Compared with sporadic EOC patients, both BRCA1- and BRCA2-associated patients have improved outcomes after primary therapy, including chemotherapy.

BACKGROUND:The objective of this study was to assess the incidence of primary breast cancer (PBC) and contralateral breast cancer (CBC) in patients who had BRCA1/BRCA2-associated epithelial ovarian cancer (OC). METHODS: From the database of the Rotterdam Family Cancer Clinic, patients who had BRCA-associated OC without a history of unilateral breast cancer (BC) (at risk of PBC; n ¼ 79) or with a history of unilateral BC (at risk of CBC; n ¼ 37) were selected. The control groups consisted of unaffected BRCA mutation carriers (n ¼ 351) or mutation carriers who had a previous unilateral BC (n ¼ 294), respectively. The risks of PBC and CBC were calculated using the Kaplan-Meier survival method with death considered as a competing risk event. RESULTS: Women with BRCA-associated OC had lower 2-year, 5-year, and 10-year risks of PBC (3%, 6%, and 11%, respectively) compared with unaffected mutation carriers (6%, 16%, and 28%, respectively; P ¼ .03), although they had a considerably higher mortality rate at similar time points (13%, 33%, and 61%, respectively, vs 1%, 2%, and 2%, respectively; P < .001). In BRCA mutation carriers with a previous unilateral BC, the 2-year, 5-year, and 10-year risks of CBC were nonsignificantly lower in patients with OC than in those without OC (0%, 7%, and 7%, respectively, vs 6%, 16%, and 34%, respectively; P ¼ .06), whereas the mortality rate was higher in patients with OC (19%, 34%, and 55%, respectively, vs 4%, 11%, and 21%, respectively; P < .001). CONCLUSIONS: Patients with BRCA-associated OC had a lower risk of developing a subsequent PBC or CBC than mutation carriers without OC, whereas the risk of dying from OC was greater than the risk of developing BC. These data may facilitate more tailored counseling for this patient subgroup, although confirmative studies are warranted. Cancer 2013;119:955-62. V C 2012 American Cancer Society.KEYWORDS: BRCA, ovarian cancer, primary breast cancer, contralateral breast cancer, risk-reducing mastectomy. INTRODUCTIONWomen who have a germline mutation in the breast cancer susceptibility genes BRCA1 or BRCA2 have substantially elevated risks of developing both breast cancer (BC) and ovarian cancer (OC). It has been estimated that the cumulative 10-year risk of primary BC (PBC) ranges between 6% and 23% for BRCA1/BRCA2 mutation carriers, and the highest risk is observed between ages 40 and 50 years.1-6 After unilateral BC, the estimated 10-year risk of contralateral BC (CBC) is 29% to 39% for patients with BRCA1-associated BC and 23% to 35% for patients with BRCA2-associated BC. 1,[7][8][9][10] Factors that potentially affect the latter include age at first BC diagnosis (especially when diagnosed at ages <50 years),

PFS and OS were substantially longer in BRCA2- than in BRCA1-associated EOC patients. While response rates after primary treatment were similarly high in both groups, TFI, as surrogate for chemosensitivity, was significantly longer in BRCA2 patients.

Epithelioid trophoblastic tumour (ETT) is an unusual type of trophoblastic tumour, which can cause difficulties in diagnosis and (as a consequence) in treatment. The literature suggests that surgery should be the treatment of choice for ETT as it is not responsive to chemotherapeutic agents, used in the treatment of other types of gestational trophoblastic diseases. This case report describes an ETT, which was initially diagnosed as a carcinoma of the cervix. Surgical management was chosen based on the literature. 6 months later the patient also developed a plasmacytoma and was treated with radiotherapy. The occurrence of ETT and plasmacytoma in combination has never been described before. This case report describes a rare case of an atypical trophoblastic tumour, with problematic differential diagnosis. Treatment of carcinoma of the cervix would have necessitated postoperative radiotherapy, but on diagnosis of ETT, surgical management was considered sufficient. Hence, it is important to consider the occurrence of ETTs, although rare, in patients with atypical cervical or endometrial cancer, and in patients diagnosed with a gestational trophoblastic tumour, who do not respond to appropriate chemotherapy.

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