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Endometrial Stromal Sarcoma

Endometrial stromal sarcoma is a rare type of uterine malignancy. It is typically diagnosed in women during their fifth and sixth decade of life. 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. Magnetic resonance imaging, together with a biopsy obtained during curettage and subsequent histopathological examination, as well as immunohistochemistry, are crucial components of the workup.


Presentation

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]
Ecchymosis
  • A 40-year-old woman was referred due to generalized ecchymosis 7 years after laparoscopic-assisted myomectomy. Coagulogram was markedly deranged with severe thrombocytopenia.[ncbi.nlm.nih.gov]
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]
Respiratory Distress
  • She developed acute respiratory distress, from which she recovered after transfer to the intensive care unit.[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]
  • Other associated symptoms were the abdominal enlargement and the presence of the pelviabdominal mass generated by the tumor, low to medium abdominal pain or polakiuria.[ncbi.nlm.nih.gov]
Peritoneal Disease
  • All 3 were associated with an aggressive clinical course, including multisite bony metastases in 1 patient, progressive peritoneal disease after chemotherapy in another, and metastases to the lung and skin in the last patient.[ncbi.nlm.nih.gov]
Arthralgia
  • Myalgias, hot flashes, and arthralgias were not observed during the follow-up period in any patients.[ncbi.nlm.nih.gov]
  • Toxicities during aromatase inhibitor treatment - any dose reduction/ discontinuation Toxicities observed during 1 st line treatment with AIs included only grade 1–2 side effects; specifically, grade 1 hot flushes in 2 patients, grade 2 arthralgias in[clinicalsarcomaresearch.biomedcentral.com]
Myalgia
  • Myalgias, hot flashes, and arthralgias were not observed during the follow-up period in any patients.[ncbi.nlm.nih.gov]
Adnexal Mass
  • View Article Google Scholar Mathevet P, Nessah K, Dargent D, Mellier G: Laparoscopic management of adnexal masses in pregnancy: a case series. Eur J Obstet Gynecol Reprod Biol. 2003, 108: 217-222.[jmedicalcasereports.com]
  • Definitive diagnosis of any uterine sarcoma based on sonography alone is not possible [ 5 ] because these kinds of tumors may have nonspecific findings such as polypoid endometrial masses, endomyometrial thickening, and adnexal masses.[ajronline.org]
  • Pelvic examination displayed a nontender mass from the lateral wall of the uterus without pain, and no adnexal mass was palpatable.[alliedacademies.org]
  • Clinically, a level of CA125 1,000 m m accompanied by a pelvic or adnexal mass would suggest ovarian epithelial carcinoma.[karger.com]
Urinary Retention
  • We report a case of a large endometrial stromal sarcoma in a 42 year nulliparous woman with chronic kidney disease presenting with acute urinary retention and irregular per vaginal bleeding.[ncbi.nlm.nih.gov]
  • We report a case of a 39-year-old female who presented with irregular bleeding per vaginum and urinary retention.[jnsbm.org]
  • Definition / general A rare entity ( Pathology 2013 Feb;45:104 ) Usually presents with urinary retention; also abnormal digital rectal examination, hematuria or hematospermia, palpable rectal mass Includes phyllodes tumors (like those of the breast) Essential[pathologyoutlines.com]
  • Clinical Features and Imaging Common clinical presentations in men with prostatic stromal sarcoma are urinary retention, abnormal digital rectal examination (DRE), hematuria or hematospermia, and a palpable rectal mass.[emedicine.medscape.com]
Incontinence
  • While surviving at 24 months from recurrent disease, the second became paraplegic with double incontinence and was wheelchair bound.[ncbi.nlm.nih.gov]
  • 2007 Surgery Type : TAH Ovaries : Removed both low-grade endometrial stromal sarcoma The link is: I have a weird situation (I think, or maybe this is normal), I had a routine ablation on 10/8 for abnormal menstrual bleeding and a cystoscopy for urinary incontinence[hystersisters.com]

Workup

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]
Ovarian Mass
  • Intraoperative findings included a solitary solid and multicystic right ovarian mass without evidence of any extraovarian disease.[ncbi.nlm.nih.gov]
  • Sonographic examination suggested an appendicular plastron and was interpreted as an ovarian mass. Subsequently, a laparoscopy was performed in a district hospital.[jmedicalcasereports.com]
Foam Cell
  • Foam cells were present in 6 tumors. The tumors showed minimal cytologic atypia. The mitotic index ranged from[ncbi.nlm.nih.gov]
  • May have foam cells or hyalinization in the stroma.[en.wikipedia.org]

Treatment

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

Prognosis

  • 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]

Etiology

  • 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 Unknown. Epidemiology The true incidence of t(X;17) LGESS is unknown. Two cases in females of respectively reproductive and postmenopausal age have been reported to date (Amant et al.,2003; Dewaele et al, 2014).[atlasgeneticsoncology.org]
  • These findings were suggestive of neoplastic etiology. In view of above findings t otal abdominal hysterectomy with bilateral salpingo-o o phorectomy was performed.[jpgo.org]

Epidemiology

  • 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]
Sex distribution
Age distribution

Pathophysiology

  • 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]

Prevention

  • 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]
  • 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]
  • Prevention - Endometrial stromal sarcoma Not supplied.[checkorphan.org]
  • […] local recurrence in the pelvis but clearly does not prevent recurrences at distant sites. 5 There is no significant difference in overall survival or in disease-free survival comparing those who undergo post-operative pelvic radiation and those who do[touchoncology.com]
  • 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]

References

Article

  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.

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Last updated: 2019-06-28 09:58