Malignant struma ovarii: a case report and analysis of cases reported in the literature with focus on survival and i131 therapy

doi:10.1016/S0090-8258(03)00141-0

Gynecologic Oncology

Volume 89, Issue 3, June 2003, Pages 543-548

https://doi.org/10.1016/S0090-8258(03)00141-0 Get rights and content

Abstract

Background

Malignant struma ovarii is a rare type of germ cell tumor that is most often diagnosed postoperatively. The natural history and optimal treatment regimen for the disease are essentially unknown due to the small numbers of published cases.

Case

A 32-year-old woman presented with pelvic pain and an ovarian mass that was ultimately treated by total abdominal hysterectomy/bilateral salpingo-oophorectomy. Postoperatively, she was diagnosed with a malignant struma ovarii. The patient was subsequently treated with thyroidectomy and I¹³¹ ablation and is currently disease free. A Medline literature search was performed and clinical data from 23 additional cases were compiled.

Conclusion

In this review of 24 cases, 16 patients were followed conservatively postoperatively while 8 received varied additional therapy (4 with I¹³¹). There were 8 recurrences and all occurred in the conservatively managed patients. I¹³¹ for recurrent disease provided an initial complete response in 7 women. Treatment with thyroidectomy and I¹³¹ should be considered in the first line of management for malignant struma ovarii.

Introduction

Malignant struma ovarii is a rare presentation of a specialized germ cell tumor. Germ cell tumors account for 15 to 20% of all ovarian tumors and 95% of these are mature teratomas [1], [2], [3]. These tumors are composed of epithelial tissue and can include hair, skin, teeth, bone, and thyroid tissue among other tissues. Fifteen percent of all teratomas contain thyroid tissue [1], [3], [4], [5], [6]. To be classified as a struma ovarii, thyroid tissue must be the predominant histology within a teratoma [2], [3], [4], [7]. Struma ovarii accounts for only 2% of all mature teratomas and malignant transformation has been reported in fewer than 5% of all struma [1], [2], [3], [4], [5], [8], [9], [10], [11], [12], [13], [14].

Most cases of malignant struma ovarii are subclinical. The typical patient presents with pelvic pain. It is rare for patients to present with overt signs of hyperthyroidism. Imaging studies usually diagnose an ovarian mass and surgical intervention follows. In the absence of metastatic lesions, the diagnosis of malignant struma ovarii is based on criteria used to diagnose thyroid carcinoma. These include the presence of overlapping “ground glass nuclei,” vascular space invasion, or mitotic activity [7]. Both papillary and follicular carcinomas have been reported in a setting of struma ovarii with follicular carcinomas being more common [2], [5], [7], [8], [13].

Presented is a case of struma ovarii with malignant histologic features. In addition, an analysis of malignant struma ovarii cases documented in the literature since 1970 was performed and recommendations regarding potential treatment options follow.

Section snippets

Case report

S.M. is a 32-year-old white female who presented with pelvic pain. Her pain was dull and constant throughout her cycle; however, during menses the pain was described as sharp and stabbing. It typically radiated to her left lower quadrant. She had irregular cycles and described two to three episodes of spotting (duration of 3–4 days) every month. The patient also complained of severe dyspareunia and frequent episodes of diarrhea during menses. Abdominal ultrasonography revealed a hypoechoic left

Materials and methods

A Medline literature search of the English language articles since 1970 was performed. Key words utilized included malignant struma ovarii, struma ovarii, and germ cell tumors. All articles were then reviewed and any additional referenced articles examined as well. All cases of malignant struma ovarii were collected and data abstracted on presentation, treatment, and outcomes. Those cases without the appropriate clinical information were not included. Statistical analysis was performed using

Results of literature search

Listed in Table 1 are the clinical features of 24 cases of malignant struma ovarii, the 1 from our institution and 23 that have been previously reported in the literature. The mean age of patients was 42.9 years. The most common finding on presentation was a pelvic mass (58% of patients). The mean size was 10.5 cm ± 4.7 cm (range 5–20 cm). If a mass was present, it frequently involved the left ovary (63%); ascites was present in 17% of the cases. Pelvic pain was the most common presenting

Discussion

Malignant struma ovarii is a rare gynecologic cancer. Most of our knowledge has been gleaned from individual case reports. The peak age of incidence is 50 years [2], [4], [8]. Malignant struma ovarii arises within mature teratomas in 0.1% of cases [4], [6], [15]. They are usually unilateral with clinical hyperthyroidism being found in fewer than 8% of reported cases [4], [6], [7], [8], [9], [14].

Initial criteria for pathologic diagnosis were proposed by Geist and Smith in the mid 1940s.

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Citation Excerpt :
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Struma ovarii (SO) is a rare ovarian mature teratoma which is composed of 50% or more thyroid tissues. Malignant transformation is reported to occur in less than 5% of all cases, and it leads to 5–6% metastatic disease. The most common type is a papillary carcinoma. However, follicular carcinoma of arising from SO is extremely rare.
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It was diagnosed with follicular carcinoma, stage IA. After the multidisciplinary team meeting, we decided not to conduct additional treatment, including total thyroidectomy followed by radioiodine treatment and T4 therapy because thyroid tests were normal findings.
There is still no established treatment consensus for follicular carcinoma of SO patients. The choice for a conservative or radical approach depends on the doctor. However, conservative surgery could be considered for low risk, early stage patients who have childbearing potential.
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Short communicationMalignant struma ovarii: a case report and analysis of cases reported in the literature with focus on survival and i131 therapy

Abstract

Background

Case

Conclusion

Introduction

Section snippets

Case report

Materials and methods

Results of literature search

Discussion

Gynecol Oncol

Gynecol Oncol

Gynecol Oncol

Gynecol Oncol

Gynecol Oncol

Gynecol Oncol

Malignant struma ovarii treated by ovariectomy, thyroidectomy, and I-131 administration

Cancer

Malignant struma ovarii. A case report and review of the literature

Gynecol Obstet Invest

Malignant struma ovarii

Pathol Oncol Res

Malignant strum ovariian unusual presentation

Thyroid

Malignant struma ovariireport of two cases and selected review of the literature

J Surg Oncol

Short communication
Malignant struma ovarii: a case report and analysis of cases reported in the literature with focus on survival and i¹³¹ therapy