Systemic Therapy for Non-Serous Ovarian Carcinoma

Yanin Chávarri-Guerra; Eduardo González-Ochoa; Héctor De-la-Mora-Molina; Enrique Soto-Perez-de-Celis

Disclosures

Chin Clin Oncol. 2020;9(4):52 

In This Article

Carcinosarcoma

Ovarian carcinosarcoma is a rare tumor composed of malignant epithelial and mesenchymal components. It is estimated that carcinosarcoma represents 1–4% of all EOC.[58–60] Patients diagnosed with carcinosarcoma are significantly older at the time of presentation,[61,62] more often present with advanced stage disease,[62] and have a shorter OS when compared to other EOC subtypes[63] (Table 2).

Although prospective evidence is limited since patients with carcinosarcoma are usually excluded from RCT, the management of carcinosarcoma is similar to other ovarian tumors and includes cytoreductive surgery followed by adjuvant chemotherapy for women with advanced disease.[29] Therapeutic strategies for carcinosarcoma have been extrapolated from the management experience of both EOC and of uterine carcinosarcoma, anecdotal experience, and/or small retrospective and prospective series. Treatment regimens utilized for carcinosarcoma have included platinum, paclitaxel, ifosfamide, doxorubicin and dacarbazine as single-agents or in combination. A prospective cohort study led by GOG showed that among 44 patients with carcinosarcoma treated with cisplatin (50 mg/m2), the RR was 20%, with a median PFS and OS of 5.2 and 11.7 months, respectively.[64] Other first-line treatments, such as doxorubicin, have shown a disappointingly low RR of only 10%,[65] while second-line therapy with ifosfamide plus mesna showed a RR of 17.9%.[66]

In the absence of RCT and under the premise that the combination of carboplatin and paclitaxel has proven to be effective in the treatment of EOC, guidelines suggest utilizing this regimen for the treatment of carcinosarcoma.[29] Data from a retrospective study conducted at the Massachusetts General Hospital found that first-line carboplatin achieved a RR of 72% in women with primary carcinosarcoma of the ovary (n=26), with an OS of 27.1 months.[67] Another case-control study including 50 patients with carcinosarcoma reported a 62% RR and a median OS of 24 months (95% CI, 18–29) when treated with carboplatin and paclitaxel, although this was lower than seen in other EOC subtypes.[68] The combination of cisplatin and ifosfamide, which has been proven effective in the treatment of uterine carcinosarcoma, has also been used for ovarian tumors, even achieving better results than paclitaxel/carboplatin in a small study.[69]

Unfortunately, patients with advanced stage carcinosarcoma rapidly develop platinum resistant tumors[61] and there is no evidence regarding the use of second-line chemotherapy in these cases. Future areas for research into the treatment of this malignancy include the use of targeted therapies, including those targeting HER2.[70]

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