Primary Cutaneous Clear Cell Sarcoma, Clinical Outcome With Sentinel Lymph Nodes Status

American Journal of Clinical Pathology
Albert AlhatemW Clark Lambert

Abstract

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 infl...Continue Reading

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Citations

Jul 9, 2020·Journal of Cutaneous Pathology·Rand Abou ShaarBen J Friedman
Jun 19, 2021·The American Journal of Dermatopathology·Jeremy K BrayChristine S Ahn

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