Nephroblastoma, also known as Wilms tumor, is a malignant neoplasm that typically accounts for more than 90% of renal tumors in children. 1 Classically, nephroblastoma frequently exhibits a polyphasic differentiation pattern with blastemal, stromal, and epithelial components. 2 Teratoma with nephroblastoma (TWN) is a rare variant of it, first described by Variend et al. in 1984. 3 According to Fernandas' criteria, the TWN should be defined as the triphasic tumor in which heterologous elements like cartilage, muscles, adipose tissue, glial tissue constituted more than 50% of the mass. 4 To the best of our knowledge, 45 cases of TWN have been reported in English literature, while only two of them were primary ovarian TWN.We recently had an additional case of an adult ovarian TWN.Due to rupture and spillage of the tumor cells, dissemination to the
BackgroundNiraparib, a poly (ADP-ribose) polymerase (PARP) inhibitor, is approved for first/second-line maintenance treatment of ovarian cancer patients with complete or partial response to platinum-based chemotherapy, and multi-line monotherapy in BRCAmt patients or platinum-sensitive recurrence patients with homologous recombination deficiency (HRD) positive. We present real-world experience from China. MethodsPatients with niraparib in Jiangsu Cancer Hospital between June 2019 to July 2020 were recruited. The initial dose was given according to individualization. Response and adverse events (AEs) were analyzed by Response Evaluation Criteria in Solid Tumors v1.1. and National Cancer Institute Common Terminology Criteria for Adverse Events v5.0, respectively. HRD testing (AmoyDx®) was detected in most patients. Treatment was given until unequivocal progression or intolerable toxicity. ResultsTwenty-two patients all received niraparib at an bolus of 200mg/d. 50% of patients with high-grade serous ovarian cancer are HRD-positive. Six patients underwent first-line maintenance therapy. Sixteen patients received exploratory therapy. Ultimately image evaluation revealed that two patients achieved partial response (PR) and one patient achieved stable disease (SD), yielding objective response rate (ORR) of 33.3% (95%CI=0.060-0.759) and DCR of 50% (95%CI=0.140-0.861) in the exploratory multi-line monotherapy group. Commonly AEs were nausea, thrombocytopenia, and anemia. Grade 3-4 thrombocytopenia were managed by dose reduction and interruption. Leg swelling was observed as a new adverse event. ConclusionIt is feasible for patients received a bolus of 200mg/d in Chinese population with promising efficacy and well tolerated. This is first real world data about niraparib in ovarian cancer patients with HRD status from China.
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