Abstract：
BACKGROUND: 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.
METHODS: 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.
CONCLUSIONS: 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.
METHODS: 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.
CONCLUSIONS: 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.
摘要：
背景：肺泡软组织肉瘤（ASPS）是一种罕见的恶性肉瘤，仅在鼻窦和头颈部报告少数病例。它显示了强大的女性优势。从未报告激素依赖性复发。
方法：一名35岁女性在第一次怀孕期间出现鼻腔ASPS，第二次中耳ASPS，在任何围产期之外的鼻窦进行第三次ASPS，伴随不利的进展和转移，终结于死亡。
结论：肿瘤的病理分析显示，在两次围产期中，孕激素受体免疫标记阳性。因此，这是具有强孕酮敏感性的ASPS围产期复发的第一份报告，加强ASPS和孕酮之间的可疑生物学联系。此病例报告可能是一项初步发现，表明孕酮阻滞剂可作为复发性ASPS的新疗法。
方法：一名35岁女性在第一次怀孕期间出现鼻腔ASPS，第二次中耳ASPS，在任何围产期之外的鼻窦进行第三次ASPS，伴随不利的进展和转移，终结于死亡。
结论：肿瘤的病理分析显示，在两次围产期中，孕激素受体免疫标记阳性。因此，这是具有强孕酮敏感性的ASPS围产期复发的第一份报告，加强ASPS和孕酮之间的可疑生物学联系。此病例报告可能是一项初步发现，表明孕酮阻滞剂可作为复发性ASPS的新疗法。
