Abstract

Malignant struma ovarii is a rare malignant germ cell tumor of the ovary. Due to the rarity of this disease, treatment has not been uniform throughout the published literature. We present three cases of malignant struma ovarii. Following primary surgery, all were subsequently treated with thyroidectomy and (131)I ablation therapy, two patients as first line management, one following the occurrence of metastatic disease. Histological diagnosis of malignant struma ovarii is similar to that of well differentiated thyroid carcinoma (WDTC). In line with the latest advice on treatment of WDTC, we believe that the best option for patients with malignant struma ovarii is surgical removal of the ovarian lesion followed by total thyroidectomy which allows the exclusion of primary thyroid carcinoma, and in addition, allows radioiodine ((131)I) ablation therapy for (micro) metastasis. After thyroidectomy, thyroglobulin can be used as a tumor marker for follow-up. Moreover, nuclear medicine imaging using radioiodine ((123)I) can be performed to demonstrate metastatic carcinoma. A multidisciplinary approach is essential.

Highlights

  • In line with the latest advice on treatment of well differentiated thyroid carcinoma (WDTC), we believe that the best option for patients with malignant struma ovarii is surgical removal of the ovarian lesion followed by total thyroidectomy which allows the exclusion of primary thyroid carcinoma, and in addition, allows radioiodine (131I) ablation therapy for metastasis

  • Thyroglobulin can be used as a tumor marker for follow-up

  • The diagnosis of malignant struma ovarii is based on classical criteria for papillary or follicular thyroid carcinoma including overlapping ground glass nuclei in the former and vascular space invasion and capsular penetration in the latter

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Summary

Conclusion

Malignant struma ovarii is a rare gynecological endocrine- oncological disorder. Due to its rarity there is little information about the natural course of this disorder after surgical resection and the best. If the diagnosis of malignant struma ovarii can be made at the time of surgery, a complete surgical removal of the tumor should be done. In line with the treatment and follow-up of WDTC we believe that the best option for patients with malignant struma ovarii larger than one cm is total thyroidectomy followed by 131I ablation therapy. After 131I ablation any detectable serum thyroglobulin points to persistent or recurrent disease. The highly sensitive post-ablation total body scan can demonstrate completeness of surgical excision or indicate metastatic disease. A multidisciplinary approach is essential, whereby the gynecologist, surgeon, endocrinologist, pathologist and nuclear physician are working closely together

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