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].

• 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]

• Soft Tissue Mass

  A 43-year-old, nulligravid, Caucasian woman presented for an annual gynecologic examination and was found to have an asymptomatic 5 x 5-mm, rubbery, soft tissue mass at the 5 o'clock position of the vaginal introitus. [ncbi.nlm.nih.gov]

  tissue mass at the 5 o�clock position of the vaginal introitus. [reproductivemedicine.com]

  Chest CT revealed a tightly packed elongated endobronchial soft tissue mass completely obstructing the right intermediate bronchus and a solitary round nodule, 3.6 cm×2.6 cm in size, in the medial basal segment of right lower lobe (figure 1). [thorax.bmj.com]

• Accelerated Growth

  Recurrence is usually confined to the pelvic space but sometimes accelerated growth and extension into the upper abdomen can be found. [ncbi.nlm.nih.gov]

• Wheelchair Bound

  While surviving at 24 months from recurrent disease, the second became paraplegic with double incontinence and was wheelchair bound. [ncbi.nlm.nih.gov]

• 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]

  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]

• Venous Insufficiency

  Upon physical examination, cachexia was apparent, with a palpable tumor from the xiphoid process to the pubis, and a Grade 2 venous insufficiency in the left leg (Figure 1,2). [austinpublishinggroup.com]

• Short Arm

  Cytogenetic analysis has been reported in 47 cases of ESS and has revealed three recurrent karyotypic features: 1) Chromosomal translocation t(7;17)(p15∼p21;q12∼q21) in 12 cases (25.5%); 2) Rearrangement of the short arm of chromosome 6, particularly [journals.plos.org]

• Radiculopathy

  […] symptoms, such as hematuria, urgency, frequency, and incontinence.2–4 Several unusual sites of presentation for LGEESS described in the literature include dysphagia and hoarseness with laryngeal involvement, pelvic fracture from a primary bone LGEESS, radiculopathy [archivesofpathology.org]

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].

• Cavitary Lesion

  A 57-year-old woman, who had undergone hysterectomy for uterine myoma 11 years earlier presented with cystic, nodular and cavitary lesions simultaneously visible on computed tomography images of the chest. [ncbi.nlm.nih.gov]

• Multiple Pulmonary Nodules

  We are reporting a case of a 47-year-old female with no significant medical history who presented with multiple pulmonary nodules. Fine needle aspiration (FNA) specimen revealed spindle cell neoplasm consistent with the diagnosis of LGESS. [ncbi.nlm.nih.gov]

• Pericardial Effusion

  More suggestive findings of cardiac metastasis include a focal cardiac contour abnormality and radiographic evidence of a pericardial effusion. [oatext.com]

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

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

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

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]

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]

Other interventions vary by etiology. Graves exophthalmos is not affected by treatment of the thyroid condition but may lessen over time. Tumors must be surgically removed. [msdmanuals.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]

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]

Data from the Surveillance Epidemiology and End Results program on low- and high-grade ESS for 1973 through 2003 were obtained. [academic.oup.com]

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]

Prevention - Endometrial stromal sarcoma Not supplied. [checkorphan.org]

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]

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]

Another chapter is devoted to the prevention of subjecting sarcomas to inadequate surgical therapeutic measures under the assumed diagnosis of leiomyoma, and includes a diagnostic-therapeutic flowchart with a diagnostic score. [degruyter.com]

1. Puliyath G, Nair VR, Singh S. Endometrial stromal sarcoma. Indian J Med Paediatr Oncol. 2010;31(1):21-23.
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.