Related Experiment Video
Updated: Mar 11, 2026
06:15
Author Spotlight: Anterior HR-OCT as a Non-Invasive Tool for Characterizing Ocular Surface Squamous Neoplasia
Published on: August 9, 2024
1.9K
Clear cell sarcoma of vulva. A case report
Ceskoslovenska Patologie
|November 22, 2016
Summary
This report details the first known case of clear cell sarcoma (CCS) of the vulva in a 67-year-old female. The rare vulvar cancer presented with widespread metastases and unique histological features.
Area of Science:
- Gynecologic Oncology
- Pathology
- Rare Cancers
Background:
- Clear cell sarcoma (CCS) is a rare soft tissue sarcoma.
- Vulvar cancers are uncommon, with squamous cell carcinoma being the most prevalent type.
- This case represents a unique presentation of CCS in the vulvar region.
Observation:
- A 67-year-old female presented with a large, exophytic vulvar mass (20 x 15 cm).
- The tumor exhibited metastases to the lung, inguinal, and pelvic lymph nodes at diagnosis.
- Histological examination revealed S-100 protein, focal Melan A, and HMB45 expression, with EWSR1 gene rearrangement.
Findings:
- The vulvar tumor demonstrated histological features consistent with clear cell sarcoma.
- Immunohistochemistry and genetic analysis confirmed the diagnosis.
- The patient presented with advanced disease, including distant and nodal metastases.
Implications:
- This case highlights the importance of considering rare sarcomas in the differential diagnosis of vulvar masses.
- Early detection and accurate diagnosis are crucial for effective management of vulvar clear cell sarcoma.
- Further research is needed to understand the behavior and optimal treatment strategies for this rare malignancy.
