Endometrial stromal sarcoma (ESS) is a malignant variant of endometrial stromal tumors (ESTs) and encompasses approximately 2-5% of all neoplasms originating in the endometrium, although it is rarely encountered clinical practice [1]. Some authors established an incidence rate of 1-2 per 100,000 women [1], while others claim it to be as low as 1-2 per 1 million women in 1 year [2]. Based on the degree of proliferation, ESSs are either low-grade or high-grade tumors, but all forms penetrate the myometrium of the uterus, which is the main distinguishing feature from benign forms of ESTs (endometrial stromal nodules) [1]. However, some authors claim that all ESSs should be considered as low-grade sarcomas, and the term undifferentiated endometrial sarcomas (UES) should be used to describe high-grade tumors with an incompletely disclosed phenotype instead [2] [3]. They are primarily diagnosed in women between 40-60 years of age, with low-grade tumors being more commonly identified in younger women, whereas postmenopausal females are the primary patient population of high-grade ESS [1] [2] [3] [4] [5]. The clinical presentation is comprised of nonspecific symptoms such as abdominal pain, vaginal bleeding, and progressive metrorrhagia in premenopausal women [3] [4]. A palpable abdominal mass may be detected in up to a quarter of individuals [4].

• Pain

  Abdominal pain, vaginal bleeding, and menorrhagia are main symptoms, whereas a palpable abdominal mass may be detected in a number of patients. Clinical suspicion towards this tumor must be raised early. [symptoma.com]

  In the post partum period, pelvic pain motivated a laparoscopic exploration which revealed severe peritoneal recurrence. [ncbi.nlm.nih.gov]

  The symptoms are non specific and include menorrhagia, abdominal pain or a palpable pelvic mass. Over the past four decades, EST classification has gone through various modifications starting from the earliest study by Norris and Taylor. [radiopaedia.org]

• Pathologist

  The listed tumor entities also constitute a particular diagnostic challenge for pathologists that contains numerous pitfalls and difficulties. [degruyter.com]

  Pathologic diagnosis was confirmed by a gynecologic pathologist. Long-term patient-reported outcomes were obtained via mailed survey. [ncbi.nlm.nih.gov]

  Moreover, around that time it was also shown that mitotic counts between different pathologists are not always well reproducible, due to the quality of the sections, differences in the inclusion criteria amongst pathologists of “ a mitotic figure ”, how [journals.plos.org]

  Pathologists at DF/BWCC are among the best and most experienced in the world with special expertise in providing an accurate diagnosis for complex cases. [brighamandwomens.org]

  Vascular invasion has also been shown to be present in high-grade sarcomas, with selected cases demonstrating metastases to the liver and lung. [2, 18, 27] Although there is no specific staging system for prostatic stromal sarcoma, pathologists should [emedicine.medscape.com]

• Surgical Procedure

  Tumor classification, CD10 and surgical procedures were significantly associated with disease-free survival (DFS). Surgical procedures were significantly associated with overall survival (OS). [ncbi.nlm.nih.gov]

• Swelling

  She developed left-lower-limb swelling, diagnosed by duplex scanning and magnetic resonance imaging as deep venous thrombosis in the inferior vena cava (IVC) that extended into the iliac veins on both sides, as well as the left femoral vein and right [ncbi.nlm.nih.gov]

  Symptoms Abnormal uterine bleeding Long or heavy bleeding Pelvic pain Swelling in the pelvis Bleeding between menstrual cycles or after menopause Large fibroids Diagnosis There is no way to know a woman has endometrial stromal sarcoma without removing [schmidtlaw.com]

  Symptoms of endometrial stromal sarcoma vary from case to case but common symptoms include: Abnormally long or heavy periods Pelvic pain Swelling of the pelvis Bleeding between menstrual cycles When caught early, the five-year survival rate for this type [disability-benefits-help.org]

  Corticosteroids are often used to reduce swelling and inflammation. You may be prescribed a high dose of a type of corticosteroid called a glucocorticoid. [hse.ie]

• Fatigue

  A 59-year-old female presented with new abdominal onset pain, fatigue, and nausea. She had a computed tomography that showed a pelvic mass, an enlarged right internal iliac lymph node, an atypical liver hemangioma, and a severe left hydronephrosis. [ncbi.nlm.nih.gov]

  We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: January 31, 2019 [patientslikeme.com]

  A 75-year-old woman status post total abdominal hysterectomy for “multiple leiomyomas” presented with fatigue, dyspnea, and anemia, and underwent colonoscopy where numerous “malignant” appearing polyps were noted. [academic.oup.com]

  GIST Symptoms of Gastrointestinal Stromal Tumor (GIST) Gastrointestinal stromal tumors may cause the following symptoms: Abdominal discomfort or pain Blood in stools Vomiting Feeling full after eating only a small amount Loss of appetite or weight loss Fatigue [brighamandwomens.org]

• Abdominal Pain

  Abdominal pain, vaginal bleeding, and menorrhagia are main symptoms, whereas a palpable abdominal mass may be detected in a number of patients. Clinical suspicion towards this tumor must be raised early. [symptoma.com]

  The symptoms are non specific and include menorrhagia, abdominal pain or a palpable pelvic mass. Over the past four decades, EST classification has gone through various modifications starting from the earliest study by Norris and Taylor. [radiopaedia.org]

  A 46-year-old woman with a complaint of postcoital vaginal bleeding, low abdominal pain, and constipation was admitted to the clinic. She had a mass of seven cm in size, located in the posterior fornix detected on physical examination. [ncbi.nlm.nih.gov]

• Pelvic Mass

  She had a computed tomography that showed a pelvic mass, an enlarged right internal iliac lymph node, an atypical liver hemangioma, and a severe left hydronephrosis. [ncbi.nlm.nih.gov]

  The symptoms are non specific and include menorrhagia, abdominal pain or a palpable pelvic mass. Over the past four decades, EST classification has gone through various modifications starting from the earliest study by Norris and Taylor. [radiopaedia.org]

• Abdominal Mass

  Abdominal pain, vaginal bleeding, and menorrhagia are main symptoms, whereas a palpable abdominal mass may be detected in a number of patients. Clinical suspicion towards this tumor must be raised early. [symptoma.com]

  ESS can, however, present as an intra-abdominal mass extending in the abdominal cavity; Patients with a bleeding intra-abdominal mass are often served best with stabilization, embolization of the bleeding mass, and interval surgical resection; An interdisciplinary [hbsn.amegroups.com]

  The patient was an infant of 18 months of age who presented with an incidental abdominal mass. The serum sex hormones were at prepubertal levels. There was no evidence of precocious puberty or any obvious genital anomaly. [ncbi.nlm.nih.gov]

  Patients with soft tissue sarcomas generally present with a large painless abdominal mass. [healthxchange.sg]

• Nausea

  A 59-year-old female presented with new abdominal onset pain, fatigue, and nausea. She had a computed tomography that showed a pelvic mass, an enlarged right internal iliac lymph node, an atypical liver hemangioma, and a severe left hydronephrosis. [ncbi.nlm.nih.gov]

  Nausea and vomiting. Changes to your skin. Irritations in your bladder. Edema (swelling) in your legs. Chemotherapy Chemotherapy uses medications to kill or slow the growth of rapidly multiplying cancer cells. [my.clevelandclinic.org]

  One week later a repeat biopsy was scheduled, however the patient presented at that time with worsening left upper quadrant pain radiating to the flank, associated with nausea, vomiting, night sweats, and fatigue. [hbsn.amegroups.com]

  They have really good meds to help with nausea and lots of tips to help with any other side effects. Tell your doctors and nurses whatever symptoms you might have and they can probably help you with them. Good luck with everything. [hystersisters.com]

  Signs and symptoms may include: nausea vomiting loss of appetite weight loss a lump in the abdomen abdominal pain or discomfort bleeding from the GI tract, such as vomiting blood or blood in the stool fatigue due to anemia caused by bleeding in the GI [cancer.ca]

• Skin Rash

  The most common side effects are swelling around the eyes or in the legs, a skin rash, and minor stomach discomfort or diarrhea. Remarkably, imatinib provides benefit to over 80% of patients with metastatic GIST. [curesarcoma.org]

  Side effects of imatinib can include: Tiredness Feeling sick Diarrhoea Swollen ankles Puffy eyes Itchy rash Side effects of sunitinib can include: Skin rash and soreness Tiredness Mouth ulcers High blood pressure Side effects of regorafenib can include [sarcoma.org.uk]

• Suggestibility

  SUGGESTED PROGRAMMATIC ASSESSMENT* Suggested MER for Evaluation: • Clinical history and examination that describes the diagnostic features of the impairment; • Pathology report of a biopsy specimen of the stroma of the endometrium; and • Surgery reports [secure.ssa.gov]

  Immunoreactivity for epidermal growth factor receptor was observed in both tumors, suggesting a relationship between the primary and secondary tumors in our case. [ncbi.nlm.nih.gov]

• Vaginal Bleeding

  Abdominal pain, vaginal bleeding, and menorrhagia are main symptoms, whereas a palpable abdominal mass may be detected in a number of patients. Clinical suspicion towards this tumor must be raised early. [symptoma.com]

  This case of Low Grade Endometrial Stromal Sarcoma (LGESS) in a 21 years old woman was presented as irregular vaginal bleeding. Clinical diagnosis of fibroid was made but analysis of endometrium showed ESS confirmed on hysterectomy specimen. [ncbi.nlm.nih.gov]

• Pelvic Pain

  In the post partum period, pelvic pain motivated a laparoscopic exploration which revealed severe peritoneal recurrence. [ncbi.nlm.nih.gov]

  Common manifestations include abnormal uterine bleeding and pelvic pain or mass. [msdmanuals.com]

  We present the case of a 48-year-old woman who underwent an exploratory laparotomy for pelvic pain and a suspected right ovarian mass based on imaging studies. [academic.oup.com]

  My consultant says that most recurrances occur in the pelvic region first then lungs and that they almost always cause symptoms. I had a bout of cystitis but they were very quick to give me a check up as I had pelvic pain. Big worry. [cancerresearchuk.org]

• Uterine Hemorrhage

  In our case we aimed to emhisase the importance of the uterine sarcomas. Anahtar Kelime:Case report, Sarcoma, Uterine Neoplasms, Myoma, Uterine Hemorrhage, Adult, Obstetric Surgical Procedures [atif.sobiad.com]

Despite the rare appearance of endometrial stromal sarcoma, the physician must perform a comprehensive clinical assessment in women who report with undisclosed abdominal pain and vaginal bleeding. A detailed patient history should be obtained first, during which the duration of symptoms and their severity, as well as the menopausal status, need to be assessed. Because a palpable mass was observed in a small, but a significant number of cases, abdominal inspection and palpation must not be overlooked during the physical examination. Imaging studies must be employed as soon as possible. Ultrasonography, despite its benefits in terms of convenience and time consumption, is not a reliable tool for discriminating between uterine lesions, and it is not uncommon for endometrial stromal sarcomas to be misdiagnosed as leiomyomas [1] [2] [3] [4]. For this reason, magnetic resonance imaging (MRI) is the recommended study, as it can confirm the presence of a lesion invading the myometrium and enlargement of the uterus [2]. A definite diagnosis is made after obtaining a viable sample for a histopathological examination with immunohistochemistry and uterine curettage is the crucial procedure that allows proper sampling [1] [2]. Expression of CD10 on immunohistochemistry is a highly specific sign for endometrial stromal sarcomas [2] [6] [7].

• Human Herpesvirus 8

  Human herpesvirus 8 has been implicated as a causative agent. osteogenic sarcoma a malignant primary tumor of bone composed of a malignant connective tissue stroma with evidence of osteoid, bone, and/or cartilage formation; depending upon the dominant [medical-dictionary.thefreedictionary.com]

[…] or combined treatments. [nature.com]

Aromatase inhibitor treatment may be a useful option as a second-line hormonal treatment to low-grade ESS. [ncbi.nlm.nih.gov]

Recurring tumors have worse prognosis compared to tumors that do not recur Response to treatment: Tumors that respond to treatment have better prognosis compared to tumors that do not respond to treatment Progression of the condition makes the outcome [dovemed.com]

[…] poor survival Individuals with bulky disease have a poorer prognosis Involvement of the regional lymph nodes, which can adversely affect the prognosis. [dovemed.com]

However, high-grade ESS with metastasis with YWHAE rearrangement shows a relatively favorable prognosis. [ncbi.nlm.nih.gov]

Endometrial stromal sarcomas (ESS) are rare uterine tumors with unknown etiological risk factors, but estrogen-dependent growth promotion. [ncbi.nlm.nih.gov]

The book provides a description at length of the epidemiology, etiology, pathological anatomy, prognosis, diagnosis, differential diagnosis, imaging and comprehensive therapy of each primary, relapsed, and metastasized tumor including surgery, chemo-, [degruyter.com]

Norris and Taylor suggested an etiologic relation between pelvic irradiation and uterine sarcomas. [glowm.com]

Etiology The causes of proptosis are broad and include a wide range of mass lesions that originate within the cranium, sinuses, paranasal spaces, and orbit 3: thyroid orbitopathy (the most common cause of uni/bilateral proptosis in adults) infection orbital [radiopaedia.org]

Eur J Obstet Gynecol Reprod Biol 109 : 209–213 Harlow BL, Weiss NS, Lofton S (1986) The epidemiology of sarcomas of the uterus. [nature.com]

Because of the rarity and heterogeneity of endometrial stromal neoplasms, little is known about their epidemiology, pathogenesis, and molecular pathology. [ncbi.nlm.nih.gov]

Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test Endometrial stromal tumors (EST) arise from the uterus and include the benign endometrial stromal nodule (ESN) and infiltrative [mayomedicallaboratories.com]

It remains unclear whether the diagnostic work-up is required prior to such embolization to prevent a missed diagnosis of sarcomas and a delay in providing definitive treatment. [ncbi.nlm.nih.gov]

In one study, there was 100% recurrence in patients who retained their ovaries versus 43% in those who had them removed.9 Nonetheless, there are studies that challenge the effectiveness of oophorectomy in preventing the recurrence of LGESS.15 The role [touchoncology.com]

How can Low-Grade Endometrial Stromal Sarcoma be Prevented? Current medical research has not established a method of preventing Low-Grade Endometrial Stromal Sarcoma. [dovemed.com]

Other more common forms of sarcoma who may require preventive chemotherapy include osteosarcoma (bone sarcoma) and Ewing sarcoma. We have come to the end of part one of our sarcoma series. [healthxchange.sg]

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2. Puliyath G, Nair MK. Endometrial stromal sarcoma: A review of the literature. Indian J Med Paediatr Oncol. 2012;33(1):1-6.
3. Ashraf-Ganjoei T, Behtash N, Shariat M, Mosavi A. Low grade Endometrial Stromal Sarcoma of uterine corpus, a clinico-pathological and survey study in 14 cases. World J Surg Oncol. 2006;4:50.
4. Kim DY, Lim KT, Kwon YS. Endometrial Stromal Sarcomas: A clinico-pathological analysis of 27 patients. Pak J Med Sci. 2013;29(1):72-76.
5. Baker P, Oliva E. Endometrial stromal tumours of the uterus: a practical approach using conventional morphology and ancillary techniques. J Clin Pathol. 2007;60(3):235-243.
6. Sumathi VP, McCluggage WG. CD10 is useful in demonstrating endometrial stroma at ectopic sites and in confirming a diagnosis of endometriosis. J Clin Pathol. 2002;55:391–32.
7. Chu PG, Arber DA, Weiss LM, Chang KL. Utility of CD10 in distinguishing between endometrial stromal sarcoma and uterine smooth muscle tumors: An immunohistochemical comparison of 34 cases. Mod Pathol. 2001;14:465–471.