- Douglas S Ross, MD
Douglas S Ross, MD
- Section Editor — Thyroid Disease
- Professor of Medicine
- Harvard Medical School
Struma ovarii is a specialized or monodermal teratoma predominantly composed of mature thyroid tissue . Thyroid tissue must comprise more than 50 percent of the overall tissue to be classified as a struma ovarii. Struma ovarii accounts for approximately 5 percent of all ovarian teratomas [2-4]. Depending on the histologic features, struma ovarii can be classified as benign or malignant . The clinical presentation, diagnosis, and management of struma ovarii will be reviewed here. Ovarian teratomas are reviewed in detail elsewhere. (See "Ovarian germ cell tumors: Pathology, clinical manifestations, and diagnosis", section on 'Teratomas'.)
The clinical manifestations of struma ovarii are based upon single case reports and small case series [2-7]. Struma ovarii is most common between the ages of 40 and 60 years. Women with struma ovarii usually present with pain and/or a pelvic mass and less frequently with ascites. In case series of 20 to 30 patients with histologically confirmed struma ovarii, abdominal pain was present in 20 to 42 percent and a palpable lower abdominal mass in 23 to 58 percent [4,8]. In one series, no definite symptoms were present in 14 patients (41.2 percent), in whom the presence of an ovarian tumor was incidentally noted on ultrasonography performed for other reasons . Ascites was present in 12 to 17 percent of patients. CA-125 was elevated in 4 of 13 women in whom it was measured (one of three who were found to have malignant struma ovarii).
Clinical and biochemical features of hyperthyroidism are uncommon in women with struma ovarii, occurring in less than 5 to 8 percent of cases [3,5,8-10]. In hyperthyroid patients, the serum TSH is low and free T4 and/or T3 are elevated. The thyroid gland typically is not enlarged, but serum thyroglobulin is elevated. Radioiodine uptake is low or absent in the thyroid gland, but present in the pelvis. Rarely, women with struma ovarii and hyperthyroidism also have a goiter . There are at least two possible explanations for this association:
●The coexistence of Graves' disease and struma ovarii, which has been reported rarely in the literature [11,12]. Serum thyroid-stimulating immunoglobulins (thyrotropin-receptor antibodies) would be expected to stimulate function of thyroid tissue in the ovary as well as in the neck.
●A toxic nodular goiter with parallel formation of thyroid autonomy in an ovarian teratoma.
- Dunzendorfer T, deLas Morenas A, Kalir T, Levin RM. Struma ovarii and hyperthyroidism. Thyroid 1999; 9:499.
- Kondi-Pafiti A, Mavrigiannaki P, Grigoriadis Ch, et al. Monodermal teratomas (struma ovarii). Clinicopathological characteristics of 11 cases and literature review. Eur J Gynaecol Oncol 2011; 32:657.
- Yassa L, Sadow P, Marqusee E. Malignant struma ovarii. Nat Clin Pract Endocrinol Metab 2008; 4:469.
- Yoo SC, Chang KH, Lyu MO, et al. Clinical characteristics of struma ovarii. J Gynecol Oncol 2008; 19:135.
- Kraemer B, Grischke EM, Staebler A, et al. Laparoscopic excision of malignant struma ovarii and 1 year follow-up without further treatment. Fertil Steril 2011; 95:2124.e9.
- Devaney K, Snyder R, Norris HJ, Tavassoli FA. Proliferative and histologically malignant struma ovarii: a clinicopathologic study of 54 cases. Int J Gynecol Pathol 1993; 12:333.
- Jean S, Tanyi JL, Montone K, et al. Papillary thyroid cancer arising in struma ovarii. J Obstet Gynaecol 2012; 32:222.
- DeSimone CP, Lele SM, Modesitt SC. Malignant struma ovarii: a case report and analysis of cases reported in the literature with focus on survival and I131 therapy. Gynecol Oncol 2003; 89:543.
- Ayhan A, Yanik F, Tuncer R, et al. Struma ovarii. Int J Gynaecol Obstet 1993; 42:143.
- Young RH. New and unusual aspects of ovarian germ cell tumors. Am J Surg Pathol 1993; 17:1210.
- Kung AW, Ma JT, Wang C, Young RT. Hyperthyroidism during pregnancy due to coexistence of struma ovarii and Graves' disease. Postgrad Med J 1990; 66:132.
- Teale E, Gouldesbrough DR, Peacey SR. Graves' disease and coexisting struma ovarii: struma expression of thyrotropin receptors and the presence of thyrotropin receptor stimulating antibodies. Thyroid 2006; 16:791.
- Makani S, Kim W, Gaba AR. Struma Ovarii with a focus of papillary thyroid cancer: a case report and review of the literature. Gynecol Oncol 2004; 94:835.
- Zalel Y, Seidman DS, Oren M, et al. Sonographic and clinical characteristics of struma ovarii. J Ultrasound Med 2000; 19:857.
- Robboy SJ, Shaco-Levy R, Peng RY, et al. Malignant struma ovarii: an analysis of 88 cases, including 27 with extraovarian spread. Int J Gynecol Pathol 2009; 28:405.
- Hasleton PS, Kelehan P, Whittaker JS, et al. Benign and malignant struma ovarii. Arch Pathol Lab Med 1978; 102:180.
- Marti JL, Clark VE, Harper H, et al. Optimal surgical management of well-differentiated thyroid cancer arising in struma ovarii: a series of 4 patients and a review of 53 reported cases. Thyroid 2012; 22:400.
- Ryder M, Nikiforov YE, Fagin JA. Follicular variant papillary thyroid carcinoma arising within an ovarian teratoma. Thyroid 2007; 17:179.
- Brown WW, Shetty KR, Rosenfeld PS. Hyperthyroidism due to struma ovarii: demonstration by radioiodine scan. Acta Endocrinol (Copenh) 1973; 73:266.
- Yeh EL, Meade RC, Ruetz PP. Radionuclide study of struma ovarii. J Nucl Med 1973; 14:118.
- Jammah AA, Driedger A, Rachinsky I. Incidental finding of ovarian teratoma on post-therapy scan for papillary thyroid cancer and impact of SPECT/CT imaging. Arq Bras Endocrinol Metabol 2011; 55:490.
- Young RH, Jackson A, Wells M. Ovarian metastasis from thyroid carcinoma 12 years after partial thyroidectomy mimicking struma ovarii: report of a case. Int J Gynecol Pathol 1994; 13:181.
- Brogioni S, Viacava P, Tomisti L, et al. A special case of bilateral ovarian metastases in a woman with papillary carcinoma of the thyroid. Exp Clin Endocrinol Diabetes 2007; 115:397.
- Gunasekaran S, Kopecka E, Maung KH, England RJ. Struma ovarii and the thyroid surgeon. J Laryngol Otol 2012; 126:858.
- Billan S, Abdah-Bortnyak R, Cohen H, et al. Metastatic malignant struma ovarii. Isr Med Assoc J 2011; 13:247.
- Michels A, Haugen B. Malignant struma ovarii. J Clin Endocrinol Metab 2010; 95:1505.
- Luo JR, Xie CB, Li ZH. Treatment for malignant struma ovarii in the eyes of thyroid surgeons: a case report and study of Chinese cases reported in the literature. Medicine (Baltimore) 2014; 93:e147.
- Willemse PH, Oosterhuis JW, Aalders JG, et al. Malignant struma ovarii treated by ovariectomy, thyroidectomy, and 131I administration. Cancer 1987; 60:178.
- Wolff EF, Hughes M, Merino MJ, et al. Expression of benign and malignant thyroid tissue in ovarian teratomas and the importance of multimodal management as illustrated by a BRAF-positive follicular variant of papillary thyroid cancer. Thyroid 2010; 20:981.
- Coyne C, Nikiforov YE. RAS mutation-positive follicular variant of papillary thyroid carcinoma arising in a struma ovarii. Endocr Pathol 2010; 21:144.