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Malignant Struma Ovarii: An Analysis of 88 Cases, Including 27 With Extraovarian Spread

Robboy, Stanley J. M.D.; Shaco-Levy, Ruthy M.D.; Peng, Ruth Y. M.D.; Snyder, Matthew J. M.D.; Donahue, John M.D.; Bentley, Rex C. M.D.; Bean, Sarah M.D.; Krigman, Hannah R. M.D.; Roth, Lawrence M. M.D.; Young, Robert H. M.D.

International Journal of Gynecological Pathology: September 2009 - Volume 28 - Issue 5 - p 405-422
doi: 10.1097/PGP.0b013e3181a27777

Struma ovarii that display extraovarian spread or later recurrence is exceedingly rare. Among 88 patients with “malignant” struma ovarii followed for prolonged periods, several features helped to predict the adverse clinical course. Adhesions (graded 2 to 4+), peritoneal fluid (≥1 L) or ovarian serosal rent were worrisome features, occurring in 74% of 27 biologically malignant tumors but only 10% of 61 clinically benign tumors. The size of the strumal component rather than the overall size of the ovarian teratoma also had some predictive value. Tumors with a strumal component ≤6 cm recurred rarely (7%), whereas 33% of the consult and 88% of the literature cases ≥12 cm were clinically malignant. Except for a papillary pattern or poorly differentiated cancer, no microscopic feature reliably predicted the clinical outcome, including those typically associated with malignancy in primary thyroid tumors. Among the consult cases, 7% with histologic follicular adenomas and 29% with papillary carcinomas were clinically malignant. Unequivocal vascular invasion was rare, precluding assessment of its effect. Optically clear nuclei, when extensive, were useful to diagnose papillary carcinoma, but were present nevertheless in smaller numbers in both macrofollicular and microfollicular adenomas. Eight tumors confined initially to the ovary (stage 1) recurred. Papillary carcinomas recurred earlier (average 4 y) than follicular adenomatous neoplasms (average 11 y, range: 1-29 y). Overall, the survival rate for all patients was 89% at 10 years and 84% at 25 years, indicating the need for routine long-term follow-up.

Department of Pathology, (S.J.R., R.S-L., R.C.B., S.B.) Duke University Medical Center, Durham, NC

Departments Obstetrics and Gynecology, (S.J.R.) Duke University Medical Center, Durham, NC

Department of Pathology, (R.S-L.) Soroka Medical Center and Ben Gurion University, Beer-Sheva, Israel

Department of Pathology, (R.Y.P.) Baltimore City Hospital, Baltimore, MD

Department of Pathology, (M.J.S.) Wake-Med Medical Center, Raleigh, NC

Department of Pathology, (J.D.) Clara Maase Medical Center, Belleview, NJ

Department of Pathology, (H.R.K.) Washington University Medical Center St. Louis, MO

Department of Pathology, (L.M.R.) Indiana University School of Medicine

Departments of Pathology, (R.H.Y.) Harvard Medical School and James Homer Wright Laboratories of the Massachusetts General Hospital, Boston, MA

Address correspondence and reprint requests to Stanley J. Robboy, MD, Department of Pathology, Duke University Medical Center, Box 3712, Durham, NC 27710. E-mail:

©2009International Society of Gynecological Pathologists