转移性Lynch综合征相关性妇科肿瘤的预后

OUTCOME OF METASTATIC LYNCH SYNDROME-ASSOCIATED GYNECOLOGIC CANCERS

Introduction: DNA mismatch repair is defective in Lynch syndrome associated cancers, but little data exists regarding their sensitivity to therapies that cause DNA damage. The objective of this study was to assess the response to treatment and survival of metastatic gynecologic cancers in women with Lynch syndrome.
Methods: Twenty-one patients (19 uterine, 1 ovarian, 1 fallopian tube) were identified from three institutions. Records were reviewed to determine patient characteristics, treatment and survival.
Results: Among 14 patients with primary advanced stage cancers, 7 had only nodal metastases and 7 had peritoneal and/or adnexal metastases. Adjuvant treatment included: chemotherapy and radiation (8), chemotherapy alone (5), radiation alone (1). Twelve of 14 patients (86%), including 7 with measurable disease after primary surgery, are alive without disease with a median survival of 69 months, and 10 of these patients are 5-year survivors. Among 7 patients treated for recurrent cancers, 2 had nodal metastases only and 5 had abdominal disease. Treatment included: chemotherapy and radiation (4), radiation alone (2), chemotherapy alone (1). Median survival was 57 months and 5/7 (71.4%) are alive without disease, including 5 patients who had measurable disease. Overall, 17/21 (81%) patients remain alive without disease.
Conclusions: Women with metastatic Lynch Syndrome-associated gynecologic cancers had a favorable response to therapy and were frequently long term survivors. These data suggest that, like BRCA1/2 associated ovarian cancers, metastatic gynecologic cancers that occur in women with Lynch Syndrome may also be highly sensitive to treatment with DNA damaging agents such as chemotherapy and radiation.

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