Presentation
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]
Skin
- Skin Ulcer
[…] involving the skin and subcutaneous tissues (Skeletal Radiol 2002;31:400) Radiology images Contributed by Borislav Alexiev, M.D. [pathologyoutlines.com]
Treatment
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]
Prognosis
[…] 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]
Etiology
[…] 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]
Epidemiology
[…] 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]
Pathophysiology
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]
Prevention
Any benefit of radiotherapy and/or chemotherapy in preventing recurrence or for palliation has not been demonstrated. [diagnosticpathology.biomedcentral.com]