[Clinical features and disease course in patients with BRCA1-dependent ovarian cancer]. / Cechy kliniczne i przebieg choroby u chorych na BRCA1-zaleznego raka jajnika.

ABSTRACT

INTRODUCTION: Ovarian cancer (OC) remains a challenge for gynecologic oncologists due to poor prognosis and increasing morbidity. About 10% of cases is hereditary and BRCA1 gene mutation-dependant. Some authors claim that clinical features, the course of the disease and prognosis of BRCA1-dependent OC vary between sporadic cases. AIM OF THE STUDY To analyze clinical features and disease courses of BRCA1-dependent OC in the material from Center of Oncology Cracow Branch. MATERIAL AND METHODS: Between 2004 and 2008, 66 mutations of BRCA1 gene were found in patients with OC. All patients were treated with primary surgery followed by platinum-based chemotherapy Outcomes were assessed by means of clinical examination and imaging tests. Patients with complete response were followed up in the outpatient office. Secondary chemotherapy was administered if persistent or progressive disease was diagnosed. RESULTS: In the analyzed group of 66 (100%) patients, the following mutations of BRCA1 gene were found in 31 (47%) - C61G (exon 5), in 21 (31,8%) - 5382insC (exon 20), in 6 (9.1%) - 185delAG and in 8 (12.1%) - other (exon 11). Mean patient age was 48. FIGO stage I and stage II were diagnosed in 7 (10,6%), stage III in 58 (89,9%) and stage IV in 1 patient (1,5%). Twenty five (37.9%) patients underwent complete macroscopic primary cytoreduction. Platinum-based chemotherapy was administered to all 66 patients after surgery Complete response (CR), partial response (PR) and progressive disease (PD) was achieved in 31 (46.9%), 30 (45,5%) and 5 (7.6%) patients, respectively Secondary surgery was performed in 29 (43.9%) of patients after completion of adjuvant therapy Second-line chemotherapy was administered in 40 (60.6%) patients due to residual or progressive disease. Mean time of follow-up was 65 months. Forty one (62. 1%) patients died due to OC progression. CONCLUSIONS: Clinical features and disease courses in BRCA1-dependent OC patients in the analyzed group were similar to other results reported in the literature.