The objective of this study is to summarize the clinical and pathologic characteristics of malignant struma ovarii to facilitate the early diagnosis and treatment of this disease. All 144 patients were females from 27 countries. The mean age of the patients at diagnosis was 42.6 years. Overall, 35.71% of the patients underwent unilateral oophorectomy, 58.57% of the patients underwent bilateral oophorectomy, 5.72% of the patients were not ovariectomized, and 38.57% of the patients received radioactive iodine treatment with an average dose of 158.22 mCI each time. “Impure” types accounted for 70.19% of the cases, while pure types accounted for 29.81% of the cases. Among these cases, papillary thyroid carcinoma accounted for 50.00%, follicular thyroid carcinoma accounted for 26.47%, follicular variant of papillary thyroid carcinoma accounted for 18.63%, papillary and follicular mixed thyroid carcinoma accounted for 2.94%, anaplastic carcinoma accounted for 0.98%, and medullary carcinoma accounted for 0.98%. In total, 21 patients (51.22%) had elevated CA125. More than half of the patients (51.94%) had metastasis outside the ovary. The most common metastatic site was the pelvic cavity. The misdiagnosis rate was 17.27%. Mortality was related to metastasis and the cancer type. Gene mutations were found in the NRAS, KRAS, BRAF, and KIT genes and were similar to those in thyroid carcinoma, but some patients (37.5%) did not exhibit any gene mutations. Regardless of the treatment received, the survival rate is high. Treatment could initially include ovariectomy; however, in cases with metastasis and iodine uptake of the metastatic tumor, thyroidectomy, radioactive iodine therapy, and thyroid hormone inhibiting therapy are indicated.

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