Primary cutaneous clear cell sarcoma (PCS) is a rare malignancy and difficult to differentiate from melanoma. We investigated factors influencing survival and recurrence.
An institutional cancer registry and literature search were used for a retrospective study. Only clear cell sarcoma cases with a primary site of skin and subcutaneous tissue were included. Kaplan-Meier and Cox regression analyses were used to assess survival time and hazard ratios.
Three eligible cases were identified at our institution. In addition, the PubMed and Google Scholar reviews identified 1,878 items, with 23 patients with PCS. The median age was 25 years with 62% female. The tumors ranged in size from 0.4 to 4.5 cm. Cytogenetics showed t(12;22)(q13;q12) in all cases and a unique variant of t(2;22)(q32.3;q12) in one case. Surgery was the most common treatment, followed by chemotherapy/radiation. PCS recurred in 46% of patients with a median relapse-free survival time of 15 months. Only two known PCS-related mortalities were recorded, at 38 and 60 months following initial diagnosis. Smaller tumor size and negative sentinel lymph node biopsy (SLNB) status were significantly associated with a better disease-free survival.
Tumor size and SLNB status influence PCS survival and recurrence. More research is needed due to the rarity of this disease.
1. Primary cutaneous clear cell sarcoma (PCS) has features that overlap with primary melanoma mostly without an epidermal component, making it a diagnostic challenge; however, it has a better prognosis.2. Tumor size and sentinel lymph nodes biopsy status were the most influential factors for survival in patients with PCS.3. Nearly half of patients showed recurrence; therefore, clinical monitoring is recommended.

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