Presentation and treatment of two cases of malignant struma ovarii
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SUBMITTER: Obeidat R
PROVIDER: S-EPMC10913386 | biostudies-literature | 2024 Jan
REPOSITORIES: biostudies-literature
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Project description:Background: Malignant struma ovarii (MSO) is an extremely rare ovarian malignant tumor and there is limited data on the survival outcomes and prognostic predictors of MSO. The objectives of this study were to investigate the disease-free survival (DFS), overall survival (OS), and disease-specific survival (DSS) rates of patients with MSO, and also evaluate the prognostic factors in this population. Methods: A retrospective study was conducted and 194 cases of MSO were selected. DFS was assessed by the logistic regression, OS by the Kaplan-Meier method, and DSS was evaluated by the Cox regression. Results: The median age of these patients was 46.0 years; 142 cases (73.2%) were confined to the ovary and 52 cases (26.8%) had extraovarian metastasis at the initial diagnosis of MSO. During the follow-up, 75.3% of these patients showed no evidence of disease and 18.0% were alive with disease. Only 13 deaths occurred, with 10 attributed to MSO. The 5, 10, and 15-year OS rates were 91.4, 87.7, and 83.5%, respectively. The 5, 10, and 15-year DSS rates were 93.8, 90.0, and 85.7%, respectively. Logistic regression revealed that International Federation of Gynecology and Obstetrics (FIGO) stage IV was the only risk factor for DFS [p < 0.001; odds ratio (OR) 7.328; 95% CI 3.103-16.885, FIGO stage IV vs. stage I; p = 0.021; OR 4.750, 95% CI 1.264-17.856, FIGO stage IV vs. stage II-III]. The multivariate Cox regression analysis showed that poor differentiation was the only risk factor for both OS (p = 0.005, OR 6.406; 95% CI 1.730-23.717) and DSS (p = 0.001, OR 9.664; 95% CI 2.409-38.760), while age ≥45 years was the prognostic predictor for OS (p = 0.038, OR 4.959; 95% CI 1.093-22.508). Conclusion: Survival outcomes were excellent in patients with MSO, irrespective of the treatment strategy, FIGO stage IV, age ≥45 years, and poor differentiation of tumors were the independent risk factors.
| S-EPMC8733601 | biostudies-literature
Project description:BackgroundMalignant struma ovarii (MSO) is a unique type of ovarian malignancy that data on the survival outcome is limited and management strategy remains controversial due to its extreme rarity.MethodsTo investigate the clinical characteristics and treatment options in patients with MSO confined to the ovary, while also evaluating the recurrent-free survival (RFS) and overall survival (OS) rate in this population, a retrospective study was conducted. One hundred twenty-five cases of MSO confined to the ovary were enrolled and their clinical characteristics, treatment strategies, and results of follow-up were analyzed. OS and RFS were assessed by Kaplan-Meier analyses and Cox regression models.ResultsThe most common pathological subtype in this cohort was papillary carcinoma (44.8%). Other reported subtypes, in order of prevalence, were follicular variant of papillary carcinoma, follicular carcinoma, and mixed follicular-papillary carcinoma. Surgical treatment options varied in this cohort that 8.0% of the patients received ovarian cystectomy, 33.6% underwent unilateral salpingo-oophorectomy (USO), 5.6% received bilateral salpingo-oophorectomy (BSO), 21.6% received total abdominal hysterectomy with BSO (TAH/BSO), and 17.6% were treated with debulking surgery; 20.0% of them received radioiodine therapy (RAI). Twenty-seven patients experienced recurrence with a median RFS of 14.0 years (95% confidence interval [CI], 9.5-18.5). The 5-year and 10-year recurrent rate were 27.1, 35.2%, respectively. Eight patients died during follow-up, with five attributed to MSO; the 5-year, 10-year, and 20-year OS rate was 95.3, 88.7 and 88.7%, respectively. However, the univariate and multivariate Cox regression showed no potential risk factor for RFS and OS.ConclusionPatients with MSO confined to the ovary had an excellent survival outcome, despite varied treatment strategies, and the recurrent rate was relatively high. We recommend USO as the preferred surgical option in this population since more aggressive surgery does not improve outcomes and the benefits of RAI are uncertain.
| S-EPMC8033663 | biostudies-literature
Project description:Malignant struma ovarii presenting with follicular carcinoma is extremely rare, and its mechanism of tumorigenesis remains unknown. Here, we present a case of malignant struma ovarii with peritoneal dissemination of follicular carcinoma, for which a molecular analysis for major oncogenic gene alterations in follicular thyroid carcinoma was performed. A 39-year-old nulliparous woman was referred with a diagnosis of highly differentiated follicular carcinoma of ovarian origin. Primary thyroid cancer was not diagnosed, and she had a normal thyroid function. 123I scintigraphy revealed multiple peritoneal dissemination that was surgically resected. Histologically, the tumor consisted of numerous follicles without nuclear features of papillary thyroid carcinoma. Tumor samples were investigated for 50 cancer-related genes, including RAS, BRAF, and p53, and PPARg-PAX8 gene fusion by targeted DNA sequencing and fluorescence in situ hybridization, respectively. No major oncogenic gene alterations were detected. These negative findings suggest a different mechanism of tumorigenesis from that of adult-type follicular thyroid carcinoma.
| S-EPMC6745508 | biostudies-literature
Project description:Malignant struma ovarii (MSO) is an extremely rare monodermal ovarian teratoma. Preoperative diagnosis and intraoperative freezing diagnosis are exceedingly difficult due to the rarity of the disease and its clinically noncharacteristic manifestations with less than 200 reports in the current literature. In this paper, a case of MSO (papillary carcinoma) with hyperthyroidism was discussed in terms of its epidemiology, clinicopathology, molecular features, treatment, and prognosis.
| S-EPMC10294126 | biostudies-literature
Project description:BackgroundSince malignant struma ovarii is a very rare disease, its carcinogenic mechanism has not been elucidated. Here, we sought to identify the genetic lesions that may have led to the carcinogenesis of a rare case of malignant struma ovarii (follicular carcinoma) with peritoneal dissemination.MethodsDNA was extracted from the paraffin-embedded sections of normal uterine tissues and malignant struma ovarii for genetic analysis. Whole-exome sequencing and DNA methylation analysis were then performed.ResultsGermline variants of RECQL4, CNTNAP2, and PRDM2, which are tumor-suppressor genes, were detected by whole-exome sequencing. Somatic uniparental disomy (UPD) was also observed in these three genes. Additionally, the methylation of FRMD6-AS2, SESN3, CYTL1, MIR4429, HIF3A, and ATP1B2, which are associated with tumor growth suppression, was detected by DNA methylation analysis.ConclusionsSomatic UPD and DNA methylation in tumor suppressor genes may be associated with the pathogenesis of malignant struma ovarii. To our knowledge, this is the first report of whole-exome sequencing and DNA methylation analysis in malignant struma ovarii. Genetic and DNA methylation analysis may help elucidate the mechanism of carcinogenesis in rare diseases and guide treatment decisions.
| S-EPMC10047136 | biostudies-literature
Project description:Malignant struma ovarii (MSO) with synchronous primary thyroid cancer in the neck is extremely rare and lacks a treatment consensus. A 44-year-old woman presenting with a left ovarian cyst was admitted to Peking Union Medical College Hospital (Beijing, China) Ultrasonography showed a 6 cm solid-cystic left ovarian mass with plentiful blood signals. Other notable findings were an elevated CA125 level and a suspected malignant thyroid nodule. A unilateral salpingo-oophorectomy (USO) was conducted, and the surgical pathology was papillary thyroid cancer (PTC) arising in a struma ovarii. The patient underwent a total thyroidectomy and cervical lymph node dissection, and the pathology of the right lobe nodule was follicular-variant PTC without capsule invasion or lymph node metastasis (5 mm; pT1aN0M0). No further adjuvant therapy was administered. The serum thyroglobulin value was normal before surgery and was undetectable after thyroidectomy. During regular follow-up examinations over 4 years, the patient remained well with no evidence of disease (NED). In a literature review, another 13 cases of MSO coexisting with cervical thyroid cancer that had reported outcomes were found. The MSO was confined to the ovary in all cases. A total of nine patients received radioiodine therapy (RAI) treatment after total thyroidectomy. Two patients relapsed and were successfully cured with RAI after the initial surgery Only one patient died due to another disease, while 11 patients showed NED and the remaining patient was alive with the disease after a median follow-up time of 2 years. This data suggests that USO with personalized RAI may be a preferred option for MSO confined to the ovary plus synchronous primary thyroid cancer due to the conferred satisfactory prognosis.
| S-EPMC10918513 | biostudies-literature
Project description:Introduction: Metastatic malignant struma ovarii (MSO) is an extremely rare disease that lacks treatment consensus and accurate prognosis. The objective of this study was to present the clinical, pathological, and treatment characteristics of metastatic MSO, while also investigate the overall survival (OS) rate and factors affecting prognosis in this population. Materials and Methods: A total of 79 cases of metastatic MSO were reviewed, including four cases of metastatic MSO from our hospital and 75 cases selected from the literature. Logistic regression was used to identify potential factors affecting disease free survival (DFS). The Kaplan-Meier method and log-rank test were used to determine OS; further Cox regression was used to evaluate factors affecting OS. Results: The mean age of all the patients at diagnosis was 43.8 years. The most common metastatic sites were peritoneum, bone, liver, omentum and lung in descending order. Only two patients (2.6%) coexisted with local primary thyroid cancer. Follicular carcinoma (41.8%) as the most prevalent subtype, followed by papillary carcinoma, follicular variant of papillary carcinoma, and mixed follicular-papillary carcinoma. 36.7% of the patients received conservative surgery, 43.0% of them underwent aggressive surgery, and 15.2% of them did not receive any surgery. 74.7% of patients who received adjuvant therapy underwent radioiodine therapy (RAI). Logistics regression revealed that FIGO stage IV was the only prognostic factor in predicting DFS (P = 0.002; Odds Ratio [OR] 5.333; 95% confidence interval [CI]: 1.839-15.471). Only seven deaths occurred. The OS rates at 5, 10, 15 years were 89.3, 82.4, 65.9%, respectively. Multivariate analysis showed age over 55 years (P = 0.006; OR 9.362; 95%CI: 1.895-46.246) was the only risk factor for OS. Conclusion: Patients with metastatic MSO have an excellent disease-specific OS rate, FIGO stage IV and age over 55 years were two factors affecting disease prognosis. Conservative surgery with residual ablation by RAI after total thyroidectomy should be preferred since the benefits of aggressive surgery are uncertain.
| S-EPMC7550973 | biostudies-literature
Project description:Background: SO (Struma ovarii) is a rare form of ovarian teratoma which originates from ovarian dermoid cysts. Due to the rarity of this disease, relevant studies might not be sufficiently documented, especially cases with hyperthyroidism and multiple metastases. Case Presentation: A 40-year-old female patient was admitted to our hospital due to management of early pregnancy along with a recurrent abdominal and pelvic mass. Contrast-enhanced CT images showed an irregular mass (10.7 × 8.6 × 12.8 cm) located in the right side from the hypogastrium to the pelvic cavity and another mass (3.8 × 3.7 cm) in the liver. Laboratory examination showed that CA125 (Carbohydrate Antigen-125) was 118.10 U/mL, Tg (thyroglobulin) was >300 ng/ml, FT4 (free thyroxine) was 22.11 pmol/L, and TSH (thyroid-stimulating hormone) was <0.004 mIU/L. She subsequently underwent liver mass dissection, omentectomy, tumor dissection, peritoneal nodule resection, as well as rectal anterior wall nodule resection. The patient was diagnosed with malignant SO (papillary type) along with multiple metastases. Also, we conducted a literature review based on 290 SO cases from 257 articles. Conclusion: This study showed that malignant SO might be prone to relapse and metastasize (a metastatic rate of 52.94%) and therefore aggressive management might need to be recommended for malignant SO. Also, laparotomy might need to be recommended for large tumors that cannot be resected by laparoscopic surgery since these tumors might be prone to rupture and thus produce peritoneal implants. Furthermore, Graves' disease might need to be considered in the differential diagnosis.
| S-EPMC9127674 | biostudies-literature
Project description:IntroductionStruma 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.Case presentationA 23-year-old nullipara complained of right lower abdominal pain. CT scan revealed a large complex cystic and solid mass in pelvic cavity with moderate amount ascites with peritoneal thickening and omental infiltration. We performed a right salpingo-oophorectomy and surgical staging.Clinical discussionIt 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.ConclusionThere 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.
| S-EPMC8606842 | biostudies-literature
Project description:Struma ovarii is a rare tumor, which accounts for 2.7% of ovarian teratomas and 0.01% of ovarian tumors. It usually affects women in the fifth decades of life and is most often detected incidentally during ultrasound and then confirmed histologically. The gold standard treatment is surgery and prognosis is excellent. We here report the particular case of a young female patient with giant struma ovarii whose radiological features were classified as unusual presentation. We match patient's data with those from the literature.
| S-EPMC5491739 | biostudies-other