A 36-year-old woman presented with an intraperitoneally disseminated malignant struma ovarii, diagnosed by histopathology and 13'1 scintigraphy. The serum thyroglobulin level was elevated, and immu-noperoxidase staining for thyroglobulin was positive for disease both in the tumor cells lining the follicles and in the colloid. The patient was treated successfully by a bilateral ovariectomy followed by a total thyroidectomy and administration of radioactive iodine. The clinical behavior and the presence of thyroglobulin in both serum and tumor tissue demonstrate the similarity between neoplastic thyroid tissue in the ovary and in the thyroid gland. Cancer 60:178-182,1987. ERM CELL TUMORS represent 15% to 20% of all G ovarian tumors, ' 3 ' but in patients younger than 20 years of age, they account for approximately 60% of the tumors.* Only 5% of ovarian germ cell tumors are mdig-nant.3 Fortunately, 95% of all germ cell tumors are benign cystic teratomas, not leading to disagreements in diagnosis or treatment. In 5% to 15% of the teratomas, thyroid tissue may be present, but the term struma ovarii is reserved for tumors that mainly consist of thyroid tissue. Because this occurs only in less than 25% of those t u m ~ r s , ~. ~ struma ovarii represents about 2% of all teratomas. Five percent of all struma ovarii cases may be classified as malignant.6 Although most other malignant germ cell tumors respond well to combination chemo-therapy,' the treatment of a malignant struma ovarii remains controversial. According to some authors, the tumors respond well to treatment with radioactive iodine .*q9 This requires specific diagnostic and therapeutic measures, which will be discussed in the following case history. Leeuwarden, who made the initial diagnosis and did the immunoper-oxidase staining, and M. E. Pol, gynecologist, the Sionsberg Hospital, Dokkum, for refemng the patient. Mrs. W. J. A. Bruins-van der Weij has put everything into a readable form. Case Report In April 1984, a 36-year-old patient was admitted to another hospital with micturition complaints. Physical examination revealed an enlarged left ovary approximately 10 cm in diamter, which was confirmed during laparotomy. The left ovary was removed and a biopsy from the right ovary was taken. Gross examination showed a round to oval tumor measuring 10 X 10 X 6 cm with a smooth, slightly lobulated surface. On cross-section, the lesion was composed of multiple thin-walled cysts without significant solid areas. Eight blocks were taken for histologic examination. The biopsy from the right ovary measured 3 X 1.5 X 1 cm. On its surface were small, thin-walled multilocular cysts. Microscopic examination showed that the large cysts and follicles of varying size were lined by low cubic epithelium, and contained colloid. The follicles resembled those of normal thyroid tissue. No significant atypia, mitotic activity, nor vascular invasion were found (Fig. 1). The small cystic structures on the surface of the right ovary were histologically similar to the tumor tissue of t...