Citation: Journal of the Endocrine Society. 2(8):944-948, 2018 Aug 01..Journal: Journal of the Endocrine Society.Published: 2018ISSN: 2472-1972.Full author list: Gomes-Lima CJ; Nikiforov YE; Lee W; Burman KD.UI/PMID: 30087949.Subject(s): IN PROCESS -- NOT YET INDEXEDInstitution(s): MedStar Health Research Institute | MedStar Washington Hospital CenterDepartment(s): Medicine/EndocrinologyActivity type: Journal Article.Medline article type(s): Journal ArticleOnline resources: Click here to access onlineDigital Object Identifier: https://dx.doi.org/10.1210/js.2018-00132 (Click here)Abbreviated citation: J. endocr. soc.. 2(8):944-948, 2018 Aug 01.Abstract: Struma ovarii is a rare ovarian teratoma predominantly composed of thyroid tissue. The simultaneous presence of thyroid carcinoma in the struma ovarii and the thyroid gland is extremely rare. It remains unclear if these carcinomas represent independent primary tumors and whether the molecular mechanisms of the tumors developing in the thyroid and ovarian tissues are similar. We present the case of a patient with two independent papillary thyroid carcinomas (PTCs) in struma ovarii and the thyroid gland that are driven by different RAS mutations. A 62-year-old woman with a history of chronic lymphocytic leukemia/small lymphocytic lymphoma was diagnosed with a pelvic mass during a CT scan. She had surgery that included removal of her ovaries. A 7.2-cm classical variant of PTC arising in a struma ovarii was identified in the right ovary. Two months after the pelvic surgery, total thyroidectomy was performed, and a small nodule (0.8 cm) in the left lobe was diagnosed as a classical variant of PTC. Molecular analysis of tissues obtained from both the malignant struma ovarii and thyroid gland was performed. RAS mutations both in the PTC located in the thyroid and ovarian tissues were identified. However, whereas the thyroid gland tumor showed an HRAS Q61R mutation, the PTC in struma ovarii harbored an NRAS Q61R mutation. In this case, the finding of distinct types of RAS point mutation in thyroid cancers at two different locations provides definitive evidence that these cancers are synchronously developed independent primary tumors.