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Molecularly and histologically, MSO mirrors thyroid carcinoma and includes analogous subtypes as defined in the 2022 WHO classification: \u2018BRAF\u2010like\u2019 tumours (commonly driven by BRAF^V600E mutations or kinase fusions) and \u2018RAS\u2010like\u2019 tumours (driven by mutations in the RAS pathway), along with rare high\u2010grade variants with aggressive behaviour. Next\u2010generation sequencing shows that MSO harbours a mutational spectrum closely matching primary thyroid cancers. Genotype\u2010guided targeted therapies (e.g. BRAF\/MEK inhibitors, selective RET or NTRK inhibitors and multikinase inhibitors) are emerging as promising options for advanced or radioiodine\u2010refractory cases. Surgical excision of the ovarian tumour is typically curative for localized disease. Thyroidectomy followed by radioactive iodine (RAI) is reserved for high\u2010risk tumours. Long\u2010term surveillance is essential, as late recurrences can occur. In this first comprehensive review of MSO, we integrate our own case series findings with published data to provide an up\u2010to\u2010date synthesis of its clinicopathologic spectrum and management.<\/jats:p>","DOI":"10.1111\/his.70119","type":"journal-article","created":{"date-parts":[[2026,2,13]],"date-time":"2026-02-13T07:09:21Z","timestamp":1770966561000},"page":"26-46","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Malignant struma ovarii: Advances in molecular pathogenesis, classification, diagnosis and treatment"],"prefix":"10.1111","volume":"89","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0457-0097","authenticated-orcid":false,"given":"Wanrun","family":"Lin","sequence":"first","affiliation":[{"name":"Laboratory of Pathology National Cancer Institute, National Institutes of Health  Bethesda Maryland 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