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Struma Ovarii

Struma Ovarii

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Struma ovarii is a rare ovarian tumor that was first described in 1899. It is defined by the presence of thyroid tissue comprising more than 50% of the overall mass. It most commonly occurs as part of a teratoma, but may occasionally be encountered with serous or mucinous cystadenomas. [1] Strumae ovarii comprise 1% of all ovarian tumors and 2-5% of ovarian teratomas.

Several variants of the tumor exist. Benign strumosis is a rare version where mature thyroid tissue implants are present throughout the peritoneal cavity. Strumal carcinoid is defined by the presence of carcinoid tissue within a struma ovarii. The vast majority of strumae ovarii are benign, but malignant disease is found in a small percentage of cases, the most common being papillary thyroid carcinoma.

The symptoms of struma ovarii are similar to those of other ovarian tumors and are nonspecific in nature. The tumor can be characterized by imaging, but the final diagnosis is made by pathological and histological examination. Surgical resection remains the definitive treatment for benign disease, and surgery with adjuvant radioiodine therapy has been shown to be successful in treating metastatic and recurrent disease. [2]

United States

Struma ovarii is rare. Approximately 1% of all ovarian tumors and 2.7% of all dermoid tumors are classified as struma ovarii. [3]

Malignancy is defined by various criteria in different studies, principally differing on classifying struma as either a thyroid or ovarian cancer. In the most recent World Health Organization classification, malignant struma ovarii are included in the thyroid tumor group [3] Several other types of tumors, such as Brenner tumor or cystadenoma, may also be found with a struma.

·         Malignant change seems to occur in about a third of cases. [4]

·         Metastatic spread, which follows the pattern of ovarian cancer, occurs in approximately 5% of malignant cases. [4]

·         Survival rates are excellent. [5]

Although the tumor is predominately composed of thyroid tissue, thyrotoxicosis is seen in only 5% of all cases. Only 1 case of thyrotoxicosis resulting from peritoneal strumosis has been reported. [6]

In a study of 68 patients with malignant struma ovarii, Goffredo et al found excellent disease-specific survival rates for the condition no matter which treatment—unilateral oophorectomy, bilateral oophorectomy, oophorectomy and omentectomy, or debulking surgery—was used. The report, which utilized the Surveillance, Epidemiology, and End Results (SEER) database of the National Cancer Institute, also determined that the risk of aggressive thyroid cancer in patients with malignant struma ovarii is high. The overall 5-, 10-, and 20-year survival rates in the study were 96.7%, 94.3%, and 84.9%, respectively, with only one of the deaths that occurred being attributed to malignant struma ovarii. The investigators found, however, that six patients (8.8%) were diagnosed concomitantly or subsequently with thyroid cancer, with two thirds of the thyroid cancers growing beyond the thyroid gland. All of the thyroid cancer patients were still alive at the last follow-up. [5]

 

Because of its rarity, no clear racial predilection for struma ovarii has been determined.

Defined as a tumor of ovarian origin, struma ovarii occurs exclusively in genetic females.

See the list below:

Struma ovarii typically presents between the ages of 40-60.

Struma ovarii rarely occurs before puberty.

Utsunomiya D, Shiraishi S, Kawanaka K, Lwakatare F, Tomiguchi S, Kido R, et al. Struma ovarii coexisting with mucinous cystadenoma detected by radioactive iodine. Clin Nucl Med. 2003 Sep. 28 (9):725-7. [Medline].

Yoo SC, Chang KH, Lyu MO, Chang SJ, Ryu HS, Kim HS. Clinical characteristics of struma ovarii. J Gynecol Oncol. 2008 Jun. 19(2):135-8. [Medline]. [Full Text].

Roth LM, Talerman A. The enigma of struma ovarii. Pathology. 2007 Feb. 39(1):139-46. [Medline].

Bal A, Mohan H, Singh SB, Sehgal A. Malignant transformation in mature cystic teratoma of the ovary: report of five cases and review of the literature. Arch Gynecol Obstet. 2007 Mar. 275(3):179-82. [Medline].

Goffredo P, Sawka AM, Pura J, et al. Malignant Struma Ovarii: A Population-Level Analysis of a Large Series of 68 Patients. Thyroid. 2014 Nov 6. [Medline].

Kim D, Cho HC, Park JW, Lee WA, Kim YM, Chung PS. Struma ovarii and peritoneal strumosis with thyrotoxicosis. Thyroid. 2009 Mar. 19(3):305-8. [Medline].

Mui MP, Tam KF, Tam FK, Ngan HY. Coexistence of struma ovarii with marked ascites and elevated CA-125 levels: case report and literature review. Arch Gynecol Obstet. 2009 May. 279(5):753-7. [Medline].

Shen J, Xia X, Lin Y, Zhu W, Yuan J. Diagnosis of Struma ovarii with medical imaging. Abdom Imaging. 2011 Oct. 36(5):627-31. [Medline].

Jung SI, Kim YJ, Lee MW, Jeon HJ, Choi JS, Moon MH. Struma ovarii: CT findings. Abdom Imaging. 2008 Nov-Dec. 33(6):740-3. [Medline].

Weinberger V, Kadlecova J, Minář L, Felsinger M, Anton M, Ovesna P, et al. Struma ovarii – ultrasound features of a rare tumor mimicking ovarian cancer. Med Ultrason. 2018 Aug 30. 20 (3):355-361. [Medline].

Wei S, Baloch ZW, LiVolsi VA. Pathology of Struma Ovarii: A Report of 96 Cases. Endocr Pathol. 2015 Dec. 26 (4):342-8. [Medline].

Shaco-Levy R, Peng RY, Snyder MJ, Osmond GW, Veras E, Bean SM, et al. Malignant struma ovarii: a blinded study of 86 cases assessing which histologic features correlate with aggressive clinical behavior. Arch Pathol Lab Med. 2012 Feb. 136(2):172-8. [Medline].

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 Jun. 89(3):543-8. [Medline].

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 Sep. 94(3):835-9. [Medline].

Robboy SJ, Shaco-Levy R, Peng RY, Snyder MJ, Donahue J, Bentley RC. Malignant struma ovarii: an analysis of 88 cases, including 27 with extraovarian spread. Int J Gynecol Pathol. 2009 Sep. 28(5):405-22. [Medline].

Lisa Rubinsak, MD Fellow in Advanced Pelvic Surgery, Emory University School of Medicine

Lisa Rubinsak, MD is a member of the following medical societies: American Congress of Obstetricians and Gynecologists, American Medical Association

Disclosure: Nothing to disclose.

David Chelmow, MD Leo J Dunn Professor and Chair, Department of Obstetrics and Gynecology, Virginia Commonwealth University Medical Center

David Chelmow, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, American Medical Association, American Society for Colposcopy and Cervical Pathology, Association of Professors of Gynecology and Obstetrics, Council of University Chairs of Obstetrics and Gynecology, Phi Beta Kappa, Sigma Xi, Society for Academic Specialists in General Obstetrics and Gynecology, Society for Reproductive Investigation

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

A David Barnes, MD, MPH, PhD, FACOG Consulting Staff, Department of Obstetrics and Gynecology, Mammoth Hospital (Mammoth Lakes, CA), Pioneer Valley Hospital (Salt Lake City, UT), Warren General Hospital (Warren, PA), and Mountain West Hospital (Tooele, UT)

A David Barnes, MD, MPH, PhD, FACOG is a member of the following medical societies: American College of Forensic Examiners Institute, American College of Obstetricians and Gynecologists, Association of Military Surgeons of the US, American Medical Association, Utah Medical Association

Disclosure: Nothing to disclose.

Michel E Rivlin, MD Former Professor, Department of Obstetrics and Gynecology, University of Mississippi School of Medicine

Michel E Rivlin, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, American Medical Association, Mississippi State Medical Association, Royal College of Surgeons of Edinburgh, Royal College of Obstetricians and Gynaecologists

Disclosure: Nothing to disclose.

Jordan G Pritzker, MD, MBA, FACOG Adjunct Professor of Obstetrics/Gynecology, Hofstra North Shore-LIJ School of Medicine at Hofstra University; Attending Physician, Department of Obstetrics and Gynecology, Long Island Jewish Medical Center; Medical Director, Aetna, Inc; Private Practice in Gynecology

Disclosure: Nothing to disclose.

Jeannie Chen Kelly, MD Resident Physician, Department of Obstetrics and Gynecology, Tufts Medical Center

Disclosure: Nothing to disclose.

Sarah H Hughes, MD Assistant Professor, Department of Obstetrics and Gynecology, Division of Gynecologic Oncology, University of Massachusetts Medical School

Sarah H Hughes, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, Society of Gynecologic Oncology

Disclosure: Nothing to disclose.

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author Bradford W Fenton, MD, PhD, FACOG to the development and writing of this article.

Struma Ovarii

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