M and Jacinto E reported a different presentation in the form of a polypoidal fleshy mass projecting through the cervix (5). This tumour has a high tendency for local recurrence and metastasis; and hence, an early diagnosis is important as patient survival is determined by the stage of the tumour (6).

The pathogenesis of ESS remains largely unknown, although specific somatic mutations have been discovered through cytogenetic, Fluorescence In Situ Hybridisation (FISH), and Polymerase Chain Reaction (PCR) analysis (7). Most ESS cases are characterised by an overexpression of estrogen and progesterone receptors (6). Diagnosing ESS is usually difficult preoperatively as it may be mistaken for leiomyoma, uterine LMS, or other sarcomas. The definitive diagnosis is made through histopathological examination coupled with immunohistochemistry of the hysterectomy specimen (8).

Ultrasonographic findings may be confused with uterine leiomyoma and adenomyosis. However, MRI can be used for a preoperative diagnosis with some accuracy. The lesion of endometrial stromal sarcoma is isointense relative to the myometrium on T1-weighted MRI and hyperintense on T2-weighted MRI. It also shows heterogeneous but prominent enhancement in contrast-enhanced images due to its rich vascularity (9). The characteristic findings of ESS on MRI are worm-like permeation of cancerous cells in the myometrium (9). The main tumour mass is almost always intramyometrial, but most ESS cases involve the endometrium. Uterine curettage may be helpful in preoperative diagnosis, but when the lesion is within the myometrium, the scrapings may not be useful (10).

The immunohistochemical markers, such as CD10, H-caldesmon, and hormone receptors, are helpful in the diagnosis. CD10 staining is positive in LG-ESS but not in HG-ESS and leiomyoma (11). These tumours are estrogen and progesterone receptor positive. CD10 has a sensitivity of 100% and specificity of 90%, while ER/PR shows a sensitivity and specificity of 80% and 100%, respectively, in diagnosing LG-ESS (12). In our patient, there was cytoplasmic positivity of CD10 staining, indicating a provisional diagnosis of LG-ESS.

The International Federation of Gynecology and Obstetrics staging of uterine sarcoma is used to determine the stage of ESS (13). Stage I is the most significant prognostic factor, and the 5-year Overall Survival (OS) rate for Stage I LG-ESS patients is more than 90%, but it decreases to 50% for Stage III and IV (14).

The treatment for LG-ESS involves total abdominal hysterectomy; however, ovary removal and pelvic and para-aortic lymphadenectomy remain debatable. Bilateral salphingo opherectomy has been recommended, even in premenopausal women with Stage I ESS disease, as it is a hormone-sensitive tumour, and the recurrence rate is high in women in whom ovaries were retained (15).

Recent reports suggest that preserving the ovaries may be possible in premenopausal women with Stage I ESS if the tumour is completely removed (16). Fertility-sparing treatment, along with adjuvant treatment, has been reported in cases of LG-ESS using local excision, hormone therapy, and photodynamic therapy (17). Studies have indicated that lymphadenectomy does not have a significant role in recurrence-free survival or OS in LG-ESS (16). Postoperative hormone therapy is an effective adjuvant treatment providing high local control and preventing recurrences. Hormone therapy with medroxyprogesterone, tamoxifen, Gonadotropin Releasing Hormone (GnRH) analogues, and aromatase inhibitors is suggested for LG-ESS stage 3-4 and recurrent disease (18).

The mechanism of action of progestins is to bind progesterone receptors and cause downregulation of gene transcription, leading to decreased endometrial gland and stromal proliferation (19). In our patient, the decision for hysterectomy was based on the currently available diagnostic tools and the lack of knowledge regarding the long-term consequences of conservative treatment.

Conclusion

Endometrial stromal sarcoma is a rarely encountered malignancy, especially in young individuals. Despite its rarity, ESS holds significant clinical importance due to the diagnostic and therapeutic challenges it presents. The authors reported this case to emphasise the need for a high level of suspicion and thorough evaluation of fibroid masses causing prolonged bleeding in young women, in order to promptly rule out malignancy at an early stage. The timely diagnosis and intervention are crucial for improving patient survival.

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DOI and Others

DOI: 10.7860/JCDR/2023/63730.18388

Date of Submission: Feb 27, 2023
Date of Peer Review: Apr 27, 2023
Date of Acceptance: Jun 23, 2023
Date of Publishing: Sep 01, 2023

AUTHOR DECLARATION:
• Financial or Other Competing Interests: None
• Was informed consent obtained from the subjects involved in the study? Yes
• For any images presented appropriate consent has been obtained from the subjects. Yes

PLAGIARISM CHECKING METHODS:
• Plagiarism X-checker: Mar 07, 2023
• Manual Googling: May 13, 2023
• iThenticate Software: Jun 16, 2023 (10%)

ETYMOLOGY: Author Origin

EMENDATIONS: 6

JCDR is now Monthly and more widely Indexed .
  • Emerging Sources Citation Index (Web of Science, thomsonreuters)
  • Index Copernicus ICV 2017: 134.54
  • Academic Search Complete Database
  • Directory of Open Access Journals (DOAJ)
  • Embase
  • EBSCOhost
  • Google Scholar
  • HINARI Access to Research in Health Programme
  • Indian Science Abstracts (ISA)
  • Journal seek Database
  • Google
  • Popline (reproductive health literature)
  • www.omnimedicalsearch.com