Journal of Clinical and Diagnostic Research, ISSN - 0973 - 709X

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On Sep 2018




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Prof. Somashekhar Nimbalkar
Head, Department of Pediatrics, Pramukhswami Medical College, Karamsad
Chairman, Research Group, Charutar Arogya Mandal, Karamsad
National Joint Coordinator - Advanced IAP NNF NRP Program
Ex-Member, Governing Body, National Neonatology Forum, New Delhi
Ex-President - National Neonatology Forum Gujarat State Chapter
Department of Pediatrics, Pramukhswami Medical College, Karamsad, Anand, Gujarat.
On Sep 2018




Dr. Kalyani R

"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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Sri Devaraj Urs Medical College
Sri Devaraj Urs Academy of Higher Education and Research , Kolar, Karnataka
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Dr. Saumya Navit

"As a peer-reviewed journal, the Journal of Clinical and Diagnostic Research provides an opportunity to researchers, scientists and budding professionals to explore the developments in the field of medicine and dentistry and their varied specialities, thus extending our view on biological diversities of living species in relation to medicine.
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Professor and Head
Department of Pediatric Dentistry
Saraswati Dental College
Lucknow
On Sep 2018




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Dr. Arunava Biswas
MD, DM (Clinical Pharmacology)
Assistant Professor
Department of Pharmacology
Calcutta National Medical College & Hospital , Kolkata




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Best regards,
C.S. Ramesh Babu,
Associate Professor of Anatomy,
Muzaffarnagar Medical College,
Muzaffarnagar.
On Aug 2018




Dr. Arundhathi. S
"Journal of Clinical and Diagnostic Research (JCDR) is a reputed peer reviewed journal and is constantly involved in publishing high quality research articles related to medicine. Its been a great pleasure to be associated with this esteemed journal as a reviewer and as an author for a couple of years. The editorial board consists of many dedicated and reputed experts as its members and they are doing an appreciable work in guiding budding researchers. JCDR is doing a commendable job in scientific research by promoting excellent quality research & review articles and case reports & series. The reviewers provide appropriate suggestions that improve the quality of articles. I strongly recommend my fraternity to encourage JCDR by contributing their valuable research work in this widely accepted, user friendly journal. I hope my collaboration with JCDR will continue for a long time".



Dr. Arundhathi. S
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.
The journal has a monthly publication and the articles are published quite fast. In time compared to other journals. The on-line first publication is also a great advantage and facility to review one's own articles before going to print. The response to any query and permission if required, is quite fast; this is quite commendable. I have a very good experience about seeking quick permission for quoting a photograph (Fig.) from a JCDR article for my chapter authored in an E book. I never thought it would be so easy. No hassles.
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.
An yearly reward for the best article authored can also incentivize the authors. Though the process of finding the best article will be not be very easy. I do not know how reviewing process can be improved. If an article is being reviewed by two reviewers, then opinion of one can be communicated to the other or the final opinion of the editor can be communicated to the reviewer if requested for. This will help one’s reviewing skills.
My best wishes to Dr. Hemant Jain and all the editorial staff of JCDR for their untiring efforts to bring out this journal. I strongly recommend medical fraternity to publish their valuable research work in this esteemed journal, JCDR".



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.
Writing is the representation of language in a textual medium i e; into the words and sentences on paper. Quality medical manuscript writing in particular, demands not only a high-quality research, but also requires accurate and concise communication of findings and conclusions, with adherence to particular journal guidelines. In medical field whether working in teaching, private, or in corporate institution, everyone wants to excel in his / her own field and get recognised by making manuscripts publication.


Authors are the souls of any journal, and deserve much respect. To publish a journal manuscripts are needed from authors. Authors have a great responsibility for producing facts of their work in terms of number and results truthfully and an individual honesty is expected from authors in this regards. Both ways its true "No authors-No manuscripts-No journals" and "No journals–No manuscripts–No authors". Reviewing a manuscript is also a very responsible and important task of any peer-reviewed journal and to be taken seriously. It needs knowledge on the subject, sincerity, honesty and determination. Although the process of reviewing a manuscript is a time consuming task butit is expected to give one's best remarks within the time frame of the journal.
Salient features of the JCDR: It is a biomedical, multidisciplinary (including all medical and dental specialities), e-journal, with wide scope and extensive author support. At the same time, a free text of manuscript is available in HTML and PDF format. There is fast growing authorship and readership with JCDR as this can be judged by the number of articles published in it i e; in Feb 2007 of its first issue, it contained 5 articles only, and now in its recent volume published in April 2011, it contained 67 manuscripts. This e-journal is fulfilling the commitments and objectives sincerely, (as stated by Editor-in-chief in his preface to first edition) i e; to encourage physicians through the internet, especially from the developing countries who witness a spectrum of disease and acquire a wealth of knowledge to publish their experiences to benefit the medical community in patients care. I also feel that many of us have work of substance, newer ideas, adequate clinical materials but poor in medical writing and hesitation to submit the work and need help. JCDR provides authors help in this regards.
Timely publication of journal: Publication of manuscripts and bringing out the issue in time is one of the positive aspects of JCDR and is possible with strong support team in terms of peer reviewers, proof reading, language check, computer operators, etc. This is one of the great reasons for authors to submit their work with JCDR. Another best part of JCDR is "Online first Publications" facilities available for the authors. This facility not only provides the prompt publications of the manuscripts but at the same time also early availability of the manuscripts for the readers.
Indexation and online availability: Indexation transforms the journal in some sense from its local ownership to the worldwide professional community and to the public.JCDR is indexed with Embase & EMbiology, Google Scholar, Index Copernicus, Chemical Abstracts Service, Journal seek Database, Indian Science Abstracts, to name few of them. Manuscriptspublished in JCDR are available on major search engines ie; google, yahoo, msn.
In the era of fast growing newer technologies, and in computer and internet friendly environment the manuscripts preparation, submission, review, revision, etc and all can be done and checked with a click from all corer of the world, at any time. Of course there is always a scope for improvement in every field and none is perfect. To progress, one needs to identify the areas of one's weakness and to strengthen them.
It is well said that "happy beginning is half done" and it fits perfectly with JCDR. It has grown considerably and I feel it has already grown up from its infancy to adolescence, achieving the status of standard online e-journal form Indian continent since its inception in Feb 2007. This had been made possible due to the efforts and the hard work put in it. The way the JCDR is improving with every new volume, with good quality original manuscripts, makes it a quality journal for readers. I must thank and congratulate Dr Hemant Jain, Editor-in-Chief JCDR and his team for their sincere efforts, dedication, and determination for making JCDR a fast growing journal.
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 Series
Year : 2023 | Month : August | Volume : 17 | Issue : 8 | Page : ER15 - ER19 Full Version

Endometrial Stromal Tumour: Clinicopathological Series of Seven Cases


Published: August 1, 2023 | DOI: https://doi.org/10.7860/JCDR/2023/64407.18372
Srimoyee Sen, Manisha Sarkar, Moumita Sengupta, Uttara Chatterjee

1. Junior Resident, Department of Pathology, IPGME&R, Kolkata, West Bengal, India. 2. Associate Professor, Department of Pathology, Dr. B C Roy PGIPS, Kolkata, West Bengal, India. 3. Associate Professor, Department of Pathology, IPGME&R, Kolkata, West Bengal, India. 4. Professor, Department of Pathology, IPGME&R, Kolkata, West Bengal, India.

Correspondence Address :
Dr. Manisha Sarkar,
Flat-2C, Anushka Tower, 10/2A, Umakanta Sen Lane, Kolkata-700030, West Bengal, India.
E-mail: manishasarkar1979@gmail.com

Abstract

Endometrial Stromal Tumour (EST) mimics other neoplasms and is difficult to diagnose due to its wide range of morphologies. This is a clinicopathological study of seven cases of EST, which includes Endometrial Stromal Nodule (ESN), Low-grade Endometrial Stromal Sarcoma (LGESS), and High-grade Endometrial Stromal Sarcoma (HGESS). The age ranges from 34 to 75 years. Five out of seven cases presented with abnormal uterine bleeding, abdominal pain, and were radiologically suspected to be leiomyomas. After histopathological and Immunohistochemical (IHC) examination, one was diagnosed as ESN, three as LGESS, and the remaining three cases as HGESS. One case was initially diagnosed as a cellular leiomyoma and experienced multiple recurrences, eventually being diagnosed as HGESS with a fatal outcome within 36 months of the onset of the first symptoms. ESN and LGESS should be differentiated from leiomyoma and leiomyosarcoma. IHC plays an important role in distinguishing these tumours from the more common smooth muscle counterparts.

Keywords

Cluster of differentiation 10, Mimics, Wilms tumour gene 1

The EST are a rare group of tumours, accounting for <1% of all uterine tumours (1). However, accurate diagnosis is important as most of them fall under the malignant category unlike their smooth muscle counterparts. There are four main categories, include ESN, LGESS, HGESS and Undifferentiated Stromal Sarcoma (USS). LGESS is the most common type (2). While a few case reports are available [2-6], there is a lack of large case series with follow-up data. The present study describes a series of seven EST cases, detailing gross findings, morphology, IHC findings, and follow-up data.

Case Report

The present case series describes a study of seven ESTs collected over a period of four years (from 2018 to 2022), with ages ranging from 34 to 75 years. Detailed clinical, radiological, and treatment histories of each case were recorded before histopathological examination. IHC staining was done with formalin-fixed paraffin-embedded sections using a detection system based on the peroxidase-antiperoxidase method. Heat-induced epitope retrieval and enzymatic activation of chromogen were conducted for visualising the antigen-antibody reaction product. A positive tissue control and the same tissue for internal negative control were used for each case. The clinicopathological profiles of the cases are summarised in (Table/Fig 1).

Case 1

The first case involved a 35-year-old parous woman who presented with abnormal uterine bleeding for two years, with no significant past medical history. Ultrasonography (USG) revealed an intramural mass suggestive of a leiomyoma. On gross examination, a well-circumscribed yellowish nodule measuring 4×4 cm was observed (Table/Fig 2)a. Microscopically, densely packed round to oval cells resembling the proliferative phase of the endometrium were seen, with low mitotic activity (<3/10HPF) (Table/Fig 2)b,c. Periarteriolar whorling arrangement and expansile non infiltrative margins were noted. The neoplastic cells were positive for CD10, leading to a diagnosis of ESN (Table/Fig 2)d. The patient had an uneventful long-term postoperative follow-up period.

Case 2

A 40-year-old woman who presented with menorrhagia, pelvic pain, and an abdominal mass lasting for one year. The sonographic diagnosis was a fibroid uterus, and she underwent myomectomy (Table/Fig 3)a. However, she returned with menorrhagia after three months. Subsequently, a total abdominal hysterectomy and omental sampling were performed. The provisional clinical diagnosis was leiomyosarcoma. On gross examination, multiple grayish-white intrauterine masses were observed, with the largest measuring 7×4×3 cm. Microscopically, the tumour masses consisted of proliferating round to oval stromal cells arranged in sheets with prominent vascularity and the presence of spiral vessels. Hyalinised stroma with myxoid change, as well as lymphatic invasion and myometrial invasions, were noted (Table/Fig 3)b. The neoplastic cells exhibited mild to moderate pleomorphism, moderate nuclear atypia, and increased mitotic activity (>4/10 HPF). The presence of focal atypical spindle cells and collagen deposition (Table/Fig 3)c made it challenging to differentiate between LGESS, leiomyosarcoma, and sex cord stromal tumour. Omental tissue sampling revealed the presence of sarcomatous deposits. IHC showed diffuse positive staining for CD10, WT1 (Table/Fig 3)d, Estrogen Receptor (ER), Progesterone Receptor (PR), and negative staining for desmin, inhibin, and CD56. The final diagnosis was LGESS.

Case 3

A 75-year-old postmenopausal woman presented with pelvic pain and a mass that had been present for two and a half years. She had a history of myomectomy 15 years ago. The clinical and radiological diagnosis was a fibroid uterus, and the patient underwent abdominal hysterectomy and Bilateral Salpingo-Oophorectomy (BSO). On gross examination, multiple intramural fleshy white masses were identified, with the largest measuring 8×6 cm (Table/Fig 4)a. Morphologically, it shared similar features with the second case. It was a cellular round cell tumour that exhibited tongue-like myometrial invasion, moderate nuclear atypia, brisk mitoses, and areas of vascular prominence (Table/Fig 4)b,c. The differential diagnosis was LGESS versus leiomyosarcoma. Immunohistochemically, the tumour cells were positive for CD10, WT1, ER (Table/Fig 4)d, PR, and negative for desmin. Therefore, the final diagnosis was LGESS.

Case 4

A 54-year-old woman presented with complaints of postmenopausal bleeding lasting for eight months. She underwent hysterectomy following a radiological diagnosis of a fibroid uterus. On gross examination, intramural fleshy masses were noted, with the largest measuring 8×4 and 4×3 cm (Table/Fig 5)a. Microscopically, there was proliferation of round to oval stromal cells arranged in sheets, with lymphovascular and myometrial invasion (Table/Fig 5)b. The neoplastic cells exhibited nuclear atypia and increased mitotic activity (7/10 HPF) (Table/Fig 5)c. The diagnosis of LGESS was made after conducting an IHC study using a similar antibody panel as in previous two cases. The tumour cells were positive for CD10, WT1 [Table/Fig-5d], ER, PR, and negative for desmin. In all three cases of LGESS, the initial differential diagnosis was leiomyosarcoma. The postoperative follow-up period was unremarkable in these cases.

Case 5

A 60-year-old woman presented with postmenopausal bleeding and a pelvic mass lasting for two months, without any significant past medical history. Her serum CA-125 level was mildly elevated (45 U/mL), and the radiological diagnosis was a possibly malignant endometrial polyp.

Following hysterectomy and BSO an irregular ulcerated polypoid lesion measuring 6×5 cm was observed, filling up the uterine cavity (Table/Fig 6)a. Microscopically, there were sheets of neoplastic round to oval cell proliferation with areas of necrosis, haemorrhage, arborising vasculature, and a high mitotic count (15/10 HPF) (Table/Fig 6)b,c. The tumour had invaded into the myometrium. The differential diagnoses were HGESS and poorly differentiated carcinoma. IHC study showed focal CD10 positivity (Table/Fig 6)d, positive staining for Cyclin D1, and negative staining for WT1, ER, PR, EMA, and desmin. Therefore, the final diagnosis of HGESS was made. Unfortunately, the patient was lost within one month of the operation despite her receiving initial chemotherapy.

Case 6

A 60-year-old woman with a clinical suspicion of intraabdominal malignancy. The CT abdomen revealed a tumour arising from the 17fundus of the uterus and adhering to a segment of the small bowel. Palliative surgery was performed, which involved resection of the mass along with a portion of the attached small bowel. On gross examination, a fragile mass measuring 6x5x4 cm with necrotic areas was observed (Table/Fig 7)a. Microscopically, it demonstrated an atypical proliferation of round to spindle cells, tumour giant cells, hyalinised vessels, lymphovascular invasion, myxoid areas, necrosis, haemorrhage, and a high mitotic activity (15/10HPF) (Table/Fig 7)c. The tumour invaded the small bowel wall through the muscularis propria (Table/Fig 7)b. The closest differential diagnoses included Gastrointestinal Stromal Tumour (GIST), spindle cell sarcoma, HGESS and desmoplastic small round cell tumour. The tumour showed focal positivity for CD10, Cyclin D1 (Table/Fig 7)d, and negativity for desmin, myogenin, WT1, S-100, ER, PR, and EMA. The final diagnosis was HGESS; however, the patient did not return for follow-up.

Case 7

A 34-year-old female presented in 2019 with abdominal swelling and pain lasting for eight months. She had a history of cholecystectomy with myomectomy in 2018, with a histopathological report of chronic cholecystitis and leiomyoma. Following abdominal hysterectomy in June 2019, a histopathological diagnosis of cellular leiomyoma was given. She returned back in February 2020 with a large recurrent mass and an Magnetic Resonance Imaging (MRI) showed a pelvic mass with mixed intensity and solid cystic areas, herniating into subcutaneous tissue. The mass was separated from the liver, pancreas, kidney, and spleen, and it displaced the stomach and bowel loops. The patient underwent debulking surgery, and a 5 kg tumour mass was removed (Table/Fig 8)a. A provisional histopathological diagnosis of desmoplastic small round cell tumour was made. Microscopically, it was a hypercellular oval to spindle cell tumour with areas of necrosis, lymphovascular invasion, high mitotic count, and focal fibro-myxoid appearance (Table/Fig 8)b-d. She underwent chemo-radiation but eventually presented with abdominal and lung metastasis in November 2020. IHC study was conducted, which showed negative staining for Cytokeratin, EMA, desmin, and WT1. Vimentin and cyclin D1 were positive, and CD10 was focally positive. Finally, the diagnosis of HGESS was made, but unfortunately, the patient had already succumbed to death within a month of the last surgery.

Discussion

Endometrial Stromal Tumours (ESTs) are a rare group of tumours that typically occur in perimenopausal women. Due to their rarity, diagnosing ESTs can be challenging. LGESS tends to occur at a younger age than HGESS and USS with a median age ranging from 45 to 55 years (3). The most common clinical presentation of EST is abnormal uterine bleeding, although patients may also experience pelvic pain and dysmenorrhoea (7). ESNs are clinically considered benign, while LGESSs are tumours of low malignant potential, often exhibiting indolent clinical behaviour. Some cases may experience late recurrence after hysterectomy, as seen in two cases. HGESS, on the other hand, is a tumour of high malignant potential with a more aggressive clinical outcome. UUS displays high-grade morphological features and exhibits very aggressive clinical behaviour (8). The differential diagnosis for ESN includes cellular leiomyoma and LGESS. ESNs typically exhibit uniform bland ovoid cells resembling proliferative phase endometrial stroma. The vascular pattern, composed of typical arterioles, is not a prominent feature of cellular leiomyoma. While ESNs may contain large blood vessels, a characteristic of cellular leiomyoma, they are not as conspicuous as in cellular leiomyoma (9). Additionally, CD10 can be useful in differentiating ESN from cellular leiomyoma. The presence of a pushing margin and the absence of lymphovascular invasion help differentiate ESN from LGESS. Therefore, a definitive diagnosis of ESN can be rendered on resected specimens, allowing for extensive sampling, and cannot be confidently established on small biopsies (8).

The differential diagnosis for LGESS includes HGESS, cellular leiomyoma, leiomyosarcoma with an extensive intravascular component, Uterine Tumours Resembling Ovarian Sex-Cord Tumours (UTROSCT), and adenosarcoma (10). LGESS is morphologically characterised by uniform ovoid cells with mild to moderate atypia, tongue-like invasion into the myometrium, lymphovascular permeation, and a mitotic activity ranging from 1-13/10HPF (11). IHC panel including CD10, inhibin, calretinin, and CD56 can be useful in distinguishing ESS from sex cord-stromal tumours, although it is important to note that markers of sex cord-like differentiation may be strongly positive in sex cord-like areas of ESS. CD56 is typically positive in sex cord-stromal tumours but negative in ESS. In the abdominal cavity, GIST is an important differential consideration (12). Morphologically, both tumours are usually distinct. GIST demonstrates cellular, plump, and uniformly spindle cell proliferation with nuclear palisading and characteristic perinuclear vacuolisation, in contrast to the plump, round to oval cell proliferation of EST. GISTs typically show limited atypia, with mitotic activity rarely exceeding 10/50 HPF (13). GISTs are positive for c-Kit, CD34, and DOG1, while LGESS shows diffuse positivity for ER, PR, and CD10.

There can be significant overlap in staining for CD10 and desmin between ESS and smooth muscle tumours, making it helpful to include additional smooth muscle markers, such as h-caldesmon or calponin, which are usually negative in ESS. In cases presenting with urinary symptoms and a bladder mass, the differential diagnosis includes Solitary Fibrous Tumour (SFT), synovial sarcoma, carcinoid, Primitive Neuroectodermal Tumours (PNET) and large nested variants of urothelial carcinoma. The focal presence of spindle cell morphology, bland cytology, and abundant collagen in LGESS may lead to a differential diagnosis of SFT. Additionally, endometrial stromal sarcoma can occasionally show stag horn-like vessels similar to those seen in SFT. However, these findings are seen only in focal areas of LGESS, and the presence of typical small round arterioles helps establish the diagnosis. CD34 and STAT6 are positive in SFT and negative in EST.

Carcinoid tumours and ESS can exhibit overlapping IHC staining. ESS is focally positive for CD56 and synaptophysin, while carcinoid tumours variably stain positive for ER, PR, or CD10. However, the characteristic organoid pattern and salt and pepper nuclear chromatin in carcinoid tumours, along with diffuse ER, PR, and CD10 positivity in LGESS, aid in distinguishing these two entities. LGESS typically shows negative CD99 staining, which is seen in PNET. The wormlike pattern of invasion observed in LGESS is similar to the infiltration seen in the large, nested variant of urothelial carcinoma. GATA3 and thrombomodulin are positive in large, nested variants of urothelial carcinoma and negative in ESS (12). WT1 positivity has been observed in non neoplastic endometrial stroma. Diffuse WT1 positivity is characteristic of endometrial stromal neoplasms and can aid in the differential diagnosis (14). All three of the LGESS cases showed positive staining for WT1.

The term HG-ESS has been reintroduced in the classification of EST following the discovery of t(10;17)(q22;p13) resulting in YWHAE-NUTM2A/B fusion, which is associated with distinct morphological characteristics (15). The characteristic morphological features of HG-ESS include a tumour with high mitotic activity (>20-30 mitoses/10 HPF), a fibrous or myxoid appearance, extensive lymphovascular invasion, diffuse positivity for cyclin D1, and negative staining for smooth muscle markers and WT1 (16). Genetic analysis may assist in identifying HG-ESS in cases where the diagnosis is challenging (15). Patients with HG-ESS often lack expression of ER and PR. Therefore, it remains unclear whether the ovaries should be preserved in premenopausal women with HG-ESS. In a study by Zhang YY et al., six patients underwent ovarian preservation surgery, and four of them were in Stage I without recurrence. Two additional patients, who were misdiagnosed as uterine leiomyoma, underwent hysterectomy and BSO. Both patients died due to the disease (survival was 10 and 12 months, respectively). Furthermore, the research found that the prognosis of postmenopausal patients was poor (17).

UUS is a rare uterine sarcoma that encompasses a diverse group of neoplasms with no specific line of differentiation, and its diagnosis is made by exclusion (8). UUS typically shows positive staining with p16 and focal positivity with CD10, PR, cyclin D1, and beta-catenin (18). The most common cytogenetic abnormality observed in LGESS is a recurrent translocation involving chromosomes 7 and 17, t(7;17) (p15;q21), resulting in a fusion between JAZF1 and SUZ12. Highgrade endometrial stromal sarcoma typically harbours the YWHAEFAM22 genetic fusion as a result of t(10;17)(q22;p13) (1).

Conclusion

Endometrial stromal neoplasms are rare uterine tumours that can have a fatal outcome. They can mimic a variety of benign and malignant neoplasms, making accurate diagnosis challenging. In the present case series, an equal number of Low-grade and High-grade Endometrial Stromal Sarcomas (LGESS and HGESS) were observed, while Endometrial Stromal Nodules (ESN) were the rarest. The age of presentation ranged from 34 to 75 years. Given that EST can resemble various abdominopelvic tumours, thorough pathological sampling is necessary to identify the characteristic histomorphology. Cyclin D1 and WT1 play important roles in differentiating LGESS from HGESS, as WT1 positivity is seen in LGESS and Cyclin D1 positivity is seen in HGESS. Tumour size and grade are two important factors for disease progression. Therefore, an integrated morphological and immunohistochemical study, including a large number of cases in the future, will be helpful in planning specific therapeutic strategies for each subcategory.

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DOI and Others

DOI: 10.7860/JCDR/2023/64407.18372

Date of Submission: Apr 03, 2023
Date of Peer Review: May 10, 2023
Date of Acceptance: Jun 23, 2023
Date of Publishing: Aug 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: Apr 07, 2023
• Manual Googling: May 17, 2023
• iThenticate Software: Jun 19, 2023 (13%)

ETYMOLOGY: Author Origin

EMENDATIONS: 6

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