The course of epithelioid sarcoma is often unpredictable and it is common for patients to present with extensive disease, lymph node metastases, or distant metastases. [pathologyoutlines.com]

In the present study, the case of a patient with vulvar PES is presented and the US and CDUS findings are discussed. [ncbi.nlm.nih.gov]

Johnston Medicine, Biology Ultrastructural pathology 1993 A 47-year-old man presented with a soft tissue mass of the distal right thigh near the knee that appears to be a predominantly epithelioid form of monophasic synovial sarcoma, which is important [semanticscholar.org]

Case presentation In January 2012 a 63 year-old woman presented to the Department of Obstetrics and Gynecology of “Tor Vergata” University of Rome because of a pruritic vulvar plaque with irregular margins appeared three months before in the vulvar paraclitoral [diagnosticpathology.biomedcentral.com]

• Pathologist

  The initial diagnosis was successively confirmed by another external pathologist. Surgical margins were positive. [diagnosticpathology.biomedcentral.com]

• Suggestibility

  Evidence suggesting a more aggressive behavior than extra-genital epithelioid sarcoma. Cancer. 1983, 52: 1462-1469. 10.1002/1097-0142(19831015)52:8<1462::AID-CNCR2820520821>3.0.CO;2-Y. [diagnosticpathology.biomedcentral.com]

  […] in the fingers and hand Proximal type: more common in deep soft tissue, truncal tissue (including pelvic peritoneal, genital and inguinal) and buttock / hip Pathophysiology Tumor of uncertain differentiation Structural and immunohistochemical studies suggest [pathologyoutlines.com]

  The initial pathology report suggested that the mass was rich in myxoid stroma. Subsequently, the surgical specimen was sent to the West China Hospital for consultation. [ncbi.nlm.nih.gov]

Optimal treatment for PES of the vulva has not been established due to its rarity. There is no universally accepted treatment for vulvar epithelioid sarcoma (12). [ncbi.nlm.nih.gov]

Frequently histological diagnosis was missed at first and the patients typical experience was that of repeated biopsies before definitive treatment. [diagnosticpathology.biomedcentral.com]

Wide surgical excision remains the mainstay of treatment (Am J Surg 2008;196:407) Systemic chemotherapy provides satisfactory palliation but response is of short duration (Adv Anat Pathol 2016;23:41) Relapse rates remain high despite multimodal therapy [pathologyoutlines.com]

[…] general A malignant mesenchymal neoplasm that exhibits epithelioid cytomorphology and a predominantly epithelial phenotype Essential features Malignant epithelioid tumor occurring in pediatric and adult populations Unpredictable clinical course; better prognosis [pathologyoutlines.com]

The lack of symptoms in the first period and the rapid tendency of spread to the body justify the poor prognosis of these cases. [diagnosticpathology.biomedcentral.com]

[…] differentiation Structural and immunohistochemical studies suggest a multidirectional differentiation, including epithelial, histiocytic, fibroblastic, myofibroblastic, endothelial and perineural (Arch Pathol Lab Med 2009;133:814, Adv Anat Pathol 2006;13:114) Etiology [pathologyoutlines.com]

[…] or conventional type is also known as distal type Proximal type is also known as large cell type Defunct name: sarcoma aponeuroticum ICD coding ICD-10: C49.0 - malignant neoplasm of other connective and soft tissue ICD-O: 8804/3 - epithelioid sarcoma Epidemiology [pathologyoutlines.com]

Sites Either type may arise anywhere Classic type: usually distal upper extremity, > 60% arising in the fingers and hand Proximal type: more common in deep soft tissue, truncal tissue (including pelvic peritoneal, genital and inguinal) and buttock / hip Pathophysiology [pathologyoutlines.com]

Any benefit of radiotherapy and/or chemotherapy in preventing recurrence or for palliation has not been demonstrated. [diagnosticpathology.biomedcentral.com]