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Malignant struma Ovarii - A case report and review of the literature

Division of Gynecologic Oncology, Department of Obstetrics and Gynecology and Women's Health, Bronx, NY 10461, USA.
Gynecologic and Obstetric Investigation (Impact Factor: 1.1). 02/2008; 65(2):104-7. DOI: 10.1159/000108654
Source: PubMed

ABSTRACT Struma ovarii is a rare monodermal ovarian teratoma composed predominantly of mature thyroid tissue. Of these cases, 5-8% are clinically hyperthyroid and 5-10% of these tumors are malignant.
A 53-year-old female presented with a 19 x 5 x 5 cm pelvic mass that was treated with bilateral salpingo-oophorectomy, pelvic and para-aortic lymph node sampling, omentectomy and appendectomy and staging for an ovarian tumor. There was no evidence of distant metastases or lymph node invasion. Re-evaluation of the patient after surgery revealed that she was clinically euthyroid and there was no thyroid malignancy. Histopathology revealed papillary thyroid carcinoma arising in struma ovarii (malignant struma ovarii).
Malignant struma ovarii is a very rare malignant ovarian teratoma. In young patients unilateral oophorectomy and complete surgical staging should be considered when the tumor is confined to the one ovary (stage Ia). Long-term follow-up for the detection of metastases or tumor recurrence by serial serum thyroglobulin and (131)I scan or positron emission tomography/computed tomography may be required in selected patients with this rare tumor.

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    ABSTRACT: Malignant struma ovarii is a rare type of ovarian tumor. Metastasis from malignant struma ovarii is rare and has only been documented in 5-6% of cases. The natural history and optimal treatment strategy for malignant struma ovarii remains controversial due to its rarity. The current report presents the case of a 45-year-old female who presented with a tumor of the rib bone. Following resection, the postoperative diagnosis was a metastasizing thyroid carcinoma. No abnormality was detected in the thyroid gland, however, computed tomography revealed a tumor in the left ovary. The patient underwent a left salpingo-oophorectomy and a wedge resection of the right ovary. The postoperative diagnosis was determined as a mature cystic teratoma with malignant struma ovarii (thyroid type, follicular carcinoma) of the left ovary and mature cystic teratoma of the right ovary. Four years subsequent to the initial diagnosis, multiple lung metastases were detected. The following chemotherapies were administered sequentially and intermittently: Tegafur-uracil, paclitaxel/carboplatin and oral etoposide. During this period, the metastatic lesions extended into the bone and progressed slowly. The patient continues to survive with the disease and 24 years have passed since the initial diagnosis, 20 years following the diagnosis of multiple lung metastates. The present report describes a rare case of malignant struma ovarii in which surgical resection and pathological examination of a metastatic rib tumor resulted in the identification of the primary ovarian lesion. The clinical behavior of malignant struma ovarii does not necessarily indicate a histological malignancy, therefore, prediction of future metastasis is difficult and the optimal treatment strategy for malignant struma ovarii is controversial. The present case indicates that the long-term use of oral anticancer agents may facilitate the maintenance of tumor dormancy.
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    ABSTRACT: Struma ovarii is an ovarian teratoma, represented in more than 50% by thyroid tissue. Five percent of struma ovarii cases have been proven to be malignant and, as in the thyroid gland, papillary thyroid carcinoma is the most common histotype arising in struma ovarii. Because of the unusual occurrence of this tumor, its management and follow-up after pelvic surgery is still controversial. Usually, total thyroidectomy followed by radioiodine treatment is the choice treatment in metastatic malignant struma ovarii, while these procedures are still controversial in non-metastatic thyroid cancer arising in struma ovarii. We report a female with follicular variant of papillary thyroid carcinoma arising in struma ovarii. After pelvic surgery, thyroid morphofunctional examinations were performed and a single nodular lesion in the left lobe was discovered. The patient underwent total thyroidectomy and histological examination showed a papillary carcinoma. Radioiodine-ablation of residual thyroid tissue was performed and levothyroxine mildly-suppressive treatment was started. A more aggressive treatment should not be denied for malignant struma ovarii without any evidence, even when apparently confined into the ovary. However, in selected cases, aggressive treatment may be advisable to decrease the risk of recurrence and to allow an accurate follow-up.