Recurrent peripartum alveolar soft-part sarcoma: Case report using CARE methodology.

ABSTRACT

INTRODUCTION: Alveolar soft-part sarcoma (ASPS) is a rare malignant sarcoma with only a few cases reported in the sinus and head and neck region. It shows strong female predominance. Hormone-dependent recurrence was never reported. CASE REPORT A 35 year-old woman presented nasal cavity ASPS during her first pregnancy, middle-ear ASPS during the second, and a third ASPS in the sinus outside of any peripartum period, with unfavorable progression and metastasis, terminating in death. DISCUSSION: Pathology analysis of the tumors showed positive immunolabeling for progesterone receptors in the two peripartum episodes. This was thus the first report of peripartum recurrence of ASPS with strong progesterone sensitivity, reinforcing the suspected biological link between ASPS and progesterone. This case report may be a preliminary finding suggesting progesterone blockers as a novel treatment for recurrent ASPS.