Hormone receptor expression in patients with dermatofibrosarcoma protuberans.

Background: Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma for which the exact etiology is unknown. Case reports exist of DFSP appearing and growing rapidly during pregnancy, suggesting a hormonal role.

Objective: Our goal was to determine the expression of estrogen receptors (ERs) and progesterone receptors (PRs) in patients with DFSP.

Methods: Archived formalin-fixed, paraffin-embedded tissue from patients with DFSP in the past 20 years at a single institution were analyzed for ER and PR using immunohistochemistry. A semiquantitative scoring method was used to evaluate the expression as positive or negative. Analysis was used to determine whether there was an association between receptor positivity and tumor site, age at diagnosis, sex, race, or disease recurrence.

Results: Forty-four patients with DFSP were included in the study. Tumors were 22.7% ER/PR, 34.1% ER/PR, 9.1% ER/PR, and 34.1% ER/PR. There was no significant association between expression of ER and PR and sex, age at diagnosis, race, or tumor location. Loss of receptor expression was observed in all recurrent tumors.

Limitations: This study is limited by a lack of follow-up and a new scoring system.

Conclusions: The data presented warrant additional study to determine hormone receptor function and the potential efficacy of antihormone therapies for the treatment of patients with DFSP.